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Association of CNS demyelination and COVID-19 infection: an updated systematic review
Ismail Ibrahim Ismail & Sara Salama
doi : 10.1007/s00415-021-10752-x
Journal of Neurology volume 269, pages 541–576 (2022)
Since the declaration of COVID-19 pandemic, several case reports of demyelination of both peripheral and central nervous systems have been published. The association between CNS demyelination and viral infection has long been documented, and this link was recently reported following SARS-CoV-2 infection as well.
A rise in cases of nitrous oxide abuse: neurological complications and biological findings
Maximilian Einsiedler, Paul Voulleminot, Stanislas Demuth, Pauline Kalaaji, Thomas Bogdan, Lucas Gauer, Cécile Reschwein, Aleksandra Nadaj-Pakleza, Jérôme de Sèze, Laurent Kremer, Ivana Schroder & Kévin Bigaut
doi : 10.1007/s00415-021-10702-7
Journal of Neurology volume 269, pages 577–582 (2022)
The recent lockdown due to the COVID-19 pandemic has been linked to a higher incidence of psychiatric manifestations and substance abuse. The recreative use of nitrous oxide is more and more widespread and neurological complications are frequent.
Case report: cerebral sinus vein thrombosis in two patients with AstraZeneca SARS-CoV-2 vaccination
Mathias Fousse, David Schub, Fatma Merzou, Klaus Fassbender, Martina Sester, Michael Kettner, Piergiorgio Lochner, Tina Schmidt & João Reinoldo Goi Júnior
doi : 10.1007/s00415-021-10731-2
Journal of Neurology volume 269, pages 583–586 (2022)
SARS-CoV-2 infection is associated with an increased rate of thromboembolic events and mortality. Different vaccines are globally used to limit the pandemic. In this report, we present the case of two young female patients with newly diagnosed cerebral sinus vein thrombosis occurring after injection of the vector-based ChAdOx1 vaccine. Both patients presented with unusual headache only. The two of them used an estrogen-containing contraception, had had a history of deep venous thrombosis, and both had MTHFR mutations. Both patients developed SARS-CoV-2 specific humoral and cellular immunity including both CD4 and CD8 T cells. This rare, but serious complication needs to be considered after vaccination of young females, even if there is no evidence of heparin-induced thrombocytopenia.
Autonomic dysfunction in post-COVID patients with and witfhout neurological symptoms: a prospective multidomain observational study
Alex Buoite Stella, Giovanni Furlanis, Nicolò Arjuna Frezza, Romina Valentinotti, Milos Ajcevic & Paolo Manganotti
doi : 10.1007/s00415-021-10735-y
Journal of Neurology volume 269, pages 587–596 (2022)
The autonomic nervous system (ANS) can be affected by COVID-19, and dysautonomia may be a possible complication in post-COVID individuals. Orthostatic hypotension (OH) and postural tachycardia syndrome (POTS) have been suggested to be common after SARS-CoV-2 infection, but other components of ANS function may be also impaired. The Composite Autonomic Symptom Scale 31 (COMPASS-31) questionnaire is a simple and validated tool to assess dysautonomic symptoms. The aim of the present study was to administer the COMPASS-31 questionnaire to a sample of post-COVID patients with and without neurological complaints. Participants were recruited among the post-COVID ambulatory services for follow-up evaluation between 4 weeks and 9 months from COVID-19 symptoms onset. Participants were asked to complete the COMPASS-31 questionnaire referring to the period after COVID-19 disease. Heart rate and blood pressure were manually taken during an active stand test for OH and POTS diagnosis. One-hundred and eighty participants were included in the analysis (70.6% females, 51 ± 13 years), and OH was found in 13.8% of the subjects. Median COMPASS-31 score was 17.6 (6.9–31.4), with the most affected domains being orthostatic intolerance, sudomotor, gastrointestinal and pupillomotor dysfunction. A higher COMPASS-31 score was found in those with neurological symptoms (p < 0.01), due to more severe orthostatic intolerance symptoms (p < 0.01), although gastrointestinal (p < 0.01), urinary (p < 0.01), and pupillomotor (p < 0.01) domains were more represented in the non-neurological symptoms group. This study confirms the importance of monitoring ANS symptoms as a possible complication of COVID-19 disease that may persist in the post-acute period.
Decrease in intravenous thrombolysis and poor short-term functional prognosis for acute ischemic stroke during the COVID-19 pandemic
Xueqian Xu, Yuenan Xiao, Jia Li, Liuzhu Chen, Gangqiang Lin, Ledan Dong, Yisi Lin, Luqian Zhan, Jincai He & Xiaoqian Luan
doi : 10.1007/s00415-021-10740-1
Journal of Neurology volume 269, pages 597–602 (2022)
Since the outbreak of the coronavirus pandemic in 2019 (COVID-19), healthcare systems around the world have been hit to varying degrees. As a neurologist team, for patients with acute ischemic stroke (AIS), we compared the situations of intravenous thrombolysis (IVT) treatment from 2019 to 2020 to investigate the influence of COVID-19 pandemic on the attendance and prognosis of the IVT patients.
Delays in thrombolysis during COVID-19 are associated with worse neurological outcomes: the Society of Vascular and Interventional Neurology Multicenter Collaboration
Dinesh V. Jillella, Fadi Nahab, Thanh N. Nguyen, Mohamad Abdalkader, David S. Liebeskind, Nirav Vora, Vivek Rai, Diogo C. Haussen, Raul G. Nogueira, Shashvat Desai, Ashutosh P. Jadhav, Alexandra L. Czap, Alicia M. Zha, Italo Linfante, Ameer E Hassan, Darko Quispe-Orozco, Santiago Ortega-Gutierrez, Priyank Khandelwal, Pratit Patel, Osama Zaidat, Tudor G. Jovin, Scott Kamen & James E. Siegler
doi : 10.1007/s00415-021-10734-z
Journal of Neurology volume 269, pages 603–608 (2022)
We have demonstrated in a multicenter cohort that the COVID-19 pandemic has led to a delay in intravenous thrombolysis (IVT) among stroke patients. Whether this delay contributes to meaningful short-term outcome differences in these patients warranted further exploration.
Frontal predominant encephalopathy with early paligraphia as a distinctive signature of CAR T-cell therapy-related neurotoxicity
Umberto Pensato, Giulia Amore, Roberto D’Angelo, Rita Rinaldi, Marianna Nicodemo, Francesca Rondelli, Susanna Mondini, Rossella Santoro, Susanna Sammali, Andrea Farolfi, Luca Spinardi, Luca Faccioli, Beatrice Casadei, Michele Dicataldo, Francesca Bonifazi, Pierluigi Zinzani, Pietro Cortelli, Andrea Stracciari & Maria Guarino
doi : 10.1007/s00415-021-10766-5
Journal of Neurology volume 269, pages 609–615 (2022)
Chimeric antigen receptor (CAR) T-cell therapy is an emerging highly effective treatment for refractory haematological malignancies. Unfortunately, its therapeutic benefit may be hampered by treatment-related toxicities, including neurotoxicity. Early aggressive treatment is paramount to prevent neurological sequelae, yet it potentially interferes with the anti-cancer action of CAR T-cells. We describe four CAR T-cells infused patients who presented with reiterative writing behaviours, namely paligraphia, as an early manifestation of neurotoxicity, and eventually developed frontal predominant encephalopathy (one mild, three severe). Paligraphia may represent an early, specific, and easily detectable clinical finding of CAR T-cell therapy-related neurotoxicity, potentially informing its management.
Misinterpretation of glioblastoma as ADEM: potentially harmful consequences of over-diagnosis of COVID-19 vaccine-associated adverse events
C. O´Sullivan, F. Zach, T. Moser, G. Pilz, A. Harrer, E. Trinka, C. Enzinger, J. A. R. Pfaff & P. Wipfler
doi : 10.1007/s00415-021-10707-2
Journal of Neurology volume 269, pages 616–618 (2022)
Prevention of recurrent benign paroxysmal positional vertigo with vitamin D supplementation: a meta-analysis
Seong-Hae Jeong, Sun-Uk Lee & Ji-Soo Kim
doi : 10.1007/s00415-020-09952-8
Journal of Neurology volume 269, pages 619–626 (2022)
Vitamin D insufficiency/deficiency is known to be related to occurrences and recurrences of benign paroxysmal positional vertigo (BPPV). However, the efficacy of vitamin D supplementation in reducing recurrences of BPPV remains to be established. We performed a meta-analysis to determine the therapeutic effects of vitamin D supplementation, with or without calcium, for preventing recurrences of BPPV.
Telerehabilitation in response to constrained physical distance: an opportunity to rethink neurorehabilitative routines
Arturo Nuara, Maddalena Fabbri-Destro, Emilia Scalona, Stefano Elio Lenzi, Giacomo Rizzolatti & Pietro Avanzini
doi : 10.1007/s00415-021-10397-w
Journal of Neurology volume 269, pages 627–638 (2022)
Ensuring proper dosage of treatment and repetition over time is a major challenge in neurorehabilitation. However, a requirement of physical distancing to date compromises their achievement. While mostly associated to COVID-19, physical distancing is not only required in a pandemic scenario, but also advised for several clinical conditions (e.g. immunocompromised individuals) or forced for specific social contexts (e.g. people living in remote areas worldwide). All these contexts advocate for the implementation of alternative healthcare models. The objective of this perspective is to highlight the benefits of remote administration of rehabilitative treatment, namely telerehabilitation, in counteracting physical distancing barriers in neurorehabilitation. Sustaining boosters of treatment outcome, such as compliance, sustainability, as well as motivation, telerehabilitation may adapt to multiple neurological conditions, with the further advantage of a high potential for individualization to patient’s or pathology’s specificities. The effectiveness of telerehabilitation can be potentiated by several technologies available to date: virtual reality can recreate realistic environments in which patients may bodily operate, wearable sensors allow to quantitatively monitor the patient’s performance, and signal processing may contribute to the prediction of long-term dynamics of patient recovery. Telerehabilitation might spark its advantages far beyond the mere limitation of physical distancing effects, mitigating criticalities of daily neurorehabilitative practice, and thus paving the way to the envision of mixed models of care, where hospital-based procedures are complementarily integrated with telerehabilitative ones.
Differentiating dementia with Lewy bodies from Alzheimer's disease and Parkinson's disease dementia: an update on imaging modalities
Rotem Iris Orad & Tamara Shiner
doi : 10.1007/s00415-021-10402-2
Journal of Neurology volume 269, pages 639–653 (2022)
Dementia with Lewy bodies is the second most common cause of neurodegenerative dementia after Alzheimer's disease. Dementia with Lewy bodies can provide a diagnostic challenge due to the frequent overlap of clinical signs with other neurodegenerative conditions, namely Parkinson's disease dementia, and Alzheimer's disease. Part of this clinical overlap is due to the neuropathological overlap. Dementia with Lewy bodies is characterized by the accumulation of aggregated α-synuclein protein in Lewy bodies, similar to Parkinson's disease and Parkinson's disease dementia. However, it is also frequently accompanied by aggregation of amyloid-beta and tau, the pathological hallmarks of Alzheimer's disease. Neuroimaging is central to the diagnostic process. This review is an overview of both established and evolving imaging methods that can improve diagnostic accuracy and improve management of this disorder.
Functional neurological disorder and multiple sclerosis: a systematic review of misdiagnosis and clinical overlap
Dennis Walzl, Andrew J. Solomon & Jon Stone
doi : 10.1007/s00415-021-10436-6
Journal of Neurology volume 269, pages 654–663 (2022)
Multiple sclerosis (MS) and functional neurological disorder (FND) are both diagnostically challenging conditions which can present with similar symptoms. We systematically reviewed the literature to identify patients with MS who were misdiagnosed with FND, patients with FND who were misdiagnosed with MS, and reports of patients with both conditions. In addition to FND, we included studies of patients with other functional and psychiatric disorders where these caused symptoms leading to investigation for or a diagnosis of MS, which in a different context would likely have been labeled as FND. Our review suggests that MS is one of the most common causes of misdiagnosis of FND and vice versa. We discuss the clinical errors that appear to result in misdiagnoses, such as over-reliance on psychiatric comorbidity when making a diagnosis of FND or over-reliance on neuroimaging for the diagnosis of MS, and practical ways to avoid them. Comorbidity between these two conditions is also likely common, has been poorly studied, and adds complexity to diagnosis and treatment in patients with both MS and FND. Misdiagnosis and comorbidity in a landscape of emerging evidence-based treatments for both MS and FND are issues not only of clinical importance to the care of these patients, but also to treatment trials, especially of MS, where FND could be a hidden confounder.
Risk of intracranial hemorrhage with direct oral anticoagulants: a systematic review and meta-analysis of randomized controlled trials
Tingting Wu, Chenyang Lv, Lishui Wu, Wenjun Chen, Meina Lv, Shaojun Jiang & Jinhua Zhang
doi : 10.1007/s00415-021-10448-2
Journal of Neurology volume 269, pages 664–675 (2022)
We performed a systematic review and meta-analysis to compare the risk of intracranial hemorrhage (ICH) between direct oral anticoagulants (DOACs) and other antithrombotic drugs in detail across all diseases.
Genetic counseling and testing practices for late-onset neurodegenerative disease: a systematic review
Ashley Crook, Chris Jacobs, Toby Newton-John, Rosie O’Shea & Alison McEwen
doi : 10.1007/s00415-021-10461-5
Journal of Neurology volume 269, pages 676–692 (2022)
To understand contemporary genetic counseling and testing practices for late-onset neurodegenerative diseases (LONDs), and identify whether practices address the internationally accepted goals of genetic counseling: interpretation, counseling, education, and support.
Coping strategies among amyotrophic lateral sclerosis (ALS) patients: an integrative review
Georgiana Soares Leandro, Mário EmÃlio Teixeira Dourado Júnior, Glauciane Costa Santana & Luan Samy Xavier Dantas
doi : 10.1007/s00415-021-10472-2
Journal of Neurology volume 269, pages 693–702 (2022)
To identify coping strategies used by amyotrophic lateral sclerosis (ALS) patients.
Helicobacter pylori infection is associated with a poor response to levodopa in patients with Parkinson’s disease: a systematic review and meta-analysis
Rui Zhong, Qingling Chen, Xinyue Zhang, Mengmeng Li & Weihong Lin
doi : 10.1007/s00415-021-10473-1
Journal of Neurology volume 269, pages 703–711 (2022)
Helicobacter pylori (HP) infection has been reported to be associated with increased severity of Parkinson's disease (PD) and have negative effects on drug response in patients. We aimed to investigate the influence of HP infection on patients with PD using a systematic review and meta-analysis approach.
Efficacy and safety of intravenous immunoglobulins for the treatment of viral encephalitis: a systematic literature review
Judith N. Wagner, Annette Leibetseder, Anna Troescher, Juergen Panholzer & Tim J. von Oertzen
doi : 10.1007/s00415-021-10494-w
Journal of Neurology volume 269, pages 712–724 (2022)
For most viral encephalitides, therapy is merely supportive. Intravenous immunoglobulins (IVIG) have been used as a prophylactic and therapeutic approach. We conduct a systematic review on the safety and efficacy of IVIG in viral encephalitis.
Is postural orthostatic tachycardia syndrome (POTS) a central nervous system disorder?
Svetlana BlitshteynÂ
doi : 10.1007/s00415-021-10502-z
Journal of Neurology volume 269, pages 725–732 (2022)
Postural orthostatic tachycardia syndrome (POTS), a disorder of the autonomic nervous system characterized by a rise in heart rate of at least 30Â bpm from supine to standing position, has been traditionally viewed as a dysfunction of the peripheral nervous system. However, recent studies and evidence from overlapping conditions suggest that in addition to being considered a disorder of the peripheral nervous system, POTS should be viewed also as a central nervous system (CNS) disorder given (1) significant CNS symptom burden in patients with POTS; (2) structural and functional differences found on neuroimaging in patients with POTS and other forms of orthostatic intolerance; (3) evidence of cerebral hypoperfusion and possible alteration in cerebrospinal fluid volume, and (4) positive response to medications targeting the CNS and non-pharmacologic CNS therapies. This review outlines existing evidence of POTS as a CNS disorder and proposes a hypothetical model combining key mechanisms in the pathophysiology of POTS. Redefining POTS as a CNS disorder can lead to new possibilities in pharmacotherapy and non-pharmacologic therapeutic interventions in patents affected by this disabling syndrome.
Clinical efficacy of the enzyme replacement therapy in patients with late-onset Pompe disease: a systematic review and a meta-analysis
Berli Sarah, Brandi Giovanna, Keller Emanuela, Najia Nadi, Vitale Josè & Pagnamenta Alberto
doi : 10.1007/s00415-021-10526-5
Journal of Neurology volume 269, pages 733–741 (2022)
In patients with late-onset Pompe disease (LOPD), the efficacy of the enzyme replacement therapy (ERT) with recombinant human alpha-glucosidase (rhGAA) is difficult to evaluate, due to the clinical heterogeneity and the small sample sizes in published studies. Therefore, we conduct a systematic literature review and meta-analysis of the literature to evaluate the efficacy of ERT in LOPD patients considering the walking distance, respiratory function and muscle strength. Particularly, six-minute walk test (6MWT), forced vital capacity (FVC), medical research council (MRC) grading, quantitative muscle testing (QMT), and quick motor function test (QMFT) were outcomes of interest. Overall, 619 studies were identified in PubMed, EMBASE and by manual search on July 18th, 2020. After an initial assessment, 16 studies were included in the meta-analysis, containing clinical data from 589 patients with LOPD. For the 6MWT, 419 patients were analyzed. Walking distance improved on average, 32.2 m greater during the observed period (p = 0.0003), compared to the distance at the baseline. The meta-analysis did not show any improvement in FVC and only a tendency towards better muscle strength after treatment with ERT, but the difference was not statistically significant. In conclusion, the available data showed that ERT has a significant beneficial efficacy in the improvement of walking distance in LOPD patients and a non-significant improvement of muscle strength. No improvement in respiratory capacity was found. More prospective and controlled trials are needed to demonstrate a clear clinical benefit of ERT.
A systematic review and meta-analyses on the prevalence of pregnancy outcomes in migraine treated patients: a contribution from the IMI2 ConcePTION project
Daniel C. Dudman, Fatima Tauqeer, Moninder Kaur, Mary E. Ritchey, Hu Li & Sandra Lopez-Leon
doi : 10.1007/s00415-021-10534-5
Journal of Neurology volume 269, pages 742–749 (2022)
The present study aims to summarize the safety profile of the medications used to treat migraine during pregnancy by performing a systematic review and meta-analyses. The term “migrain*� combined with pregnancy terms were used to search Embase, PubMed, PsychInfo, Scopus, and Web of Science through 31 December 2020. Pooled prevalences of untreated and treated migraine patients were estimated using MetaXL software. Pooled odds ratios (OR) using random effects models were estimated in RevMan 5. All the identified studies assessed medications used to treat acute migraine. The pooled prevalence of adverse pregnancy outcomes in patients prescribed any migraine medication ranged from 0.4% (95% CI 0.2–0.7%) for stillbirth to 12.0% (95%CI 7.8–16.9%) for spontaneous abortions. Among untreated patients with migraine, the pooled prevalence of the assessed pregnancy outcomes ranged from 0.6% (95% CI: 0–1.7%) for stillbirth to 10.4% (95% CI: 8.9–12%) for gestational age < 37 weeks. Given the limited data, it was only possible to perform OR meta-analyses for triptans. The adjusted ORs for triptan users compared the general population were: for major malformations 1.07 (95%CI: 0.83–1.39, p = 0.60); birth weight < 2500g 1.18 (95%CI: 0.94–1.48, p = 0.16); gestational age < 37 weeks 1.49 (95% CI: 0.37–6.08, p = 0.58). In conclusion, triptans do not appear to increase the risk of pregnancy outcomes when compared to the general population. It was not possible to assess other migraine medications. Further studies are needed to investigate the safety of individual medications of acute and prophylactic migraine treatment among pregnant women.
Opsoclonus–myoclonus–ataxia syndrome in children
Poonam Bhatia, Jennifer Heim, Patricia Cornejo, Lauren Kane, Jason Santiago & Michael C. Kruer
doi : 10.1007/s00415-021-10536-3
Journal of Neurology volume 269, pages 750–757 (2022)
Opsoclonus–myoclonus–ataxia syndrome is a rare neuroimmunologic disorder typically presenting in previously healthy infants and toddlers. It is characterized by a clinical triad of (1) erratic saccadic intrusions; (2) myoclonus and/or ataxia; (3) behavioral features, typified by developmental plateauing, irritability and insomnia. About half of cases are associated with an underlying neuroblastoma and diagnostic imaging is essential once OMAS is suspected. A thorough workup, including serum, urine, and cerebrospinal fluid studies is critical to identify underlying biomarkers of OMAS itself or neuroblastoma. Historically, many children had relatively poor long-term outcomes, with residual neurologic and/or neuropsychiatry sequelae typical. More recent concepts have emphasized combined immunotherapy regimens that offer hope for better outcomes in children with this remarkable, challenging disease.
Unexpected (123I)FP-CIT SPECT findings: SWIDD, SWEDD and all DAT
Balestrino Roberta, Barone Paolo, Filippi Massimo & Erro Roberto
doi : 10.1007/s00415-021-10809-x
Journal of Neurology volume 269, pages 758–770 (2022)
Although the diagnosis of Parkinson’s disease (PD) is essentially clinical, the implementation of imaging techniques can improve diagnostic accuracy. While some techniques (e.g. magnetic resonance imaging—MRI, computerized tomography—CT) are used to exclude secondary syndromes, presynaptic dopaminergic imaging including imaging of dopamine transporter (DAT)—can help the Neurologist in the differential diagnosis between neurodegenerative parkinsonian syndromes and parkinsonism without dopamine deficiency. DAT imaging can be useful in cases in which the clinical picture is not univocal, as in case of overlapping clinical features in patients with early disease, atypical syndromes or unsatisfying response to therapy. Currently, (123I)FP-CIT ([123I]N-ω-fluoropropyl-2β-carbomethoxy-3β-(4-iodophenyl)nortropane) (trade name DaTSCAN) is the only agent approved by international regulatory agencies for this purpose. With the increasing use of this technique, some unexpected findings have been reported, including patients clinically diagnosed with PD with a normal SPECT scan [e.g. Scans Without Evidence of Dopaminergic Deficit (SWEDD)]; PD patients with a greater dopaminergic deficit in the striatum ipsilateral to the clinically more affected side [e.g. Scans With Ipsilateral Dopaminergic Deficit (SWIDD)]; as well as some artifacts. Moreover, the neurologist must remember that structural lesions and administration of some drugs might alter the result of DAT imaging. Unexpected findings, artifacts, and misinterpretation of imaging findings can lead to an erroneous diagnosis and inappropriate therapy, neglect of other medical conditions that might explain the clinical picture, and undermine the selection phase in clinical trials. The aim of the present review is to bring clarity on these controversial (and sometimes erroneous) results, in order to inform of these possibilities the clinicians requesting a DaTSCAN in clinical practice.
Correction to: Unexpected (123I)FP-CIT SPECT findings: SWIDD, SWEDD and all DAT
Roberta Balestrino, Paolo Barone, Massimo Filippi & Roberto Erro
doi : 10.1007/s00415-021-10923-w
Journal of Neurology volume 269, page 771 (2022)
Safety and outcome of mechanical thrombectomy in ischaemic stroke related to carotid artery dissection
A. Karam, N. Bricout, M. Khyeng, C. Cordonnier, X. Leclerc, H. Henon & B. Casolla
doi : 10.1007/s00415-021-10656-w
Journal of Neurology volume 269, pages 772–779 (2022)
The net clinical benefit of mechanical thrombectomy (MT) in patients with anterior circulation ischaemic stroke associated with large vessel occlusion (AIS-LVO) related to carotid artery dissection (CAD) is uncertain. The aim of the study was to investigate the safety and clinical outcomes of patients treated by MT for a CAD-related stroke.
The impact of device-assisted therapies on the gut microbiome in Parkinson’s disease
Michal Lubomski, Xiangnan Xu, Andrew J. Holmes, Jean Y. H. Yang, Carolyn M. Sue & Ryan L. Davis
doi : 10.1007/s00415-021-10657-9
Journal of Neurology volume 269, pages 780–795 (2022)
Microbiome feedbacks are proposed to influence Parkinson’s disease (PD) pathophysiology. A number of studies have evaluated the impact of oral medication on the gut microbiome (GM) in PD. However, the influence of PD device-assisted therapies (DATs) on the GM remains to be investigated.
What happens after fingolimod discontinuation? A multicentre real-life experience
Doriana Landi, Alessio Signori, Maria Cellerino, Giuseppe Fenu, Carolina Gabri Nicoletti, Marta Ponzano, Elisabetta Mancuso, Marzia Fronza, Maria Elena Ricchiuto, Giacomo Boffa, Matilde Inglese, Girolama Alessandra Marfia, Eleonora Cocco & Jessica Frau
doi : 10.1007/s00415-021-10658-8
Journal of Neurology volume 269, pages 796–804 (2022)
To analyse the course of multiple sclerosis (MS) after fingolimod withdrawal in a multicentre cohort.
Mitochondrial genome variations are associated with amyotrophic lateral sclerosis in patients from mainland China
Jie Ni, Zhen Liu, Yanchun Yuan, Wanzhen Li, Yiting Hu, Pan Liu, Xiaorong Hou, Xiangyu Zhu, Xuxiong Tang, Mingyu liang, Siqi Zheng, Xuan Hou, Juan Du, Jianguang Tang, Hong Jiang, Lu Shen, Beisha Tang & Junling Wang
doi : 10.1007/s00415-021-10659-7
Journal of Neurology volume 269, pages 805–814 (2022)
Amyotrophic lateral sclerosis (ALS) is a late-onset neurodegenerative disorder. Mitochondrial dysfunction is involved in the complex pathophysiology of ALS; however, the role of mitochondrial DNA (mtDNA) variants in ALS is poorly understood. We aimed to elucidate the role of mtDNA variants in the pathogenesis of ALS.
Serum neurofilament light chain or glial fibrillary acidic protein in the diagnosis and prognosis of brain metastases
Xinqing Lin, Tingting Lu, Haiyi Deng, Chunxin Liu, Yilin Yang, Tao Chen, Yinyin Qin, Xiaohong Xie, Zhanhong Xie, Ming Liu, Ming Ouyang, Shiyue Li, Yong Song, Nanshan Zhong, Wei Qiu & Chengzhi Zhou
doi : 10.1007/s00415-021-10660-0
Journal of Neurology volume 269, pages 815–823 (2022)
Brain metastases (BM) remains the most cumbersome disease burden in patients with lung cancer. This study aimed to investigate whether serum brain injury biomarkers can indicate BM, to further establish related diagnostic models, or to predict prognosis of BM.
Safety of alemtuzumab in a nationwide cohort of Finnish multiple sclerosis patients
Ilkka Rauma, Tiina Mustonen, Juha Matti Seppä, Maritta Ukkonen, Marianne Männikkö, Auli Verkkoniemi-Ahola, Marge Kartau, Jukka T. Saarinen, Liisa Luostarinen, Sakari Simula, Mervi Ryytty, Riitta Ahmasalo, Jussi O. T. Sipilä, Ilkka Pieninkeroinen, Tero Tapiola, Anne M. Remes & Hanna Kuusisto
doi : 10.1007/s00415-021-10664-w
Journal of Neurology volume 269, pages 824–835 (2022)
Alemtuzumab is an effective disease-modifying therapy (DMT) for highly active multiple sclerosis (MS). However, safety concerns limit its use in clinical practice.
NMOSD and MS prevalence in the Indigenous populations of Australia and New Zealand
Wajih Bukhari, Elham Khalilidehkordi, Deborah F. Mason, Michael H. Barnett, Bruce V. Taylor, Marzena Fabis-Pedrini, Allan G. Kermode, Sankar Subramanian, Patrick Waters, Simon A. Broadley on behalf of The Australian and New Zealand NMO Collaboration
doi : 10.1007/s00415-021-10665-9
Journal of Neurology volume 269, pages 836–845 (2022)
We studied the prevalence of neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) in Indigenous populations of Australia and New Zealand with the aim of assessing potential differences.
Optimizing the selection of Parkinson’s disease patients for neuromodulation using the levodopa challenge test
Dinkar Kulshreshtha, Marcus Pieterman, Greydon Gilmore & Mandar Jog
doi : 10.1007/s00415-021-10666-8
Journal of Neurology volume 269, pages 846–852 (2022)
In Parkinson’s disease (PD), early stages are associated with a good long-duration response and as the disease advances, the short-duration response predominates. The transition between the long-duration and short-duration responses may be an important and measurable intermediate stage. A critical criterion in determining the candidature for neuromodulation is a beneficial response to an ‘off–on’ levodopa challenge test. This test is usually reserved for those that have already developed marked short-duration response and are candidates for deep brain stimulation (DBS) surgery. However, identifying those that are in transition may allow DBS to be offered earlier.
Paradigm shift in acute dizziness: is caloric testing obsolete?
Miranda Morrison, Athanasia Korda, Ewa Zamaro, Franca Wagner, Marco D. Caversaccio, Thomas C. Sauter, Roger Kalla & Georgios Mantokoudis
doi : 10.1007/s00415-021-10667-7
Journal of Neurology volume 269, pages 853–860 (2022)
Cold and warm water ear irrigation, also known as bithermal caloric testing, has been considered for over 100 years the ‘Gold Standard’ for the detection of peripheral vestibular hypofunction. Its discovery was awarded a Nobel Prize. We aimed to investigate the diagnostic accuracy of Caloric Testing when compared to the video head impulse test (vHIT) in differentiating between vestibular neuritis and vestibular strokes in acute dizziness.
Prediagnosis epilepsy and survival in patients with glioma: a nationwide population-based cohort study from 2009 to 2018
Mirketa Marku, Birthe Krogh Rasmussen, Federica Belmonte, Steinbjørn Hansen, Elisabeth Anne Wreford Andersen, Christoffer Johansen & Pernille Envold Bidstrup
doi : 10.1007/s00415-021-10668-6
Journal of Neurology volume 269, pages 861–872 (2022)
Considering that epilepsy is common, and knowledge is lacking on its role especially for the prognosis of high-grade gliomas, the objective of this study was to investigate the association between epilepsy prior to glioma diagnosis and survival among glioma patients.
Traumatic brain injury fast-forwards Alzheimer’s pathology: evidence from amyloid positron emission tomorgraphy imaging
Abdalla Z. Mohamed, Peter J. Nestor, Paul Cumming & Fatima A. Nasrallah for the Alzheimer’s Disease Neuroimaging Initiative
doi : 10.1007/s00415-021-10669-5
Journal of Neurology volume 269, pages 873–884 (2022)
Traumatic brain injury (TBI) has been proposed as a risk factor for Alzheimer’s disease (AD), although the mechanisms underlying the putative association are poorly understood. We investigated elderly individuals with a remote history of TBI, aiming to understand how this may have influenced amyloidosis, neurodegeneration, and clinical expression along the AD continuum.
Randomized phase 2 study of perampanel for sporadic amyotrophic lateral sclerosis
Hitoshi Aizawa, Haruhisa Kato, Koji Oba, Takuya Kawahara, Yoshihiko Okubo, Tomoko Saito, Makiko Naito, Makoto Urushitani, Akira Tamaoka, Kiyotaka Nakamagoe, Kazuhiro Ishii, Takashi Kanda, Masahisa Katsuno, Naoki Atsuta, Yasushi Maeda, Makiko Nagai, Kazutoshi Nishiyama, Hiroyuki Ishiura, Tatsushi Toda, Akihiro Kawata, Koji Abe, Ichiro Yabe, Ikuko Takahashi-Iwata, Hidenao Sasaki, Hitoshi Warita, Masashi Aoki, Gen Sobue, Hidehiro Mizusawa, Yutaka Matsuyama, Tomohiro Haga & Shin Kwak
doi : 10.1007/s00415-021-10670-y
Journal of Neurology volume 269, pages 885–896 (2022)
To evaluate the efficacy and safety of perampanel in patients with sporadic amyotrophic lateral sclerosis (SALS).
Shorter visual aura characterizes young and middle-aged stroke patients with migraine with aura
Claudia Altamura, Angelo Cascio Rizzo, Giovanna Viticchi, Paola Maggio, Carmelina Maria Costa, Nicoletta Brunelli, Giuditta Giussani, Matteo Paolucci, Fabrizio Fiacco, Vincenzo Di Lazzaro, Elio Clemente Agostoni, Mauro Silvestrini & Fabrizio Vernieri
doi : 10.1007/s00415-021-10671-x
Journal of Neurology volume 269, pages 897–906 (2022)
To identify the clinical profile and aura characteristics of patients with Migraine with Aura (MwA) having acute cerebral ischemia, we compared stroke phenotype and risk factors in stroke patients with (S+MwA+) or without (S+MwA−) MwA and aura features in MwA patients with (S+MwA+) or without (S−MwA+) stroke.
Prolonged distal motor latency of median nerve does not improve diagnostic accuracy for CIDP
Emanuele Spina, Pietro Emiliano Doneddu, Giuseppe Liberatore, Dario Cocito, Raffaella Fazio, Chiara Briani, Massimiliano Filosto, Luana Benedetti, Giovanni Antonini, Giuseppe Cosentino, Stefano Jann, Anna Mazzeo, Andrea Cortese, Girolama Alessandra Marfia, Angelo Maurizio Clerici, Gabriele Siciliano, Marinella Carpo, Marco Luigetti, Giuseppe Lauria, Tiziana Rosso, Guido Cavaletti, Erdita Peci, Stefano Tronci, Marta Ruiz, Stefano Cotti Piccinelli, Angelo Schenone, Luca Leonardi, Luca Gentile, Laura Piccolo, Giorgia Mataluni, Lucio Santoro, Eduardo Nobile-Orazio, Fiore Manganelli on behalf of The Italian CIDP Database Study Group
doi : 10.1007/s00415-021-10672-w
Journal of Neurology volume 269, pages 907–912 (2022)
Compression of the median nerve at the carpal tunnel can give demyelinating features and result in distal motor latency (DML) prolongation fulfilling the EFNS/PNS demyelinating criteria for chronic inflammatory demyelinating polyneuropathy (CIDP). Accordingly, being carpal tunnel syndrome (CTS) common in the general population, the EFNS/PNS guidelines recommend excluding the DML of the median nerve when DML prolongation may be consistent with median neuropathy at the wrist from CTS. The main aims of this study were to verify whether the inclusion of DML of the median nerve (when consistent with CTS) could improve electrophysiological diagnostic accuracy for CIDP and if the median nerve at the carpal tunnel was more prone to demyelination. We analyzed electrophysiological data from 499 patients included consecutively into the Italian CIDP Database. According to the EFNS/PNS criteria, 352 patients had a definite, 10 a probable, and 57 a possible diagnosis of CIDP, while 80 were not fulfilling the diagnostic criteria. The inclusion of DML prolongation of median nerve did not improve significantly the diagnostic accuracy for CIDP; overall diagnostic class changed in 6 out of 499 patients (1.2%) and electrodiagnostic class of CIDP changed from not fulfilling to possible in only 2 patients (2.5% of not-fulfilling patients). In conclusion, we can infer that excluding DML prolongation of median nerve does not increase the risk of missing a diagnosis of CIDP thus corroborating the current EFNS/PNS criteria.
Initial treatment strategy and clinical outcomes in Finnish MS patients: a propensity-matched study
K. Hänninen, M. Viitala, S. Atula, S. M. Laakso, H. Kuusisto & M. Soilu-Hänninen
doi : 10.1007/s00415-021-10673-9
Journal of Neurology volume 269, pages 913–922 (2022)
The optimal treatment strategy with disease-modifying therapies (DMTs) in relapsing–remitting multiple sclerosis (RRMS) remains uncertain.
Quantitative sensory testing and norepinephrine levels in REM sleep behaviour disorder – a clue to early peripheral autonomic and sensory dysfunction?
Julia Koch, Kira Willemsen, Imis Dogan, Roman Rolke, Jörg B. Schulz, Johannes Schiefer, Kathrin Reetz & Andrea Maier
doi : 10.1007/s00415-021-10675-7
Journal of Neurology volume 269, pages 923–932 (2022)
Studies have reported autonomic impairment in patients with idiopathic REM sleep behaviour disorder (iRBD), which is considered a prodromal stage of alpha-synucleinopathies. It is still debated whether central or peripheral pathologies are first manifestations of alpha-synucleinopathies. This study aimed to characterize autonomic and somatosensory function in iRBD patients.
PML risk is the main factor driving the choice of discontinuing natalizumab in a large multiple sclerosis population: results from an Italian multicenter retrospective study
Clara G. Chisari, Giancarlo Comi, Massimo Filippi, Damiano Paolicelli, Pietro Iaffaldano, Mauro Zaffaroni, Vincenzo Brescia Morra, Eleonora Cocco, Girolama Alessandra Marfia, Luigi Maria Grimaldi, Matilde Inglese, Simona Bonavita, Alessandra Lugaresi, Giuseppe Salemi, Giovanna De Luca, Salvatore Cottone, Antonella Conte, Patrizia Sola, Umberto Aguglia, Giorgia Teresa Maniscalco, Claudio Gasperini, Maria Teresa Ferrò, Ilaria Pesci, Maria Pia Amato, Marco Rovaris, Claudio Solaro, Giacomo Lus, Davide Maimone, Roberto Bergamaschi, Franco Granella, Alessia Di Sapio, Antonio Bertolotto, Rocco Totaro, Marika Vianello, Paola Cavalla, Paolo Bellantonio, Vito Lepore, Francesco Patti & the Italian MS Register Study Group.
doi : 10.1007/s00415-021-10676-6
Journal of Neurology volume 269, pages 933–944 (2022)
Natalizumab (NTZ) is an effective treatment for relapsing–remitting multiple sclerosis (RRMS). However, patients and physicians may consider discontinuing NTZ therapy due to safety or efficacy issues. The aim of our study was to evaluate the NTZ discontinuation rate and reasons of discontinuation in a large Italian population of RRMS patients.
Clinical outcome of CIDP one year after start of treatment: a prospective cohort study
S. R. M. Bus, M. C. Broers, I. M. Lucke, C. Bunschoten, G. G. A. van Lieverloo, M. E. Adrichem, R. van Veen, L. Wieske, H. F. Lingsma, H. S. Goedee, W. L. van der Pol, I. N. van Schaik, P. A. Van Doorn, B. C. Jacobs & F. Eftimov for the ICOS Consortium
doi : 10.1007/s00415-021-10677-5
Journal of Neurology volume 269, pages 945–955 (2022)
To assess clinical outcome in treatment-naive patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
Initiation of antidepressants in young adults after ischemic stroke: a registry-based follow-up study
Jenna Broman, Karoliina Aarnio, Anna But, Ivan Marinkovic, Jorge RodrÃguez-Pardo, Markku Kaste, Turgut Tatlisumak & Jukka Putaala
doi : 10.1007/s00415-021-10678-4
Journal of Neurology volume 269, pages 956–965 (2022)
Data on post-stroke use of antidepressants in young individuals are scarce. We examined pattern and factors associated with initiating post-stroke antidepressants (PSAD) after ischemic stroke (IS) in young adults.
Cystatin C predicts futile recanalization in patients with acute ischemic stroke after endovascular treatment
Mouxiao Su, Ying Zhou, Zhonglun Chen, Mingjun Pu, Zhaokun Li, Hongcai Du & Gelin Xu
doi : 10.1007/s00415-021-10680-w
Journal of Neurology volume 269, pages 966–972 (2022)
A previous study reported that cystatin C was related to acute ischemic stroke. The association between cystatin C and the clinical outcome in acute ischaemic stroke patients with successful recanalization after endovascular thrombectomy has rarely been reported. This study aimed to evaluate the association between cystatin C and futile recanalization in AIS patients who underwent endovascular thrombectomy.
Pembrolizumab for treatment of progressive multifocal leukoencephalopathy in primary immunodeficiency and/or hematologic malignancy: a case series of five patients
Timo Volk, Klaus Warnatz, Reinhard Marks, Horst Urbach, Gisela Schluh, Valentina Strohmeier, Jessica Rojas-Restrepo, Bodo Grimbacher & Sebastian Rauer
doi : 10.1007/s00415-021-10682-8
Journal of Neurology volume 269, pages 973–981 (2022)
Progressive multifocal leukoencephalopathy is a rare opportunistic infection of the brain by John Cunningham polyomavirus in immune-compromised patients. In cases where no overt option for immune reconstitution is available [e.g., in patients with primary immunodeficiency (PID)], the disease is lethal in the majority of patients. Immune checkpoint inhibition has been applied in recent years with mixed outcomes. We present four novel patients and the follow-up of a previously published patient suffering from progressive multifocal leukoencephalopathy (PML) due to PID and/or hematologic malignancy who were treated with the immune checkpoint inhibitor pembrolizumab. In two patients with PID, symptoms improved and stabilized. One patient died because of worsening PML another of intracranial hemorrhage which was unrelated to PML or its treatment with pembrolizumab. The fifth patient suffered from PID and died of a pre-existing immune dysregulation, possibly exacerbated by pembrolizumab. The long-term follow-up of the first patient provides support for therapeutic decisions during this therapy and is the longest published clinical course of a patient with checkpoint inhibition for PML. We conclude that pembrolizumab can control PML symptoms long term in a subgroup of patients with PID, in our cases for 21 and 36Â months. However, therapy must be started early because symptoms are only partially reversible. In light of severe adverse events, application of pembrolizumab is only justified if the prognosis for the individual patient is very poor.
Diagnosis and follow-up evaluation of central nervous system vasculitis: an evaluation of vessel-wall MRI findings
Maximilian Patzig, Robert Forbrig, Clemens Küpper, Ozan Eren, Tobias Saam, Lars Kellert, Thomas Liebig & Florian Schöberl
doi : 10.1007/s00415-021-10683-7
Journal of Neurology volume 269, pages 982–996 (2022)
To approach the clinical value of MRI with vessel wall imaging (VWI) in patients with central nervous system vasculitis (CNSV), we analyzed patterns of VWI findings both at the time of initial presentation and during follow-up.
The angiographic presentation of European Moyamoya angiopathy
Sara Pilgram-Pastor, René Chapot & Markus Kraemer
doi : 10.1007/s00415-021-10684-6
Journal of Neurology volume 269, pages 997–1006 (2022)
Little is known about the angiographic presentation of Moyamoya angiopathy (MMA) in non-Asian patients.
Blink reflex recovery cycle distinguishes patients with idiopathic normal pressure hydrocephalus from elderly subjects
Alessandro Mechelli, Andrea Quattrone, Rita Nisticò, Marianna Crasà , Domenico La Torre, Basilio Vescio & Aldo Quattrone
doi : 10.1007/s00415-021-10687-3
Journal of Neurology volume 269, pages 1007–1012 (2022)
The R2 component of blink reflex recovery cycle (R2BRrc) is a simple neurophysiological tool to detect the brainstem hyperexcitability commonly occurring in several neurological diseases such as Parkinson’s disease and atypical parkinsonisms. In our study, we investigated for the first time the usefulness of R2BRrc to assess brainstem excitability in patients with idiopathic Normal Pressure Hydrocephalus (iNPH) in comparison with healthy subjects.
Association of the Careggi Collateral Score with 3-month modified Rankin Scale score after thrombectomy for stroke with occlusion of the middle cerebral artery
Manuel Cappellari, Valentina Saia, Giovanni Pracucci, Fainardi Enrico, Arturo Consoli, Sergio Nappini, Lucio Castellan, Sandra Bracco, Mauro Bergui, Mirco Cosottini, Alessandra Briatico Vangosa, Sergio Vinci, Maria Ruggiero, Edoardo Puglielli, Luigi Chiumarulo, Giacomo Cester, Chiara Comelli, Umberto Silvagni, Daniele Morosetti, Valentina Caldiera, Nicola Cavasin, Valeria Ledda, Giuseppina Sanfilippo, Andrea Saletti, Pietro Filauri, Ivan Gallesio, Nunzio Paolo Nuzzi, Pitero Amistá, Cecilia Zivelonghi, Mauro Plebani, Marco Pavia, Daniele Romano, Francesco Biraschi, Roberto Menozzi, Roberto Gasparotti, Andrea Giorgianni, Andrea Zini, Domenico Inzitari, Danilo Toni, Salvatore Mangiafico on behalf of The IRETAS Group
doi : 10.1007/s00415-021-10898-8
Journal of Neurology volume 269, pages 1013–1023 (2022)
The Careggi Collateral Score (CCS) (qualitative–quantitative evaluation) was developed from a single-centre cohort as an angiographic score to describe both the extension and effectiveness of the pial collateral circulation in stroke patients with occlusion of the anterior circulation. We aimed to examine the association between CCS (quantitative evaluation) and 3-month modified Rankin Scale (mRS) score in a large multi-center cohort of patients receiving thrombectomy for stroke with occlusion of middle cerebral artery (MCA).
Correction to: Association of the Careggi Collateral Score with 3-month modified Rankin Scale score after thrombectomy for stroke with occlusion of the middle cerebral artery
Manuel Cappellari, Valentina Saia, Giovanni Pracucci, Enrico Fainardi, Arturo Consoli, Sergio Nappini, Lucio Castellan, Sandra Bracco, Mauro Bergui, Mirco Cosottini, Alessandra Briatico Vangosa, Sergio Vinci, Maria Ruggiero, Edoardo Puglielli, Luigi Chiumarulo, Giacomo Cester, Chiara Comelli, Umberto Silvagni, Daniele Morosetti, Valentina Caldiera, Nicola Cavasin, Valeria Ledda, Giuseppina Sanfilippo, Andrea Saletti, Pietro Filauri, Ivan Gallesio, Nunzio Paolo Nuzzi, Pitero Amistá, Cecilia Zivelonghi, Mauro Plebani, Marco Pavia, Daniele Romano, Francesco Biraschi, Roberto Menozzi, Roberto Gasparotti, Andrea Giorgianni, Andrea Zini, Domenico Inzitari, Danilo Toni, Salvatore Mangiafico on behalf of The IRETAS Group
doi : 10.1007/s00415-021-10941-8
Journal of Neurology volume 269, pages 1024–1025 (2022)
Ocular flutter is a rare and treatment-responsive symptom in Lyme neuroborreliosis: a case report
Fiona Jäger, Stefan Greisenegger, Felix K. Schwarz & Gerald Wiest
doi : 10.1007/s00415-021-10769-2
Journal of Neurology volume 269, pages 1026–1027 (2022)
Paroxysmal central positional nystagmus responsive to clonazepam
Eun Hye Oh, Hyunsung Kim, Seo-Young Choi, Kwang-Dong Choi & Jae-Hwan Choi
doi : 10.1007/s00415-021-10770-9
Journal of Neurology volume 269, pages 1028–1031 (2022)
When should we consider chronic patients as non-responders to monoclonal antibodies targeting the CGRP pathway?
Claudia Altamura, Sabina Cevoli, Nicoletta Brunelli, Cinzia Aurilia, Luisa Fofi, Gabriella Egeo, Piero Barbanti, Fabrizio Vernieri
doi : 10.1007/s00415-021-10772-7
Journal of Neurology volume 269, pages 1032–1034 (2022)
Pathogenic events in very early Guillain–Barré syndrome: neither demyelination nor axonal degeneration but endoneurial inflammatory oedema
José BercianoÂ
doi : 10.1007/s00415-021-10773-6
Journal of Neurology volume 269, pages 1035–1037 (2022)
Alzheimer’s disease clinical trial update 2019–2021
Joseph Pleen & Ryan Townley
doi : 10.1007/s00415-021-10790-5
Journal of Neurology volume 269, pages 1038–1051 (2022)
The current clinical trial landscape targeting Alzheimer’s disease (AD) is reviewed in the context of studies completed from 2019 to 2021. This review focuses on available data for observational and phase II/III clinical trial results, which will have the most impact on the field. ClinicalTrials.gov, the United States (US) comprehensive federal registry, was queried to identify completed trials. There are currently 226 interventional clinical trials and 51 observational studies completed, suspended, terminated, or withdrawn within our selected time frame. This review reveals that the role of biomarkers is expanding and although many lessons have been learned, many challenges remain when targeting disease modification of AD through amyloid and tau. In addition, to halt or slow clinical progression of AD, new clinical and observational trials are focusing on prevention as well as the role of more diverse biological processes known to influence AD pathology.
Effects on cognition of DMTs in multiple sclerosis: moving beyond the prevention of inflammatory activity
Paolo Preziosa, Lorenzo Conti, Maria A. Rocca & Massimo Filippi
doi : 10.1007/s00415-021-10832-y
Journal of Neurology volume 269, pages 1052–1064 (2022)
In this review, we critically summarize recent findings derived from randomized controlled trials (RCTs), observational studies and meta-analyses that have been published in the last 3Â years and that included the effects of DMTs on cognitive performances among their outcomes.
Advances in the use of stem cell transplants in the treatment of multiple sclerosis
Rachel Thomas, Ray Wynford-Thomas & Neil P. Robertson
doi : 10.1007/s00415-021-10927-6
Journal of Neurology volume 269, pages 1065–1067 (2022)
Carlo Francesco Giuseppe Bellingeri (1785–1843)
Lorenzo Lorusso, Stefano Zago & Stefano Sandrone
doi : 10.1007/s00415-021-10732-1
Journal of Neurology volume 269, pages 1068–1069 (2022)
Association of CNS demyelination and COVID-19 infection: an updated systematic review
Ismail Ibrahim Ismail & Sara Salama
doi : 10.1007/s00415-021-10752-x
Journal of Neurology volume 269, pages 541–576 (2022)
Since the declaration of COVID-19 pandemic, several case reports of demyelination of both peripheral and central nervous systems have been published. The association between CNS demyelination and viral infection has long been documented, and this link was recently reported following SARS-CoV-2 infection as well.
A rise in cases of nitrous oxide abuse: neurological complications and biological findings
Maximilian Einsiedler, Paul Voulleminot, Stanislas Demuth, Pauline Kalaaji, Thomas Bogdan, Lucas Gauer, Cécile Reschwein, Aleksandra Nadaj-Pakleza, Jérôme de Sèze, Laurent Kremer, Ivana Schroder & Kévin Bigaut
doi : 10.1007/s00415-021-10702-7
Journal of Neurology volume 269, pages 577–582 (2022)
The recent lockdown due to the COVID-19 pandemic has been linked to a higher incidence of psychiatric manifestations and substance abuse. The recreative use of nitrous oxide is more and more widespread and neurological complications are frequent.
Case report: cerebral sinus vein thrombosis in two patients with AstraZeneca SARS-CoV-2 vaccination
Mathias Fousse, David Schub, Fatma Merzou, Klaus Fassbender, Martina Sester, Michael Kettner, Piergiorgio Lochner, Tina Schmidt & João Reinoldo Goi Júnior
doi : 10.1007/s00415-021-10731-2
Journal of Neurology volume 269, pages 583–586 (2022)
SARS-CoV-2 infection is associated with an increased rate of thromboembolic events and mortality. Different vaccines are globally used to limit the pandemic. In this report, we present the case of two young female patients with newly diagnosed cerebral sinus vein thrombosis occurring after injection of the vector-based ChAdOx1 vaccine. Both patients presented with unusual headache only. The two of them used an estrogen-containing contraception, had had a history of deep venous thrombosis, and both had MTHFR mutations. Both patients developed SARS-CoV-2 specific humoral and cellular immunity including both CD4 and CD8 T cells. This rare, but serious complication needs to be considered after vaccination of young females, even if there is no evidence of heparin-induced thrombocytopenia.
Autonomic dysfunction in post-COVID patients with and witfhout neurological symptoms: a prospective multidomain observational study
Alex Buoite Stella, Giovanni Furlanis, Nicolò Arjuna Frezza, Romina Valentinotti, Milos Ajcevic & Paolo Manganotti
doi : 10.1007/s00415-021-10735-y
Journal of Neurology volume 269, pages 587–596 (2022)
The autonomic nervous system (ANS) can be affected by COVID-19, and dysautonomia may be a possible complication in post-COVID individuals. Orthostatic hypotension (OH) and postural tachycardia syndrome (POTS) have been suggested to be common after SARS-CoV-2 infection, but other components of ANS function may be also impaired. The Composite Autonomic Symptom Scale 31 (COMPASS-31) questionnaire is a simple and validated tool to assess dysautonomic symptoms. The aim of the present study was to administer the COMPASS-31 questionnaire to a sample of post-COVID patients with and without neurological complaints. Participants were recruited among the post-COVID ambulatory services for follow-up evaluation between 4 weeks and 9 months from COVID-19 symptoms onset. Participants were asked to complete the COMPASS-31 questionnaire referring to the period after COVID-19 disease. Heart rate and blood pressure were manually taken during an active stand test for OH and POTS diagnosis. One-hundred and eighty participants were included in the analysis (70.6% females, 51 ± 13 years), and OH was found in 13.8% of the subjects. Median COMPASS-31 score was 17.6 (6.9–31.4), with the most affected domains being orthostatic intolerance, sudomotor, gastrointestinal and pupillomotor dysfunction. A higher COMPASS-31 score was found in those with neurological symptoms (p < 0.01), due to more severe orthostatic intolerance symptoms (p < 0.01), although gastrointestinal (p < 0.01), urinary (p < 0.01), and pupillomotor (p < 0.01) domains were more represented in the non-neurological symptoms group. This study confirms the importance of monitoring ANS symptoms as a possible complication of COVID-19 disease that may persist in the post-acute period.
Decrease in intravenous thrombolysis and poor short-term functional prognosis for acute ischemic stroke during the COVID-19 pandemic
Xueqian Xu, Yuenan Xiao, Jia Li, Liuzhu Chen, Gangqiang Lin, Ledan Dong, Yisi Lin, Luqian Zhan, Jincai He & Xiaoqian Luan
doi : 10.1007/s00415-021-10740-1
Journal of Neurology volume 269, pages 597–602 (2022)
Since the outbreak of the coronavirus pandemic in 2019 (COVID-19), healthcare systems around the world have been hit to varying degrees. As a neurologist team, for patients with acute ischemic stroke (AIS), we compared the situations of intravenous thrombolysis (IVT) treatment from 2019 to 2020 to investigate the influence of COVID-19 pandemic on the attendance and prognosis of the IVT patients.
Delays in thrombolysis during COVID-19 are associated with worse neurological outcomes: the Society of Vascular and Interventional Neurology Multicenter Collaboration
Dinesh V. Jillella, Fadi Nahab, Thanh N. Nguyen, Mohamad Abdalkader, David S. Liebeskind, Nirav Vora, Vivek Rai, Diogo C. Haussen, Raul G. Nogueira, Shashvat Desai, Ashutosh P. Jadhav, Alexandra L. Czap, Alicia M. Zha, Italo Linfante, Ameer E Hassan, Darko Quispe-Orozco, Santiago Ortega-Gutierrez, Priyank Khandelwal, Pratit Patel, Osama Zaidat, Tudor G. Jovin, Scott Kamen & James E. Siegler
doi : 10.1007/s00415-021-10734-z
Journal of Neurology volume 269, pages 603–608 (2022)
We have demonstrated in a multicenter cohort that the COVID-19 pandemic has led to a delay in intravenous thrombolysis (IVT) among stroke patients. Whether this delay contributes to meaningful short-term outcome differences in these patients warranted further exploration.
Frontal predominant encephalopathy with early paligraphia as a distinctive signature of CAR T-cell therapy-related neurotoxicity
Umberto Pensato, Giulia Amore, Roberto D’Angelo, Rita Rinaldi, Marianna Nicodemo, Francesca Rondelli, Susanna Mondini, Rossella Santoro, Susanna Sammali, Andrea Farolfi, Luca Spinardi, Luca Faccioli, Beatrice Casadei, Michele Dicataldo, Francesca Bonifazi, Pierluigi Zinzani, Pietro Cortelli, Andrea Stracciari & Maria Guarino
doi : 10.1007/s00415-021-10766-5
Journal of Neurology volume 269, pages 609–615 (2022)
Chimeric antigen receptor (CAR) T-cell therapy is an emerging highly effective treatment for refractory haematological malignancies. Unfortunately, its therapeutic benefit may be hampered by treatment-related toxicities, including neurotoxicity. Early aggressive treatment is paramount to prevent neurological sequelae, yet it potentially interferes with the anti-cancer action of CAR T-cells. We describe four CAR T-cells infused patients who presented with reiterative writing behaviours, namely paligraphia, as an early manifestation of neurotoxicity, and eventually developed frontal predominant encephalopathy (one mild, three severe). Paligraphia may represent an early, specific, and easily detectable clinical finding of CAR T-cell therapy-related neurotoxicity, potentially informing its management.
Misinterpretation of glioblastoma as ADEM: potentially harmful consequences of over-diagnosis of COVID-19 vaccine-associated adverse events
C. O´Sullivan, F. Zach, T. Moser, G. Pilz, A. Harrer, E. Trinka, C. Enzinger, J. A. R. Pfaff & P. Wipfler
doi : 10.1007/s00415-021-10707-2
Journal of Neurology volume 269, pages 616–618 (2022)
Prevention of recurrent benign paroxysmal positional vertigo with vitamin D supplementation: a meta-analysis
Seong-Hae Jeong, Sun-Uk Lee & Ji-Soo Kim
doi : 10.1007/s00415-020-09952-8
Journal of Neurology volume 269, pages 619–626 (2022)
Vitamin D insufficiency/deficiency is known to be related to occurrences and recurrences of benign paroxysmal positional vertigo (BPPV). However, the efficacy of vitamin D supplementation in reducing recurrences of BPPV remains to be established. We performed a meta-analysis to determine the therapeutic effects of vitamin D supplementation, with or without calcium, for preventing recurrences of BPPV.
Telerehabilitation in response to constrained physical distance: an opportunity to rethink neurorehabilitative routines
Arturo Nuara, Maddalena Fabbri-Destro, Emilia Scalona, Stefano Elio Lenzi, Giacomo Rizzolatti & Pietro Avanzini
doi : 10.1007/s00415-021-10397-w
Journal of Neurology volume 269, pages 627–638 (2022)
Ensuring proper dosage of treatment and repetition over time is a major challenge in neurorehabilitation. However, a requirement of physical distancing to date compromises their achievement. While mostly associated to COVID-19, physical distancing is not only required in a pandemic scenario, but also advised for several clinical conditions (e.g. immunocompromised individuals) or forced for specific social contexts (e.g. people living in remote areas worldwide). All these contexts advocate for the implementation of alternative healthcare models. The objective of this perspective is to highlight the benefits of remote administration of rehabilitative treatment, namely telerehabilitation, in counteracting physical distancing barriers in neurorehabilitation. Sustaining boosters of treatment outcome, such as compliance, sustainability, as well as motivation, telerehabilitation may adapt to multiple neurological conditions, with the further advantage of a high potential for individualization to patient’s or pathology’s specificities. The effectiveness of telerehabilitation can be potentiated by several technologies available to date: virtual reality can recreate realistic environments in which patients may bodily operate, wearable sensors allow to quantitatively monitor the patient’s performance, and signal processing may contribute to the prediction of long-term dynamics of patient recovery. Telerehabilitation might spark its advantages far beyond the mere limitation of physical distancing effects, mitigating criticalities of daily neurorehabilitative practice, and thus paving the way to the envision of mixed models of care, where hospital-based procedures are complementarily integrated with telerehabilitative ones.
Differentiating dementia with Lewy bodies from Alzheimer's disease and Parkinson's disease dementia: an update on imaging modalities
Rotem Iris Orad & Tamara Shiner
doi : 10.1007/s00415-021-10402-2
Journal of Neurology volume 269, pages 639–653 (2022)
Dementia with Lewy bodies is the second most common cause of neurodegenerative dementia after Alzheimer's disease. Dementia with Lewy bodies can provide a diagnostic challenge due to the frequent overlap of clinical signs with other neurodegenerative conditions, namely Parkinson's disease dementia, and Alzheimer's disease. Part of this clinical overlap is due to the neuropathological overlap. Dementia with Lewy bodies is characterized by the accumulation of aggregated α-synuclein protein in Lewy bodies, similar to Parkinson's disease and Parkinson's disease dementia. However, it is also frequently accompanied by aggregation of amyloid-beta and tau, the pathological hallmarks of Alzheimer's disease. Neuroimaging is central to the diagnostic process. This review is an overview of both established and evolving imaging methods that can improve diagnostic accuracy and improve management of this disorder.
Functional neurological disorder and multiple sclerosis: a systematic review of misdiagnosis and clinical overlap
Dennis Walzl, Andrew J. Solomon & Jon Stone
doi : 10.1007/s00415-021-10436-6
Journal of Neurology volume 269, pages 654–663 (2022)
Multiple sclerosis (MS) and functional neurological disorder (FND) are both diagnostically challenging conditions which can present with similar symptoms. We systematically reviewed the literature to identify patients with MS who were misdiagnosed with FND, patients with FND who were misdiagnosed with MS, and reports of patients with both conditions. In addition to FND, we included studies of patients with other functional and psychiatric disorders where these caused symptoms leading to investigation for or a diagnosis of MS, which in a different context would likely have been labeled as FND. Our review suggests that MS is one of the most common causes of misdiagnosis of FND and vice versa. We discuss the clinical errors that appear to result in misdiagnoses, such as over-reliance on psychiatric comorbidity when making a diagnosis of FND or over-reliance on neuroimaging for the diagnosis of MS, and practical ways to avoid them. Comorbidity between these two conditions is also likely common, has been poorly studied, and adds complexity to diagnosis and treatment in patients with both MS and FND. Misdiagnosis and comorbidity in a landscape of emerging evidence-based treatments for both MS and FND are issues not only of clinical importance to the care of these patients, but also to treatment trials, especially of MS, where FND could be a hidden confounder.
Risk of intracranial hemorrhage with direct oral anticoagulants: a systematic review and meta-analysis of randomized controlled trials
Tingting Wu, Chenyang Lv, Lishui Wu, Wenjun Chen, Meina Lv, Shaojun Jiang & Jinhua Zhang
doi : 10.1007/s00415-021-10448-2
Journal of Neurology volume 269, pages 664–675 (2022)
We performed a systematic review and meta-analysis to compare the risk of intracranial hemorrhage (ICH) between direct oral anticoagulants (DOACs) and other antithrombotic drugs in detail across all diseases.
Genetic counseling and testing practices for late-onset neurodegenerative disease: a systematic review
Ashley Crook, Chris Jacobs, Toby Newton-John, Rosie O’Shea & Alison McEwen
doi : 10.1007/s00415-021-10461-5
Journal of Neurology volume 269, pages 676–692 (2022)
To understand contemporary genetic counseling and testing practices for late-onset neurodegenerative diseases (LONDs), and identify whether practices address the internationally accepted goals of genetic counseling: interpretation, counseling, education, and support.
Coping strategies among amyotrophic lateral sclerosis (ALS) patients: an integrative review
Georgiana Soares Leandro, Mário EmÃlio Teixeira Dourado Júnior, Glauciane Costa Santana & Luan Samy Xavier Dantas
doi : 10.1007/s00415-021-10472-2
Journal of Neurology volume 269, pages 693–702 (2022)
To identify coping strategies used by amyotrophic lateral sclerosis (ALS) patients.
Helicobacter pylori infection is associated with a poor response to levodopa in patients with Parkinson’s disease: a systematic review and meta-analysis
Rui Zhong, Qingling Chen, Xinyue Zhang, Mengmeng Li & Weihong Lin
doi : 10.1007/s00415-021-10473-1
Journal of Neurology volume 269, pages 703–711 (2022)
Helicobacter pylori (HP) infection has been reported to be associated with increased severity of Parkinson's disease (PD) and have negative effects on drug response in patients. We aimed to investigate the influence of HP infection on patients with PD using a systematic review and meta-analysis approach.
Efficacy and safety of intravenous immunoglobulins for the treatment of viral encephalitis: a systematic literature review
Judith N. Wagner, Annette Leibetseder, Anna Troescher, Juergen Panholzer & Tim J. von Oertzen
doi : 10.1007/s00415-021-10494-w
Journal of Neurology volume 269, pages 712–724 (2022)
For most viral encephalitides, therapy is merely supportive. Intravenous immunoglobulins (IVIG) have been used as a prophylactic and therapeutic approach. We conduct a systematic review on the safety and efficacy of IVIG in viral encephalitis.
Is postural orthostatic tachycardia syndrome (POTS) a central nervous system disorder?
Svetlana BlitshteynÂ
doi : 10.1007/s00415-021-10502-z
Journal of Neurology volume 269, pages 725–732 (2022)
Postural orthostatic tachycardia syndrome (POTS), a disorder of the autonomic nervous system characterized by a rise in heart rate of at least 30Â bpm from supine to standing position, has been traditionally viewed as a dysfunction of the peripheral nervous system. However, recent studies and evidence from overlapping conditions suggest that in addition to being considered a disorder of the peripheral nervous system, POTS should be viewed also as a central nervous system (CNS) disorder given (1) significant CNS symptom burden in patients with POTS; (2) structural and functional differences found on neuroimaging in patients with POTS and other forms of orthostatic intolerance; (3) evidence of cerebral hypoperfusion and possible alteration in cerebrospinal fluid volume, and (4) positive response to medications targeting the CNS and non-pharmacologic CNS therapies. This review outlines existing evidence of POTS as a CNS disorder and proposes a hypothetical model combining key mechanisms in the pathophysiology of POTS. Redefining POTS as a CNS disorder can lead to new possibilities in pharmacotherapy and non-pharmacologic therapeutic interventions in patents affected by this disabling syndrome.
Clinical efficacy of the enzyme replacement therapy in patients with late-onset Pompe disease: a systematic review and a meta-analysis
Berli Sarah, Brandi Giovanna, Keller Emanuela, Najia Nadi, Vitale Josè & Pagnamenta Alberto
doi : 10.1007/s00415-021-10526-5
Journal of Neurology volume 269, pages 733–741 (2022)
In patients with late-onset Pompe disease (LOPD), the efficacy of the enzyme replacement therapy (ERT) with recombinant human alpha-glucosidase (rhGAA) is difficult to evaluate, due to the clinical heterogeneity and the small sample sizes in published studies. Therefore, we conduct a systematic literature review and meta-analysis of the literature to evaluate the efficacy of ERT in LOPD patients considering the walking distance, respiratory function and muscle strength. Particularly, six-minute walk test (6MWT), forced vital capacity (FVC), medical research council (MRC) grading, quantitative muscle testing (QMT), and quick motor function test (QMFT) were outcomes of interest. Overall, 619 studies were identified in PubMed, EMBASE and by manual search on July 18th, 2020. After an initial assessment, 16 studies were included in the meta-analysis, containing clinical data from 589 patients with LOPD. For the 6MWT, 419 patients were analyzed. Walking distance improved on average, 32.2 m greater during the observed period (p = 0.0003), compared to the distance at the baseline. The meta-analysis did not show any improvement in FVC and only a tendency towards better muscle strength after treatment with ERT, but the difference was not statistically significant. In conclusion, the available data showed that ERT has a significant beneficial efficacy in the improvement of walking distance in LOPD patients and a non-significant improvement of muscle strength. No improvement in respiratory capacity was found. More prospective and controlled trials are needed to demonstrate a clear clinical benefit of ERT.
A systematic review and meta-analyses on the prevalence of pregnancy outcomes in migraine treated patients: a contribution from the IMI2 ConcePTION project
Daniel C. Dudman, Fatima Tauqeer, Moninder Kaur, Mary E. Ritchey, Hu Li & Sandra Lopez-Leon
doi : 10.1007/s00415-021-10534-5
Journal of Neurology volume 269, pages 742–749 (2022)
The present study aims to summarize the safety profile of the medications used to treat migraine during pregnancy by performing a systematic review and meta-analyses. The term “migrain*� combined with pregnancy terms were used to search Embase, PubMed, PsychInfo, Scopus, and Web of Science through 31 December 2020. Pooled prevalences of untreated and treated migraine patients were estimated using MetaXL software. Pooled odds ratios (OR) using random effects models were estimated in RevMan 5. All the identified studies assessed medications used to treat acute migraine. The pooled prevalence of adverse pregnancy outcomes in patients prescribed any migraine medication ranged from 0.4% (95% CI 0.2–0.7%) for stillbirth to 12.0% (95%CI 7.8–16.9%) for spontaneous abortions. Among untreated patients with migraine, the pooled prevalence of the assessed pregnancy outcomes ranged from 0.6% (95% CI: 0–1.7%) for stillbirth to 10.4% (95% CI: 8.9–12%) for gestational age < 37 weeks. Given the limited data, it was only possible to perform OR meta-analyses for triptans. The adjusted ORs for triptan users compared the general population were: for major malformations 1.07 (95%CI: 0.83–1.39, p = 0.60); birth weight < 2500g 1.18 (95%CI: 0.94–1.48, p = 0.16); gestational age < 37 weeks 1.49 (95% CI: 0.37–6.08, p = 0.58). In conclusion, triptans do not appear to increase the risk of pregnancy outcomes when compared to the general population. It was not possible to assess other migraine medications. Further studies are needed to investigate the safety of individual medications of acute and prophylactic migraine treatment among pregnant women.
Opsoclonus–myoclonus–ataxia syndrome in children
Poonam Bhatia, Jennifer Heim, Patricia Cornejo, Lauren Kane, Jason Santiago & Michael C. Kruer
doi : 10.1007/s00415-021-10536-3
Journal of Neurology volume 269, pages 750–757 (2022)
Opsoclonus–myoclonus–ataxia syndrome is a rare neuroimmunologic disorder typically presenting in previously healthy infants and toddlers. It is characterized by a clinical triad of (1) erratic saccadic intrusions; (2) myoclonus and/or ataxia; (3) behavioral features, typified by developmental plateauing, irritability and insomnia. About half of cases are associated with an underlying neuroblastoma and diagnostic imaging is essential once OMAS is suspected. A thorough workup, including serum, urine, and cerebrospinal fluid studies is critical to identify underlying biomarkers of OMAS itself or neuroblastoma. Historically, many children had relatively poor long-term outcomes, with residual neurologic and/or neuropsychiatry sequelae typical. More recent concepts have emphasized combined immunotherapy regimens that offer hope for better outcomes in children with this remarkable, challenging disease.
Unexpected (123I)FP-CIT SPECT findings: SWIDD, SWEDD and all DAT
Balestrino Roberta, Barone Paolo, Filippi Massimo & Erro Roberto
doi : 10.1007/s00415-021-10809-x
Journal of Neurology volume 269, pages 758–770 (2022)
Although the diagnosis of Parkinson’s disease (PD) is essentially clinical, the implementation of imaging techniques can improve diagnostic accuracy. While some techniques (e.g. magnetic resonance imaging—MRI, computerized tomography—CT) are used to exclude secondary syndromes, presynaptic dopaminergic imaging including imaging of dopamine transporter (DAT)—can help the Neurologist in the differential diagnosis between neurodegenerative parkinsonian syndromes and parkinsonism without dopamine deficiency. DAT imaging can be useful in cases in which the clinical picture is not univocal, as in case of overlapping clinical features in patients with early disease, atypical syndromes or unsatisfying response to therapy. Currently, (123I)FP-CIT ([123I]N-ω-fluoropropyl-2β-carbomethoxy-3β-(4-iodophenyl)nortropane) (trade name DaTSCAN) is the only agent approved by international regulatory agencies for this purpose. With the increasing use of this technique, some unexpected findings have been reported, including patients clinically diagnosed with PD with a normal SPECT scan [e.g. Scans Without Evidence of Dopaminergic Deficit (SWEDD)]; PD patients with a greater dopaminergic deficit in the striatum ipsilateral to the clinically more affected side [e.g. Scans With Ipsilateral Dopaminergic Deficit (SWIDD)]; as well as some artifacts. Moreover, the neurologist must remember that structural lesions and administration of some drugs might alter the result of DAT imaging. Unexpected findings, artifacts, and misinterpretation of imaging findings can lead to an erroneous diagnosis and inappropriate therapy, neglect of other medical conditions that might explain the clinical picture, and undermine the selection phase in clinical trials. The aim of the present review is to bring clarity on these controversial (and sometimes erroneous) results, in order to inform of these possibilities the clinicians requesting a DaTSCAN in clinical practice.
Correction to: Unexpected (123I)FP-CIT SPECT findings: SWIDD, SWEDD and all DAT
Roberta Balestrino, Paolo Barone, Massimo Filippi & Roberto Erro
doi : 10.1007/s00415-021-10923-w
Journal of Neurology volume 269, page 771 (2022)
Safety and outcome of mechanical thrombectomy in ischaemic stroke related to carotid artery dissection
A. Karam, N. Bricout, M. Khyeng, C. Cordonnier, X. Leclerc, H. Henon & B. Casolla
doi : 10.1007/s00415-021-10656-w
Journal of Neurology volume 269, pages 772–779 (2022)
The net clinical benefit of mechanical thrombectomy (MT) in patients with anterior circulation ischaemic stroke associated with large vessel occlusion (AIS-LVO) related to carotid artery dissection (CAD) is uncertain. The aim of the study was to investigate the safety and clinical outcomes of patients treated by MT for a CAD-related stroke.
The impact of device-assisted therapies on the gut microbiome in Parkinson’s disease
Michal Lubomski, Xiangnan Xu, Andrew J. Holmes, Jean Y. H. Yang, Carolyn M. Sue & Ryan L. Davis
doi : 10.1007/s00415-021-10657-9
Journal of Neurology volume 269, pages 780–795 (2022)
Microbiome feedbacks are proposed to influence Parkinson’s disease (PD) pathophysiology. A number of studies have evaluated the impact of oral medication on the gut microbiome (GM) in PD. However, the influence of PD device-assisted therapies (DATs) on the GM remains to be investigated.
What happens after fingolimod discontinuation? A multicentre real-life experience
Doriana Landi, Alessio Signori, Maria Cellerino, Giuseppe Fenu, Carolina Gabri Nicoletti, Marta Ponzano, Elisabetta Mancuso, Marzia Fronza, Maria Elena Ricchiuto, Giacomo Boffa, Matilde Inglese, Girolama Alessandra Marfia, Eleonora Cocco & Jessica Frau
doi : 10.1007/s00415-021-10658-8
Journal of Neurology volume 269, pages 796–804 (2022)
To analyse the course of multiple sclerosis (MS) after fingolimod withdrawal in a multicentre cohort.
Mitochondrial genome variations are associated with amyotrophic lateral sclerosis in patients from mainland China
Jie Ni, Zhen Liu, Yanchun Yuan, Wanzhen Li, Yiting Hu, Pan Liu, Xiaorong Hou, Xiangyu Zhu, Xuxiong Tang, Mingyu liang, Siqi Zheng, Xuan Hou, Juan Du, Jianguang Tang, Hong Jiang, Lu Shen, Beisha Tang & Junling Wang
doi : 10.1007/s00415-021-10659-7
Journal of Neurology volume 269, pages 805–814 (2022)
Amyotrophic lateral sclerosis (ALS) is a late-onset neurodegenerative disorder. Mitochondrial dysfunction is involved in the complex pathophysiology of ALS; however, the role of mitochondrial DNA (mtDNA) variants in ALS is poorly understood. We aimed to elucidate the role of mtDNA variants in the pathogenesis of ALS.
Serum neurofilament light chain or glial fibrillary acidic protein in the diagnosis and prognosis of brain metastases
Xinqing Lin, Tingting Lu, Haiyi Deng, Chunxin Liu, Yilin Yang, Tao Chen, Yinyin Qin, Xiaohong Xie, Zhanhong Xie, Ming Liu, Ming Ouyang, Shiyue Li, Yong Song, Nanshan Zhong, Wei Qiu & Chengzhi Zhou
doi : 10.1007/s00415-021-10660-0
Journal of Neurology volume 269, pages 815–823 (2022)
Brain metastases (BM) remains the most cumbersome disease burden in patients with lung cancer. This study aimed to investigate whether serum brain injury biomarkers can indicate BM, to further establish related diagnostic models, or to predict prognosis of BM.
Safety of alemtuzumab in a nationwide cohort of Finnish multiple sclerosis patients
Ilkka Rauma, Tiina Mustonen, Juha Matti Seppä, Maritta Ukkonen, Marianne Männikkö, Auli Verkkoniemi-Ahola, Marge Kartau, Jukka T. Saarinen, Liisa Luostarinen, Sakari Simula, Mervi Ryytty, Riitta Ahmasalo, Jussi O. T. Sipilä, Ilkka Pieninkeroinen, Tero Tapiola, Anne M. Remes & Hanna Kuusisto
doi : 10.1007/s00415-021-10664-w
Journal of Neurology volume 269, pages 824–835 (2022)
Alemtuzumab is an effective disease-modifying therapy (DMT) for highly active multiple sclerosis (MS). However, safety concerns limit its use in clinical practice.
NMOSD and MS prevalence in the Indigenous populations of Australia and New Zealand
Wajih Bukhari, Elham Khalilidehkordi, Deborah F. Mason, Michael H. Barnett, Bruce V. Taylor, Marzena Fabis-Pedrini, Allan G. Kermode, Sankar Subramanian, Patrick Waters, Simon A. Broadley on behalf of The Australian and New Zealand NMO Collaboration
doi : 10.1007/s00415-021-10665-9
Journal of Neurology volume 269, pages 836–845 (2022)
We studied the prevalence of neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) in Indigenous populations of Australia and New Zealand with the aim of assessing potential differences.
Optimizing the selection of Parkinson’s disease patients for neuromodulation using the levodopa challenge test
Dinkar Kulshreshtha, Marcus Pieterman, Greydon Gilmore & Mandar Jog
doi : 10.1007/s00415-021-10666-8
Journal of Neurology volume 269, pages 846–852 (2022)
In Parkinson’s disease (PD), early stages are associated with a good long-duration response and as the disease advances, the short-duration response predominates. The transition between the long-duration and short-duration responses may be an important and measurable intermediate stage. A critical criterion in determining the candidature for neuromodulation is a beneficial response to an ‘off–on’ levodopa challenge test. This test is usually reserved for those that have already developed marked short-duration response and are candidates for deep brain stimulation (DBS) surgery. However, identifying those that are in transition may allow DBS to be offered earlier.
Paradigm shift in acute dizziness: is caloric testing obsolete?
Miranda Morrison, Athanasia Korda, Ewa Zamaro, Franca Wagner, Marco D. Caversaccio, Thomas C. Sauter, Roger Kalla & Georgios Mantokoudis
doi : 10.1007/s00415-021-10667-7
Journal of Neurology volume 269, pages 853–860 (2022)
Cold and warm water ear irrigation, also known as bithermal caloric testing, has been considered for over 100 years the ‘Gold Standard’ for the detection of peripheral vestibular hypofunction. Its discovery was awarded a Nobel Prize. We aimed to investigate the diagnostic accuracy of Caloric Testing when compared to the video head impulse test (vHIT) in differentiating between vestibular neuritis and vestibular strokes in acute dizziness.
Prediagnosis epilepsy and survival in patients with glioma: a nationwide population-based cohort study from 2009 to 2018
Mirketa Marku, Birthe Krogh Rasmussen, Federica Belmonte, Steinbjørn Hansen, Elisabeth Anne Wreford Andersen, Christoffer Johansen & Pernille Envold Bidstrup
doi : 10.1007/s00415-021-10668-6
Journal of Neurology volume 269, pages 861–872 (2022)
Considering that epilepsy is common, and knowledge is lacking on its role especially for the prognosis of high-grade gliomas, the objective of this study was to investigate the association between epilepsy prior to glioma diagnosis and survival among glioma patients.
Traumatic brain injury fast-forwards Alzheimer’s pathology: evidence from amyloid positron emission tomorgraphy imaging
Abdalla Z. Mohamed, Peter J. Nestor, Paul Cumming & Fatima A. Nasrallah for the Alzheimer’s Disease Neuroimaging Initiative
doi : 10.1007/s00415-021-10669-5
Journal of Neurology volume 269, pages 873–884 (2022)
Traumatic brain injury (TBI) has been proposed as a risk factor for Alzheimer’s disease (AD), although the mechanisms underlying the putative association are poorly understood. We investigated elderly individuals with a remote history of TBI, aiming to understand how this may have influenced amyloidosis, neurodegeneration, and clinical expression along the AD continuum.
Randomized phase 2 study of perampanel for sporadic amyotrophic lateral sclerosis
Hitoshi Aizawa, Haruhisa Kato, Koji Oba, Takuya Kawahara, Yoshihiko Okubo, Tomoko Saito, Makiko Naito, Makoto Urushitani, Akira Tamaoka, Kiyotaka Nakamagoe, Kazuhiro Ishii, Takashi Kanda, Masahisa Katsuno, Naoki Atsuta, Yasushi Maeda, Makiko Nagai, Kazutoshi Nishiyama, Hiroyuki Ishiura, Tatsushi Toda, Akihiro Kawata, Koji Abe, Ichiro Yabe, Ikuko Takahashi-Iwata, Hidenao Sasaki, Hitoshi Warita, Masashi Aoki, Gen Sobue, Hidehiro Mizusawa, Yutaka Matsuyama, Tomohiro Haga & Shin Kwak
doi : 10.1007/s00415-021-10670-y
Journal of Neurology volume 269, pages 885–896 (2022)
To evaluate the efficacy and safety of perampanel in patients with sporadic amyotrophic lateral sclerosis (SALS).
Shorter visual aura characterizes young and middle-aged stroke patients with migraine with aura
Claudia Altamura, Angelo Cascio Rizzo, Giovanna Viticchi, Paola Maggio, Carmelina Maria Costa, Nicoletta Brunelli, Giuditta Giussani, Matteo Paolucci, Fabrizio Fiacco, Vincenzo Di Lazzaro, Elio Clemente Agostoni, Mauro Silvestrini & Fabrizio Vernieri
doi : 10.1007/s00415-021-10671-x
Journal of Neurology volume 269, pages 897–906 (2022)
To identify the clinical profile and aura characteristics of patients with Migraine with Aura (MwA) having acute cerebral ischemia, we compared stroke phenotype and risk factors in stroke patients with (S+MwA+) or without (S+MwA−) MwA and aura features in MwA patients with (S+MwA+) or without (S−MwA+) stroke.
Prolonged distal motor latency of median nerve does not improve diagnostic accuracy for CIDP
Emanuele Spina, Pietro Emiliano Doneddu, Giuseppe Liberatore, Dario Cocito, Raffaella Fazio, Chiara Briani, Massimiliano Filosto, Luana Benedetti, Giovanni Antonini, Giuseppe Cosentino, Stefano Jann, Anna Mazzeo, Andrea Cortese, Girolama Alessandra Marfia, Angelo Maurizio Clerici, Gabriele Siciliano, Marinella Carpo, Marco Luigetti, Giuseppe Lauria, Tiziana Rosso, Guido Cavaletti, Erdita Peci, Stefano Tronci, Marta Ruiz, Stefano Cotti Piccinelli, Angelo Schenone, Luca Leonardi, Luca Gentile, Laura Piccolo, Giorgia Mataluni, Lucio Santoro, Eduardo Nobile-Orazio, Fiore Manganelli on behalf of The Italian CIDP Database Study Group
doi : 10.1007/s00415-021-10672-w
Journal of Neurology volume 269, pages 907–912 (2022)
Compression of the median nerve at the carpal tunnel can give demyelinating features and result in distal motor latency (DML) prolongation fulfilling the EFNS/PNS demyelinating criteria for chronic inflammatory demyelinating polyneuropathy (CIDP). Accordingly, being carpal tunnel syndrome (CTS) common in the general population, the EFNS/PNS guidelines recommend excluding the DML of the median nerve when DML prolongation may be consistent with median neuropathy at the wrist from CTS. The main aims of this study were to verify whether the inclusion of DML of the median nerve (when consistent with CTS) could improve electrophysiological diagnostic accuracy for CIDP and if the median nerve at the carpal tunnel was more prone to demyelination. We analyzed electrophysiological data from 499 patients included consecutively into the Italian CIDP Database. According to the EFNS/PNS criteria, 352 patients had a definite, 10 a probable, and 57 a possible diagnosis of CIDP, while 80 were not fulfilling the diagnostic criteria. The inclusion of DML prolongation of median nerve did not improve significantly the diagnostic accuracy for CIDP; overall diagnostic class changed in 6 out of 499 patients (1.2%) and electrodiagnostic class of CIDP changed from not fulfilling to possible in only 2 patients (2.5% of not-fulfilling patients). In conclusion, we can infer that excluding DML prolongation of median nerve does not increase the risk of missing a diagnosis of CIDP thus corroborating the current EFNS/PNS criteria.
Initial treatment strategy and clinical outcomes in Finnish MS patients: a propensity-matched study
K. Hänninen, M. Viitala, S. Atula, S. M. Laakso, H. Kuusisto & M. Soilu-Hänninen
doi : 10.1007/s00415-021-10673-9
Journal of Neurology volume 269, pages 913–922 (2022)
The optimal treatment strategy with disease-modifying therapies (DMTs) in relapsing–remitting multiple sclerosis (RRMS) remains uncertain.
Quantitative sensory testing and norepinephrine levels in REM sleep behaviour disorder – a clue to early peripheral autonomic and sensory dysfunction?
Julia Koch, Kira Willemsen, Imis Dogan, Roman Rolke, Jörg B. Schulz, Johannes Schiefer, Kathrin Reetz & Andrea Maier
doi : 10.1007/s00415-021-10675-7
Journal of Neurology volume 269, pages 923–932 (2022)
Studies have reported autonomic impairment in patients with idiopathic REM sleep behaviour disorder (iRBD), which is considered a prodromal stage of alpha-synucleinopathies. It is still debated whether central or peripheral pathologies are first manifestations of alpha-synucleinopathies. This study aimed to characterize autonomic and somatosensory function in iRBD patients.
PML risk is the main factor driving the choice of discontinuing natalizumab in a large multiple sclerosis population: results from an Italian multicenter retrospective study
Clara G. Chisari, Giancarlo Comi, Massimo Filippi, Damiano Paolicelli, Pietro Iaffaldano, Mauro Zaffaroni, Vincenzo Brescia Morra, Eleonora Cocco, Girolama Alessandra Marfia, Luigi Maria Grimaldi, Matilde Inglese, Simona Bonavita, Alessandra Lugaresi, Giuseppe Salemi, Giovanna De Luca, Salvatore Cottone, Antonella Conte, Patrizia Sola, Umberto Aguglia, Giorgia Teresa Maniscalco, Claudio Gasperini, Maria Teresa Ferrò, Ilaria Pesci, Maria Pia Amato, Marco Rovaris, Claudio Solaro, Giacomo Lus, Davide Maimone, Roberto Bergamaschi, Franco Granella, Alessia Di Sapio, Antonio Bertolotto, Rocco Totaro, Marika Vianello, Paola Cavalla, Paolo Bellantonio, Vito Lepore, Francesco Patti & the Italian MS Register Study Group.
doi : 10.1007/s00415-021-10676-6
Journal of Neurology volume 269, pages 933–944 (2022)
Natalizumab (NTZ) is an effective treatment for relapsing–remitting multiple sclerosis (RRMS). However, patients and physicians may consider discontinuing NTZ therapy due to safety or efficacy issues. The aim of our study was to evaluate the NTZ discontinuation rate and reasons of discontinuation in a large Italian population of RRMS patients.
Clinical outcome of CIDP one year after start of treatment: a prospective cohort study
S. R. M. Bus, M. C. Broers, I. M. Lucke, C. Bunschoten, G. G. A. van Lieverloo, M. E. Adrichem, R. van Veen, L. Wieske, H. F. Lingsma, H. S. Goedee, W. L. van der Pol, I. N. van Schaik, P. A. Van Doorn, B. C. Jacobs & F. Eftimov for the ICOS Consortium
doi : 10.1007/s00415-021-10677-5
Journal of Neurology volume 269, pages 945–955 (2022)
To assess clinical outcome in treatment-naive patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
Initiation of antidepressants in young adults after ischemic stroke: a registry-based follow-up study
Jenna Broman, Karoliina Aarnio, Anna But, Ivan Marinkovic, Jorge RodrÃguez-Pardo, Markku Kaste, Turgut Tatlisumak & Jukka Putaala
doi : 10.1007/s00415-021-10678-4
Journal of Neurology volume 269, pages 956–965 (2022)
Data on post-stroke use of antidepressants in young individuals are scarce. We examined pattern and factors associated with initiating post-stroke antidepressants (PSAD) after ischemic stroke (IS) in young adults.
Cystatin C predicts futile recanalization in patients with acute ischemic stroke after endovascular treatment
Mouxiao Su, Ying Zhou, Zhonglun Chen, Mingjun Pu, Zhaokun Li, Hongcai Du & Gelin Xu
doi : 10.1007/s00415-021-10680-w
Journal of Neurology volume 269, pages 966–972 (2022)
A previous study reported that cystatin C was related to acute ischemic stroke. The association between cystatin C and the clinical outcome in acute ischaemic stroke patients with successful recanalization after endovascular thrombectomy has rarely been reported. This study aimed to evaluate the association between cystatin C and futile recanalization in AIS patients who underwent endovascular thrombectomy.
Pembrolizumab for treatment of progressive multifocal leukoencephalopathy in primary immunodeficiency and/or hematologic malignancy: a case series of five patients
Timo Volk, Klaus Warnatz, Reinhard Marks, Horst Urbach, Gisela Schluh, Valentina Strohmeier, Jessica Rojas-Restrepo, Bodo Grimbacher & Sebastian Rauer
doi : 10.1007/s00415-021-10682-8
Journal of Neurology volume 269, pages 973–981 (2022)
Progressive multifocal leukoencephalopathy is a rare opportunistic infection of the brain by John Cunningham polyomavirus in immune-compromised patients. In cases where no overt option for immune reconstitution is available [e.g., in patients with primary immunodeficiency (PID)], the disease is lethal in the majority of patients. Immune checkpoint inhibition has been applied in recent years with mixed outcomes. We present four novel patients and the follow-up of a previously published patient suffering from progressive multifocal leukoencephalopathy (PML) due to PID and/or hematologic malignancy who were treated with the immune checkpoint inhibitor pembrolizumab. In two patients with PID, symptoms improved and stabilized. One patient died because of worsening PML another of intracranial hemorrhage which was unrelated to PML or its treatment with pembrolizumab. The fifth patient suffered from PID and died of a pre-existing immune dysregulation, possibly exacerbated by pembrolizumab. The long-term follow-up of the first patient provides support for therapeutic decisions during this therapy and is the longest published clinical course of a patient with checkpoint inhibition for PML. We conclude that pembrolizumab can control PML symptoms long term in a subgroup of patients with PID, in our cases for 21 and 36Â months. However, therapy must be started early because symptoms are only partially reversible. In light of severe adverse events, application of pembrolizumab is only justified if the prognosis for the individual patient is very poor.
Diagnosis and follow-up evaluation of central nervous system vasculitis: an evaluation of vessel-wall MRI findings
Maximilian Patzig, Robert Forbrig, Clemens Küpper, Ozan Eren, Tobias Saam, Lars Kellert, Thomas Liebig & Florian Schöberl
doi : 10.1007/s00415-021-10683-7
Journal of Neurology volume 269, pages 982–996 (2022)
To approach the clinical value of MRI with vessel wall imaging (VWI) in patients with central nervous system vasculitis (CNSV), we analyzed patterns of VWI findings both at the time of initial presentation and during follow-up.
The angiographic presentation of European Moyamoya angiopathy
Sara Pilgram-Pastor, René Chapot & Markus Kraemer
doi : 10.1007/s00415-021-10684-6
Journal of Neurology volume 269, pages 997–1006 (2022)
Little is known about the angiographic presentation of Moyamoya angiopathy (MMA) in non-Asian patients.
Blink reflex recovery cycle distinguishes patients with idiopathic normal pressure hydrocephalus from elderly subjects
Alessandro Mechelli, Andrea Quattrone, Rita Nisticò, Marianna Crasà , Domenico La Torre, Basilio Vescio & Aldo Quattrone
doi : 10.1007/s00415-021-10687-3
Journal of Neurology volume 269, pages 1007–1012 (2022)
The R2 component of blink reflex recovery cycle (R2BRrc) is a simple neurophysiological tool to detect the brainstem hyperexcitability commonly occurring in several neurological diseases such as Parkinson’s disease and atypical parkinsonisms. In our study, we investigated for the first time the usefulness of R2BRrc to assess brainstem excitability in patients with idiopathic Normal Pressure Hydrocephalus (iNPH) in comparison with healthy subjects.
Association of the Careggi Collateral Score with 3-month modified Rankin Scale score after thrombectomy for stroke with occlusion of the middle cerebral artery
Manuel Cappellari, Valentina Saia, Giovanni Pracucci, Fainardi Enrico, Arturo Consoli, Sergio Nappini, Lucio Castellan, Sandra Bracco, Mauro Bergui, Mirco Cosottini, Alessandra Briatico Vangosa, Sergio Vinci, Maria Ruggiero, Edoardo Puglielli, Luigi Chiumarulo, Giacomo Cester, Chiara Comelli, Umberto Silvagni, Daniele Morosetti, Valentina Caldiera, Nicola Cavasin, Valeria Ledda, Giuseppina Sanfilippo, Andrea Saletti, Pietro Filauri, Ivan Gallesio, Nunzio Paolo Nuzzi, Pitero Amistá, Cecilia Zivelonghi, Mauro Plebani, Marco Pavia, Daniele Romano, Francesco Biraschi, Roberto Menozzi, Roberto Gasparotti, Andrea Giorgianni, Andrea Zini, Domenico Inzitari, Danilo Toni, Salvatore Mangiafico on behalf of The IRETAS Group
doi : 10.1007/s00415-021-10898-8
Journal of Neurology volume 269, pages 1013–1023 (2022)
The Careggi Collateral Score (CCS) (qualitative–quantitative evaluation) was developed from a single-centre cohort as an angiographic score to describe both the extension and effectiveness of the pial collateral circulation in stroke patients with occlusion of the anterior circulation. We aimed to examine the association between CCS (quantitative evaluation) and 3-month modified Rankin Scale (mRS) score in a large multi-center cohort of patients receiving thrombectomy for stroke with occlusion of middle cerebral artery (MCA).
Correction to: Association of the Careggi Collateral Score with 3-month modified Rankin Scale score after thrombectomy for stroke with occlusion of the middle cerebral artery
Manuel Cappellari, Valentina Saia, Giovanni Pracucci, Enrico Fainardi, Arturo Consoli, Sergio Nappini, Lucio Castellan, Sandra Bracco, Mauro Bergui, Mirco Cosottini, Alessandra Briatico Vangosa, Sergio Vinci, Maria Ruggiero, Edoardo Puglielli, Luigi Chiumarulo, Giacomo Cester, Chiara Comelli, Umberto Silvagni, Daniele Morosetti, Valentina Caldiera, Nicola Cavasin, Valeria Ledda, Giuseppina Sanfilippo, Andrea Saletti, Pietro Filauri, Ivan Gallesio, Nunzio Paolo Nuzzi, Pitero Amistá, Cecilia Zivelonghi, Mauro Plebani, Marco Pavia, Daniele Romano, Francesco Biraschi, Roberto Menozzi, Roberto Gasparotti, Andrea Giorgianni, Andrea Zini, Domenico Inzitari, Danilo Toni, Salvatore Mangiafico on behalf of The IRETAS Group
doi : 10.1007/s00415-021-10941-8
Journal of Neurology volume 269, pages 1024–1025 (2022)
Ocular flutter is a rare and treatment-responsive symptom in Lyme neuroborreliosis: a case report
Fiona Jäger, Stefan Greisenegger, Felix K. Schwarz & Gerald Wiest
doi : 10.1007/s00415-021-10769-2
Journal of Neurology volume 269, pages 1026–1027 (2022)
Paroxysmal central positional nystagmus responsive to clonazepam
Eun Hye Oh, Hyunsung Kim, Seo-Young Choi, Kwang-Dong Choi & Jae-Hwan Choi
doi : 10.1007/s00415-021-10770-9
Journal of Neurology volume 269, pages 1028–1031 (2022)
When should we consider chronic patients as non-responders to monoclonal antibodies targeting the CGRP pathway?
Claudia Altamura, Sabina Cevoli, Nicoletta Brunelli, Cinzia Aurilia, Luisa Fofi, Gabriella Egeo, Piero Barbanti, Fabrizio Vernieri
doi : 10.1007/s00415-021-10772-7
Journal of Neurology volume 269, pages 1032–1034 (2022)
Pathogenic events in very early Guillain–Barré syndrome: neither demyelination nor axonal degeneration but endoneurial inflammatory oedema
José BercianoÂ
doi : 10.1007/s00415-021-10773-6
Journal of Neurology volume 269, pages 1035–1037 (2022)
Alzheimer’s disease clinical trial update 2019–2021
Joseph Pleen & Ryan Townley
doi : 10.1007/s00415-021-10790-5
Journal of Neurology volume 269, pages 1038–1051 (2022)
The current clinical trial landscape targeting Alzheimer’s disease (AD) is reviewed in the context of studies completed from 2019 to 2021. This review focuses on available data for observational and phase II/III clinical trial results, which will have the most impact on the field. ClinicalTrials.gov, the United States (US) comprehensive federal registry, was queried to identify completed trials. There are currently 226 interventional clinical trials and 51 observational studies completed, suspended, terminated, or withdrawn within our selected time frame. This review reveals that the role of biomarkers is expanding and although many lessons have been learned, many challenges remain when targeting disease modification of AD through amyloid and tau. In addition, to halt or slow clinical progression of AD, new clinical and observational trials are focusing on prevention as well as the role of more diverse biological processes known to influence AD pathology.
Effects on cognition of DMTs in multiple sclerosis: moving beyond the prevention of inflammatory activity
Paolo Preziosa, Lorenzo Conti, Maria A. Rocca & Massimo Filippi
doi : 10.1007/s00415-021-10832-y
Journal of Neurology volume 269, pages 1052–1064 (2022)
In this review, we critically summarize recent findings derived from randomized controlled trials (RCTs), observational studies and meta-analyses that have been published in the last 3Â years and that included the effects of DMTs on cognitive performances among their outcomes.
Advances in the use of stem cell transplants in the treatment of multiple sclerosis
Rachel Thomas, Ray Wynford-Thomas & Neil P. Robertson
doi : 10.1007/s00415-021-10927-6
Journal of Neurology volume 269, pages 1065–1067 (2022)
Carlo Francesco Giuseppe Bellingeri (1785–1843)
Lorenzo Lorusso, Stefano Zago & Stefano Sandrone
doi : 10.1007/s00415-021-10732-1
Journal of Neurology volume 269, pages 1068–1069 (2022)
Association of CNS demyelination and COVID-19 infection: an updated systematic review
Ismail Ibrahim Ismail & Sara Salama
doi : 10.1007/s00415-021-10752-x
Journal of Neurology volume 269, pages 541–576 (2022)
Since the declaration of COVID-19 pandemic, several case reports of demyelination of both peripheral and central nervous systems have been published. The association between CNS demyelination and viral infection has long been documented, and this link was recently reported following SARS-CoV-2 infection as well.
A rise in cases of nitrous oxide abuse: neurological complications and biological findings
Maximilian Einsiedler, Paul Voulleminot, Stanislas Demuth, Pauline Kalaaji, Thomas Bogdan, Lucas Gauer, Cécile Reschwein, Aleksandra Nadaj-Pakleza, Jérôme de Sèze, Laurent Kremer, Ivana Schroder & Kévin Bigaut
doi : 10.1007/s00415-021-10702-7
Journal of Neurology volume 269, pages 577–582 (2022)
The recent lockdown due to the COVID-19 pandemic has been linked to a higher incidence of psychiatric manifestations and substance abuse. The recreative use of nitrous oxide is more and more widespread and neurological complications are frequent.
Case report: cerebral sinus vein thrombosis in two patients with AstraZeneca SARS-CoV-2 vaccination
Mathias Fousse, David Schub, Fatma Merzou, Klaus Fassbender, Martina Sester, Michael Kettner, Piergiorgio Lochner, Tina Schmidt & João Reinoldo Goi Júnior
doi : 10.1007/s00415-021-10731-2
Journal of Neurology volume 269, pages 583–586 (2022)
SARS-CoV-2 infection is associated with an increased rate of thromboembolic events and mortality. Different vaccines are globally used to limit the pandemic. In this report, we present the case of two young female patients with newly diagnosed cerebral sinus vein thrombosis occurring after injection of the vector-based ChAdOx1 vaccine. Both patients presented with unusual headache only. The two of them used an estrogen-containing contraception, had had a history of deep venous thrombosis, and both had MTHFR mutations. Both patients developed SARS-CoV-2 specific humoral and cellular immunity including both CD4 and CD8 T cells. This rare, but serious complication needs to be considered after vaccination of young females, even if there is no evidence of heparin-induced thrombocytopenia.
Autonomic dysfunction in post-COVID patients with and witfhout neurological symptoms: a prospective multidomain observational study
Alex Buoite Stella, Giovanni Furlanis, Nicolò Arjuna Frezza, Romina Valentinotti, Milos Ajcevic & Paolo Manganotti
doi : 10.1007/s00415-021-10735-y
Journal of Neurology volume 269, pages 587–596 (2022)
The autonomic nervous system (ANS) can be affected by COVID-19, and dysautonomia may be a possible complication in post-COVID individuals. Orthostatic hypotension (OH) and postural tachycardia syndrome (POTS) have been suggested to be common after SARS-CoV-2 infection, but other components of ANS function may be also impaired. The Composite Autonomic Symptom Scale 31 (COMPASS-31) questionnaire is a simple and validated tool to assess dysautonomic symptoms. The aim of the present study was to administer the COMPASS-31 questionnaire to a sample of post-COVID patients with and without neurological complaints. Participants were recruited among the post-COVID ambulatory services for follow-up evaluation between 4 weeks and 9 months from COVID-19 symptoms onset. Participants were asked to complete the COMPASS-31 questionnaire referring to the period after COVID-19 disease. Heart rate and blood pressure were manually taken during an active stand test for OH and POTS diagnosis. One-hundred and eighty participants were included in the analysis (70.6% females, 51 ± 13 years), and OH was found in 13.8% of the subjects. Median COMPASS-31 score was 17.6 (6.9–31.4), with the most affected domains being orthostatic intolerance, sudomotor, gastrointestinal and pupillomotor dysfunction. A higher COMPASS-31 score was found in those with neurological symptoms (p < 0.01), due to more severe orthostatic intolerance symptoms (p < 0.01), although gastrointestinal (p < 0.01), urinary (p < 0.01), and pupillomotor (p < 0.01) domains were more represented in the non-neurological symptoms group. This study confirms the importance of monitoring ANS symptoms as a possible complication of COVID-19 disease that may persist in the post-acute period.
Decrease in intravenous thrombolysis and poor short-term functional prognosis for acute ischemic stroke during the COVID-19 pandemic
Xueqian Xu, Yuenan Xiao, Jia Li, Liuzhu Chen, Gangqiang Lin, Ledan Dong, Yisi Lin, Luqian Zhan, Jincai He & Xiaoqian Luan
doi : 10.1007/s00415-021-10740-1
Journal of Neurology volume 269, pages 597–602 (2022)
Since the outbreak of the coronavirus pandemic in 2019 (COVID-19), healthcare systems around the world have been hit to varying degrees. As a neurologist team, for patients with acute ischemic stroke (AIS), we compared the situations of intravenous thrombolysis (IVT) treatment from 2019 to 2020 to investigate the influence of COVID-19 pandemic on the attendance and prognosis of the IVT patients.
Delays in thrombolysis during COVID-19 are associated with worse neurological outcomes: the Society of Vascular and Interventional Neurology Multicenter Collaboration
Dinesh V. Jillella, Fadi Nahab, Thanh N. Nguyen, Mohamad Abdalkader, David S. Liebeskind, Nirav Vora, Vivek Rai, Diogo C. Haussen, Raul G. Nogueira, Shashvat Desai, Ashutosh P. Jadhav, Alexandra L. Czap, Alicia M. Zha, Italo Linfante, Ameer E Hassan, Darko Quispe-Orozco, Santiago Ortega-Gutierrez, Priyank Khandelwal, Pratit Patel, Osama Zaidat, Tudor G. Jovin, Scott Kamen & James E. Siegler
doi : 10.1007/s00415-021-10734-z
Journal of Neurology volume 269, pages 603–608 (2022)
We have demonstrated in a multicenter cohort that the COVID-19 pandemic has led to a delay in intravenous thrombolysis (IVT) among stroke patients. Whether this delay contributes to meaningful short-term outcome differences in these patients warranted further exploration.
Frontal predominant encephalopathy with early paligraphia as a distinctive signature of CAR T-cell therapy-related neurotoxicity
Umberto Pensato, Giulia Amore, Roberto D’Angelo, Rita Rinaldi, Marianna Nicodemo, Francesca Rondelli, Susanna Mondini, Rossella Santoro, Susanna Sammali, Andrea Farolfi, Luca Spinardi, Luca Faccioli, Beatrice Casadei, Michele Dicataldo, Francesca Bonifazi, Pierluigi Zinzani, Pietro Cortelli, Andrea Stracciari & Maria Guarino
doi : 10.1007/s00415-021-10766-5
Journal of Neurology volume 269, pages 609–615 (2022)
Chimeric antigen receptor (CAR) T-cell therapy is an emerging highly effective treatment for refractory haematological malignancies. Unfortunately, its therapeutic benefit may be hampered by treatment-related toxicities, including neurotoxicity. Early aggressive treatment is paramount to prevent neurological sequelae, yet it potentially interferes with the anti-cancer action of CAR T-cells. We describe four CAR T-cells infused patients who presented with reiterative writing behaviours, namely paligraphia, as an early manifestation of neurotoxicity, and eventually developed frontal predominant encephalopathy (one mild, three severe). Paligraphia may represent an early, specific, and easily detectable clinical finding of CAR T-cell therapy-related neurotoxicity, potentially informing its management.
Misinterpretation of glioblastoma as ADEM: potentially harmful consequences of over-diagnosis of COVID-19 vaccine-associated adverse events
C. O´Sullivan, F. Zach, T. Moser, G. Pilz, A. Harrer, E. Trinka, C. Enzinger, J. A. R. Pfaff & P. Wipfler
doi : 10.1007/s00415-021-10707-2
Journal of Neurology volume 269, pages 616–618 (2022)
Prevention of recurrent benign paroxysmal positional vertigo with vitamin D supplementation: a meta-analysis
Seong-Hae Jeong, Sun-Uk Lee & Ji-Soo Kim
doi : 10.1007/s00415-020-09952-8
Journal of Neurology volume 269, pages 619–626 (2022)
Vitamin D insufficiency/deficiency is known to be related to occurrences and recurrences of benign paroxysmal positional vertigo (BPPV). However, the efficacy of vitamin D supplementation in reducing recurrences of BPPV remains to be established. We performed a meta-analysis to determine the therapeutic effects of vitamin D supplementation, with or without calcium, for preventing recurrences of BPPV.
Telerehabilitation in response to constrained physical distance: an opportunity to rethink neurorehabilitative routines
Arturo Nuara, Maddalena Fabbri-Destro, Emilia Scalona, Stefano Elio Lenzi, Giacomo Rizzolatti & Pietro Avanzini
doi : 10.1007/s00415-021-10397-w
Journal of Neurology volume 269, pages 627–638 (2022)
Ensuring proper dosage of treatment and repetition over time is a major challenge in neurorehabilitation. However, a requirement of physical distancing to date compromises their achievement. While mostly associated to COVID-19, physical distancing is not only required in a pandemic scenario, but also advised for several clinical conditions (e.g. immunocompromised individuals) or forced for specific social contexts (e.g. people living in remote areas worldwide). All these contexts advocate for the implementation of alternative healthcare models. The objective of this perspective is to highlight the benefits of remote administration of rehabilitative treatment, namely telerehabilitation, in counteracting physical distancing barriers in neurorehabilitation. Sustaining boosters of treatment outcome, such as compliance, sustainability, as well as motivation, telerehabilitation may adapt to multiple neurological conditions, with the further advantage of a high potential for individualization to patient’s or pathology’s specificities. The effectiveness of telerehabilitation can be potentiated by several technologies available to date: virtual reality can recreate realistic environments in which patients may bodily operate, wearable sensors allow to quantitatively monitor the patient’s performance, and signal processing may contribute to the prediction of long-term dynamics of patient recovery. Telerehabilitation might spark its advantages far beyond the mere limitation of physical distancing effects, mitigating criticalities of daily neurorehabilitative practice, and thus paving the way to the envision of mixed models of care, where hospital-based procedures are complementarily integrated with telerehabilitative ones.
Differentiating dementia with Lewy bodies from Alzheimer's disease and Parkinson's disease dementia: an update on imaging modalities
Rotem Iris Orad & Tamara Shiner
doi : 10.1007/s00415-021-10402-2
Journal of Neurology volume 269, pages 639–653 (2022)
Dementia with Lewy bodies is the second most common cause of neurodegenerative dementia after Alzheimer's disease. Dementia with Lewy bodies can provide a diagnostic challenge due to the frequent overlap of clinical signs with other neurodegenerative conditions, namely Parkinson's disease dementia, and Alzheimer's disease. Part of this clinical overlap is due to the neuropathological overlap. Dementia with Lewy bodies is characterized by the accumulation of aggregated α-synuclein protein in Lewy bodies, similar to Parkinson's disease and Parkinson's disease dementia. However, it is also frequently accompanied by aggregation of amyloid-beta and tau, the pathological hallmarks of Alzheimer's disease. Neuroimaging is central to the diagnostic process. This review is an overview of both established and evolving imaging methods that can improve diagnostic accuracy and improve management of this disorder.
Functional neurological disorder and multiple sclerosis: a systematic review of misdiagnosis and clinical overlap
Dennis Walzl, Andrew J. Solomon & Jon Stone
doi : 10.1007/s00415-021-10436-6
Journal of Neurology volume 269, pages 654–663 (2022)
Multiple sclerosis (MS) and functional neurological disorder (FND) are both diagnostically challenging conditions which can present with similar symptoms. We systematically reviewed the literature to identify patients with MS who were misdiagnosed with FND, patients with FND who were misdiagnosed with MS, and reports of patients with both conditions. In addition to FND, we included studies of patients with other functional and psychiatric disorders where these caused symptoms leading to investigation for or a diagnosis of MS, which in a different context would likely have been labeled as FND. Our review suggests that MS is one of the most common causes of misdiagnosis of FND and vice versa. We discuss the clinical errors that appear to result in misdiagnoses, such as over-reliance on psychiatric comorbidity when making a diagnosis of FND or over-reliance on neuroimaging for the diagnosis of MS, and practical ways to avoid them. Comorbidity between these two conditions is also likely common, has been poorly studied, and adds complexity to diagnosis and treatment in patients with both MS and FND. Misdiagnosis and comorbidity in a landscape of emerging evidence-based treatments for both MS and FND are issues not only of clinical importance to the care of these patients, but also to treatment trials, especially of MS, where FND could be a hidden confounder.
Risk of intracranial hemorrhage with direct oral anticoagulants: a systematic review and meta-analysis of randomized controlled trials
Tingting Wu, Chenyang Lv, Lishui Wu, Wenjun Chen, Meina Lv, Shaojun Jiang & Jinhua Zhang
doi : 10.1007/s00415-021-10448-2
Journal of Neurology volume 269, pages 664–675 (2022)
We performed a systematic review and meta-analysis to compare the risk of intracranial hemorrhage (ICH) between direct oral anticoagulants (DOACs) and other antithrombotic drugs in detail across all diseases.
Genetic counseling and testing practices for late-onset neurodegenerative disease: a systematic review
Ashley Crook, Chris Jacobs, Toby Newton-John, Rosie O’Shea & Alison McEwen
doi : 10.1007/s00415-021-10461-5
Journal of Neurology volume 269, pages 676–692 (2022)
To understand contemporary genetic counseling and testing practices for late-onset neurodegenerative diseases (LONDs), and identify whether practices address the internationally accepted goals of genetic counseling: interpretation, counseling, education, and support.
Coping strategies among amyotrophic lateral sclerosis (ALS) patients: an integrative review
Georgiana Soares Leandro, Mário EmÃlio Teixeira Dourado Júnior, Glauciane Costa Santana & Luan Samy Xavier Dantas
doi : 10.1007/s00415-021-10472-2
Journal of Neurology volume 269, pages 693–702 (2022)
To identify coping strategies used by amyotrophic lateral sclerosis (ALS) patients.
Helicobacter pylori infection is associated with a poor response to levodopa in patients with Parkinson’s disease: a systematic review and meta-analysis
Rui Zhong, Qingling Chen, Xinyue Zhang, Mengmeng Li & Weihong Lin
doi : 10.1007/s00415-021-10473-1
Journal of Neurology volume 269, pages 703–711 (2022)
Helicobacter pylori (HP) infection has been reported to be associated with increased severity of Parkinson's disease (PD) and have negative effects on drug response in patients. We aimed to investigate the influence of HP infection on patients with PD using a systematic review and meta-analysis approach.
Efficacy and safety of intravenous immunoglobulins for the treatment of viral encephalitis: a systematic literature review
Judith N. Wagner, Annette Leibetseder, Anna Troescher, Juergen Panholzer & Tim J. von Oertzen
doi : 10.1007/s00415-021-10494-w
Journal of Neurology volume 269, pages 712–724 (2022)
For most viral encephalitides, therapy is merely supportive. Intravenous immunoglobulins (IVIG) have been used as a prophylactic and therapeutic approach. We conduct a systematic review on the safety and efficacy of IVIG in viral encephalitis.
Is postural orthostatic tachycardia syndrome (POTS) a central nervous system disorder?
Svetlana BlitshteynÂ
doi : 10.1007/s00415-021-10502-z
Journal of Neurology volume 269, pages 725–732 (2022)
Postural orthostatic tachycardia syndrome (POTS), a disorder of the autonomic nervous system characterized by a rise in heart rate of at least 30Â bpm from supine to standing position, has been traditionally viewed as a dysfunction of the peripheral nervous system. However, recent studies and evidence from overlapping conditions suggest that in addition to being considered a disorder of the peripheral nervous system, POTS should be viewed also as a central nervous system (CNS) disorder given (1) significant CNS symptom burden in patients with POTS; (2) structural and functional differences found on neuroimaging in patients with POTS and other forms of orthostatic intolerance; (3) evidence of cerebral hypoperfusion and possible alteration in cerebrospinal fluid volume, and (4) positive response to medications targeting the CNS and non-pharmacologic CNS therapies. This review outlines existing evidence of POTS as a CNS disorder and proposes a hypothetical model combining key mechanisms in the pathophysiology of POTS. Redefining POTS as a CNS disorder can lead to new possibilities in pharmacotherapy and non-pharmacologic therapeutic interventions in patents affected by this disabling syndrome.
Clinical efficacy of the enzyme replacement therapy in patients with late-onset Pompe disease: a systematic review and a meta-analysis
Berli Sarah, Brandi Giovanna, Keller Emanuela, Najia Nadi, Vitale Josè & Pagnamenta Alberto
doi : 10.1007/s00415-021-10526-5
Journal of Neurology volume 269, pages 733–741 (2022)
In patients with late-onset Pompe disease (LOPD), the efficacy of the enzyme replacement therapy (ERT) with recombinant human alpha-glucosidase (rhGAA) is difficult to evaluate, due to the clinical heterogeneity and the small sample sizes in published studies. Therefore, we conduct a systematic literature review and meta-analysis of the literature to evaluate the efficacy of ERT in LOPD patients considering the walking distance, respiratory function and muscle strength. Particularly, six-minute walk test (6MWT), forced vital capacity (FVC), medical research council (MRC) grading, quantitative muscle testing (QMT), and quick motor function test (QMFT) were outcomes of interest. Overall, 619 studies were identified in PubMed, EMBASE and by manual search on July 18th, 2020. After an initial assessment, 16 studies were included in the meta-analysis, containing clinical data from 589 patients with LOPD. For the 6MWT, 419 patients were analyzed. Walking distance improved on average, 32.2 m greater during the observed period (p = 0.0003), compared to the distance at the baseline. The meta-analysis did not show any improvement in FVC and only a tendency towards better muscle strength after treatment with ERT, but the difference was not statistically significant. In conclusion, the available data showed that ERT has a significant beneficial efficacy in the improvement of walking distance in LOPD patients and a non-significant improvement of muscle strength. No improvement in respiratory capacity was found. More prospective and controlled trials are needed to demonstrate a clear clinical benefit of ERT.
A systematic review and meta-analyses on the prevalence of pregnancy outcomes in migraine treated patients: a contribution from the IMI2 ConcePTION project
Daniel C. Dudman, Fatima Tauqeer, Moninder Kaur, Mary E. Ritchey, Hu Li & Sandra Lopez-Leon
doi : 10.1007/s00415-021-10534-5
Journal of Neurology volume 269, pages 742–749 (2022)
The present study aims to summarize the safety profile of the medications used to treat migraine during pregnancy by performing a systematic review and meta-analyses. The term “migrain*� combined with pregnancy terms were used to search Embase, PubMed, PsychInfo, Scopus, and Web of Science through 31 December 2020. Pooled prevalences of untreated and treated migraine patients were estimated using MetaXL software. Pooled odds ratios (OR) using random effects models were estimated in RevMan 5. All the identified studies assessed medications used to treat acute migraine. The pooled prevalence of adverse pregnancy outcomes in patients prescribed any migraine medication ranged from 0.4% (95% CI 0.2–0.7%) for stillbirth to 12.0% (95%CI 7.8–16.9%) for spontaneous abortions. Among untreated patients with migraine, the pooled prevalence of the assessed pregnancy outcomes ranged from 0.6% (95% CI: 0–1.7%) for stillbirth to 10.4% (95% CI: 8.9–12%) for gestational age < 37 weeks. Given the limited data, it was only possible to perform OR meta-analyses for triptans. The adjusted ORs for triptan users compared the general population were: for major malformations 1.07 (95%CI: 0.83–1.39, p = 0.60); birth weight < 2500g 1.18 (95%CI: 0.94–1.48, p = 0.16); gestational age < 37 weeks 1.49 (95% CI: 0.37–6.08, p = 0.58). In conclusion, triptans do not appear to increase the risk of pregnancy outcomes when compared to the general population. It was not possible to assess other migraine medications. Further studies are needed to investigate the safety of individual medications of acute and prophylactic migraine treatment among pregnant women.
Opsoclonus–myoclonus–ataxia syndrome in children
Poonam Bhatia, Jennifer Heim, Patricia Cornejo, Lauren Kane, Jason Santiago & Michael C. Kruer
doi : 10.1007/s00415-021-10536-3
Journal of Neurology volume 269, pages 750–757 (2022)
Opsoclonus–myoclonus–ataxia syndrome is a rare neuroimmunologic disorder typically presenting in previously healthy infants and toddlers. It is characterized by a clinical triad of (1) erratic saccadic intrusions; (2) myoclonus and/or ataxia; (3) behavioral features, typified by developmental plateauing, irritability and insomnia. About half of cases are associated with an underlying neuroblastoma and diagnostic imaging is essential once OMAS is suspected. A thorough workup, including serum, urine, and cerebrospinal fluid studies is critical to identify underlying biomarkers of OMAS itself or neuroblastoma. Historically, many children had relatively poor long-term outcomes, with residual neurologic and/or neuropsychiatry sequelae typical. More recent concepts have emphasized combined immunotherapy regimens that offer hope for better outcomes in children with this remarkable, challenging disease.
Unexpected (123I)FP-CIT SPECT findings: SWIDD, SWEDD and all DAT
Balestrino Roberta, Barone Paolo, Filippi Massimo & Erro Roberto
doi : 10.1007/s00415-021-10809-x
Journal of Neurology volume 269, pages 758–770 (2022)
Although the diagnosis of Parkinson’s disease (PD) is essentially clinical, the implementation of imaging techniques can improve diagnostic accuracy. While some techniques (e.g. magnetic resonance imaging—MRI, computerized tomography—CT) are used to exclude secondary syndromes, presynaptic dopaminergic imaging including imaging of dopamine transporter (DAT)—can help the Neurologist in the differential diagnosis between neurodegenerative parkinsonian syndromes and parkinsonism without dopamine deficiency. DAT imaging can be useful in cases in which the clinical picture is not univocal, as in case of overlapping clinical features in patients with early disease, atypical syndromes or unsatisfying response to therapy. Currently, (123I)FP-CIT ([123I]N-ω-fluoropropyl-2β-carbomethoxy-3β-(4-iodophenyl)nortropane) (trade name DaTSCAN) is the only agent approved by international regulatory agencies for this purpose. With the increasing use of this technique, some unexpected findings have been reported, including patients clinically diagnosed with PD with a normal SPECT scan [e.g. Scans Without Evidence of Dopaminergic Deficit (SWEDD)]; PD patients with a greater dopaminergic deficit in the striatum ipsilateral to the clinically more affected side [e.g. Scans With Ipsilateral Dopaminergic Deficit (SWIDD)]; as well as some artifacts. Moreover, the neurologist must remember that structural lesions and administration of some drugs might alter the result of DAT imaging. Unexpected findings, artifacts, and misinterpretation of imaging findings can lead to an erroneous diagnosis and inappropriate therapy, neglect of other medical conditions that might explain the clinical picture, and undermine the selection phase in clinical trials. The aim of the present review is to bring clarity on these controversial (and sometimes erroneous) results, in order to inform of these possibilities the clinicians requesting a DaTSCAN in clinical practice.
Correction to: Unexpected (123I)FP-CIT SPECT findings: SWIDD, SWEDD and all DAT
Roberta Balestrino, Paolo Barone, Massimo Filippi & Roberto Erro
doi : 10.1007/s00415-021-10923-w
Journal of Neurology volume 269, page 771 (2022)
Safety and outcome of mechanical thrombectomy in ischaemic stroke related to carotid artery dissection
A. Karam, N. Bricout, M. Khyeng, C. Cordonnier, X. Leclerc, H. Henon & B. Casolla
doi : 10.1007/s00415-021-10656-w
Journal of Neurology volume 269, pages 772–779 (2022)
The net clinical benefit of mechanical thrombectomy (MT) in patients with anterior circulation ischaemic stroke associated with large vessel occlusion (AIS-LVO) related to carotid artery dissection (CAD) is uncertain. The aim of the study was to investigate the safety and clinical outcomes of patients treated by MT for a CAD-related stroke.
The impact of device-assisted therapies on the gut microbiome in Parkinson’s disease
Michal Lubomski, Xiangnan Xu, Andrew J. Holmes, Jean Y. H. Yang, Carolyn M. Sue & Ryan L. Davis
doi : 10.1007/s00415-021-10657-9
Journal of Neurology volume 269, pages 780–795 (2022)
Microbiome feedbacks are proposed to influence Parkinson’s disease (PD) pathophysiology. A number of studies have evaluated the impact of oral medication on the gut microbiome (GM) in PD. However, the influence of PD device-assisted therapies (DATs) on the GM remains to be investigated.
What happens after fingolimod discontinuation? A multicentre real-life experience
Doriana Landi, Alessio Signori, Maria Cellerino, Giuseppe Fenu, Carolina Gabri Nicoletti, Marta Ponzano, Elisabetta Mancuso, Marzia Fronza, Maria Elena Ricchiuto, Giacomo Boffa, Matilde Inglese, Girolama Alessandra Marfia, Eleonora Cocco & Jessica Frau
doi : 10.1007/s00415-021-10658-8
Journal of Neurology volume 269, pages 796–804 (2022)
To analyse the course of multiple sclerosis (MS) after fingolimod withdrawal in a multicentre cohort.
Mitochondrial genome variations are associated with amyotrophic lateral sclerosis in patients from mainland China
Jie Ni, Zhen Liu, Yanchun Yuan, Wanzhen Li, Yiting Hu, Pan Liu, Xiaorong Hou, Xiangyu Zhu, Xuxiong Tang, Mingyu liang, Siqi Zheng, Xuan Hou, Juan Du, Jianguang Tang, Hong Jiang, Lu Shen, Beisha Tang & Junling Wang
doi : 10.1007/s00415-021-10659-7
Journal of Neurology volume 269, pages 805–814 (2022)
Amyotrophic lateral sclerosis (ALS) is a late-onset neurodegenerative disorder. Mitochondrial dysfunction is involved in the complex pathophysiology of ALS; however, the role of mitochondrial DNA (mtDNA) variants in ALS is poorly understood. We aimed to elucidate the role of mtDNA variants in the pathogenesis of ALS.
Serum neurofilament light chain or glial fibrillary acidic protein in the diagnosis and prognosis of brain metastases
Xinqing Lin, Tingting Lu, Haiyi Deng, Chunxin Liu, Yilin Yang, Tao Chen, Yinyin Qin, Xiaohong Xie, Zhanhong Xie, Ming Liu, Ming Ouyang, Shiyue Li, Yong Song, Nanshan Zhong, Wei Qiu & Chengzhi Zhou
doi : 10.1007/s00415-021-10660-0
Journal of Neurology volume 269, pages 815–823 (2022)
Brain metastases (BM) remains the most cumbersome disease burden in patients with lung cancer. This study aimed to investigate whether serum brain injury biomarkers can indicate BM, to further establish related diagnostic models, or to predict prognosis of BM.
Safety of alemtuzumab in a nationwide cohort of Finnish multiple sclerosis patients
Ilkka Rauma, Tiina Mustonen, Juha Matti Seppä, Maritta Ukkonen, Marianne Männikkö, Auli Verkkoniemi-Ahola, Marge Kartau, Jukka T. Saarinen, Liisa Luostarinen, Sakari Simula, Mervi Ryytty, Riitta Ahmasalo, Jussi O. T. Sipilä, Ilkka Pieninkeroinen, Tero Tapiola, Anne M. Remes & Hanna Kuusisto
doi : 10.1007/s00415-021-10664-w
Journal of Neurology volume 269, pages 824–835 (2022)
Alemtuzumab is an effective disease-modifying therapy (DMT) for highly active multiple sclerosis (MS). However, safety concerns limit its use in clinical practice.
NMOSD and MS prevalence in the Indigenous populations of Australia and New Zealand
Wajih Bukhari, Elham Khalilidehkordi, Deborah F. Mason, Michael H. Barnett, Bruce V. Taylor, Marzena Fabis-Pedrini, Allan G. Kermode, Sankar Subramanian, Patrick Waters, Simon A. Broadley on behalf of The Australian and New Zealand NMO Collaboration
doi : 10.1007/s00415-021-10665-9
Journal of Neurology volume 269, pages 836–845 (2022)
We studied the prevalence of neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) in Indigenous populations of Australia and New Zealand with the aim of assessing potential differences.
Optimizing the selection of Parkinson’s disease patients for neuromodulation using the levodopa challenge test
Dinkar Kulshreshtha, Marcus Pieterman, Greydon Gilmore & Mandar Jog
doi : 10.1007/s00415-021-10666-8
Journal of Neurology volume 269, pages 846–852 (2022)
In Parkinson’s disease (PD), early stages are associated with a good long-duration response and as the disease advances, the short-duration response predominates. The transition between the long-duration and short-duration responses may be an important and measurable intermediate stage. A critical criterion in determining the candidature for neuromodulation is a beneficial response to an ‘off–on’ levodopa challenge test. This test is usually reserved for those that have already developed marked short-duration response and are candidates for deep brain stimulation (DBS) surgery. However, identifying those that are in transition may allow DBS to be offered earlier.
Paradigm shift in acute dizziness: is caloric testing obsolete?
Miranda Morrison, Athanasia Korda, Ewa Zamaro, Franca Wagner, Marco D. Caversaccio, Thomas C. Sauter, Roger Kalla & Georgios Mantokoudis
doi : 10.1007/s00415-021-10667-7
Journal of Neurology volume 269, pages 853–860 (2022)
Cold and warm water ear irrigation, also known as bithermal caloric testing, has been considered for over 100 years the ‘Gold Standard’ for the detection of peripheral vestibular hypofunction. Its discovery was awarded a Nobel Prize. We aimed to investigate the diagnostic accuracy of Caloric Testing when compared to the video head impulse test (vHIT) in differentiating between vestibular neuritis and vestibular strokes in acute dizziness.
Prediagnosis epilepsy and survival in patients with glioma: a nationwide population-based cohort study from 2009 to 2018
Mirketa Marku, Birthe Krogh Rasmussen, Federica Belmonte, Steinbjørn Hansen, Elisabeth Anne Wreford Andersen, Christoffer Johansen & Pernille Envold Bidstrup
doi : 10.1007/s00415-021-10668-6
Journal of Neurology volume 269, pages 861–872 (2022)
Considering that epilepsy is common, and knowledge is lacking on its role especially for the prognosis of high-grade gliomas, the objective of this study was to investigate the association between epilepsy prior to glioma diagnosis and survival among glioma patients.
Traumatic brain injury fast-forwards Alzheimer’s pathology: evidence from amyloid positron emission tomorgraphy imaging
Abdalla Z. Mohamed, Peter J. Nestor, Paul Cumming & Fatima A. Nasrallah for the Alzheimer’s Disease Neuroimaging Initiative
doi : 10.1007/s00415-021-10669-5
Journal of Neurology volume 269, pages 873–884 (2022)
Traumatic brain injury (TBI) has been proposed as a risk factor for Alzheimer’s disease (AD), although the mechanisms underlying the putative association are poorly understood. We investigated elderly individuals with a remote history of TBI, aiming to understand how this may have influenced amyloidosis, neurodegeneration, and clinical expression along the AD continuum.
Randomized phase 2 study of perampanel for sporadic amyotrophic lateral sclerosis
Hitoshi Aizawa, Haruhisa Kato, Koji Oba, Takuya Kawahara, Yoshihiko Okubo, Tomoko Saito, Makiko Naito, Makoto Urushitani, Akira Tamaoka, Kiyotaka Nakamagoe, Kazuhiro Ishii, Takashi Kanda, Masahisa Katsuno, Naoki Atsuta, Yasushi Maeda, Makiko Nagai, Kazutoshi Nishiyama, Hiroyuki Ishiura, Tatsushi Toda, Akihiro Kawata, Koji Abe, Ichiro Yabe, Ikuko Takahashi-Iwata, Hidenao Sasaki, Hitoshi Warita, Masashi Aoki, Gen Sobue, Hidehiro Mizusawa, Yutaka Matsuyama, Tomohiro Haga & Shin Kwak
doi : 10.1007/s00415-021-10670-y
Journal of Neurology volume 269, pages 885–896 (2022)
To evaluate the efficacy and safety of perampanel in patients with sporadic amyotrophic lateral sclerosis (SALS).
Shorter visual aura characterizes young and middle-aged stroke patients with migraine with aura
Claudia Altamura, Angelo Cascio Rizzo, Giovanna Viticchi, Paola Maggio, Carmelina Maria Costa, Nicoletta Brunelli, Giuditta Giussani, Matteo Paolucci, Fabrizio Fiacco, Vincenzo Di Lazzaro, Elio Clemente Agostoni, Mauro Silvestrini & Fabrizio Vernieri
doi : 10.1007/s00415-021-10671-x
Journal of Neurology volume 269, pages 897–906 (2022)
To identify the clinical profile and aura characteristics of patients with Migraine with Aura (MwA) having acute cerebral ischemia, we compared stroke phenotype and risk factors in stroke patients with (S+MwA+) or without (S+MwA−) MwA and aura features in MwA patients with (S+MwA+) or without (S−MwA+) stroke.
Prolonged distal motor latency of median nerve does not improve diagnostic accuracy for CIDP
Emanuele Spina, Pietro Emiliano Doneddu, Giuseppe Liberatore, Dario Cocito, Raffaella Fazio, Chiara Briani, Massimiliano Filosto, Luana Benedetti, Giovanni Antonini, Giuseppe Cosentino, Stefano Jann, Anna Mazzeo, Andrea Cortese, Girolama Alessandra Marfia, Angelo Maurizio Clerici, Gabriele Siciliano, Marinella Carpo, Marco Luigetti, Giuseppe Lauria, Tiziana Rosso, Guido Cavaletti, Erdita Peci, Stefano Tronci, Marta Ruiz, Stefano Cotti Piccinelli, Angelo Schenone, Luca Leonardi, Luca Gentile, Laura Piccolo, Giorgia Mataluni, Lucio Santoro, Eduardo Nobile-Orazio, Fiore Manganelli on behalf of The Italian CIDP Database Study Group
doi : 10.1007/s00415-021-10672-w
Journal of Neurology volume 269, pages 907–912 (2022)
Compression of the median nerve at the carpal tunnel can give demyelinating features and result in distal motor latency (DML) prolongation fulfilling the EFNS/PNS demyelinating criteria for chronic inflammatory demyelinating polyneuropathy (CIDP). Accordingly, being carpal tunnel syndrome (CTS) common in the general population, the EFNS/PNS guidelines recommend excluding the DML of the median nerve when DML prolongation may be consistent with median neuropathy at the wrist from CTS. The main aims of this study were to verify whether the inclusion of DML of the median nerve (when consistent with CTS) could improve electrophysiological diagnostic accuracy for CIDP and if the median nerve at the carpal tunnel was more prone to demyelination. We analyzed electrophysiological data from 499 patients included consecutively into the Italian CIDP Database. According to the EFNS/PNS criteria, 352 patients had a definite, 10 a probable, and 57 a possible diagnosis of CIDP, while 80 were not fulfilling the diagnostic criteria. The inclusion of DML prolongation of median nerve did not improve significantly the diagnostic accuracy for CIDP; overall diagnostic class changed in 6 out of 499 patients (1.2%) and electrodiagnostic class of CIDP changed from not fulfilling to possible in only 2 patients (2.5% of not-fulfilling patients). In conclusion, we can infer that excluding DML prolongation of median nerve does not increase the risk of missing a diagnosis of CIDP thus corroborating the current EFNS/PNS criteria.
Initial treatment strategy and clinical outcomes in Finnish MS patients: a propensity-matched study
K. Hänninen, M. Viitala, S. Atula, S. M. Laakso, H. Kuusisto & M. Soilu-Hänninen
doi : 10.1007/s00415-021-10673-9
Journal of Neurology volume 269, pages 913–922 (2022)
The optimal treatment strategy with disease-modifying therapies (DMTs) in relapsing–remitting multiple sclerosis (RRMS) remains uncertain.
Quantitative sensory testing and norepinephrine levels in REM sleep behaviour disorder – a clue to early peripheral autonomic and sensory dysfunction?
Julia Koch, Kira Willemsen, Imis Dogan, Roman Rolke, Jörg B. Schulz, Johannes Schiefer, Kathrin Reetz & Andrea Maier
doi : 10.1007/s00415-021-10675-7
Journal of Neurology volume 269, pages 923–932 (2022)
Studies have reported autonomic impairment in patients with idiopathic REM sleep behaviour disorder (iRBD), which is considered a prodromal stage of alpha-synucleinopathies. It is still debated whether central or peripheral pathologies are first manifestations of alpha-synucleinopathies. This study aimed to characterize autonomic and somatosensory function in iRBD patients.
PML risk is the main factor driving the choice of discontinuing natalizumab in a large multiple sclerosis population: results from an Italian multicenter retrospective study
Clara G. Chisari, Giancarlo Comi, Massimo Filippi, Damiano Paolicelli, Pietro Iaffaldano, Mauro Zaffaroni, Vincenzo Brescia Morra, Eleonora Cocco, Girolama Alessandra Marfia, Luigi Maria Grimaldi, Matilde Inglese, Simona Bonavita, Alessandra Lugaresi, Giuseppe Salemi, Giovanna De Luca, Salvatore Cottone, Antonella Conte, Patrizia Sola, Umberto Aguglia, Giorgia Teresa Maniscalco, Claudio Gasperini, Maria Teresa Ferrò, Ilaria Pesci, Maria Pia Amato, Marco Rovaris, Claudio Solaro, Giacomo Lus, Davide Maimone, Roberto Bergamaschi, Franco Granella, Alessia Di Sapio, Antonio Bertolotto, Rocco Totaro, Marika Vianello, Paola Cavalla, Paolo Bellantonio, Vito Lepore, Francesco Patti & the Italian MS Register Study Group.
doi : 10.1007/s00415-021-10676-6
Journal of Neurology volume 269, pages 933–944 (2022)
Natalizumab (NTZ) is an effective treatment for relapsing–remitting multiple sclerosis (RRMS). However, patients and physicians may consider discontinuing NTZ therapy due to safety or efficacy issues. The aim of our study was to evaluate the NTZ discontinuation rate and reasons of discontinuation in a large Italian population of RRMS patients.
Clinical outcome of CIDP one year after start of treatment: a prospective cohort study
S. R. M. Bus, M. C. Broers, I. M. Lucke, C. Bunschoten, G. G. A. van Lieverloo, M. E. Adrichem, R. van Veen, L. Wieske, H. F. Lingsma, H. S. Goedee, W. L. van der Pol, I. N. van Schaik, P. A. Van Doorn, B. C. Jacobs & F. Eftimov for the ICOS Consortium
doi : 10.1007/s00415-021-10677-5
Journal of Neurology volume 269, pages 945–955 (2022)
To assess clinical outcome in treatment-naive patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
Initiation of antidepressants in young adults after ischemic stroke: a registry-based follow-up study
Jenna Broman, Karoliina Aarnio, Anna But, Ivan Marinkovic, Jorge RodrÃguez-Pardo, Markku Kaste, Turgut Tatlisumak & Jukka Putaala
doi : 10.1007/s00415-021-10678-4
Journal of Neurology volume 269, pages 956–965 (2022)
Data on post-stroke use of antidepressants in young individuals are scarce. We examined pattern and factors associated with initiating post-stroke antidepressants (PSAD) after ischemic stroke (IS) in young adults.
Cystatin C predicts futile recanalization in patients with acute ischemic stroke after endovascular treatment
Mouxiao Su, Ying Zhou, Zhonglun Chen, Mingjun Pu, Zhaokun Li, Hongcai Du & Gelin Xu
doi : 10.1007/s00415-021-10680-w
Journal of Neurology volume 269, pages 966–972 (2022)
A previous study reported that cystatin C was related to acute ischemic stroke. The association between cystatin C and the clinical outcome in acute ischaemic stroke patients with successful recanalization after endovascular thrombectomy has rarely been reported. This study aimed to evaluate the association between cystatin C and futile recanalization in AIS patients who underwent endovascular thrombectomy.
Pembrolizumab for treatment of progressive multifocal leukoencephalopathy in primary immunodeficiency and/or hematologic malignancy: a case series of five patients
Timo Volk, Klaus Warnatz, Reinhard Marks, Horst Urbach, Gisela Schluh, Valentina Strohmeier, Jessica Rojas-Restrepo, Bodo Grimbacher & Sebastian Rauer
doi : 10.1007/s00415-021-10682-8
Journal of Neurology volume 269, pages 973–981 (2022)
Progressive multifocal leukoencephalopathy is a rare opportunistic infection of the brain by John Cunningham polyomavirus in immune-compromised patients. In cases where no overt option for immune reconstitution is available [e.g., in patients with primary immunodeficiency (PID)], the disease is lethal in the majority of patients. Immune checkpoint inhibition has been applied in recent years with mixed outcomes. We present four novel patients and the follow-up of a previously published patient suffering from progressive multifocal leukoencephalopathy (PML) due to PID and/or hematologic malignancy who were treated with the immune checkpoint inhibitor pembrolizumab. In two patients with PID, symptoms improved and stabilized. One patient died because of worsening PML another of intracranial hemorrhage which was unrelated to PML or its treatment with pembrolizumab. The fifth patient suffered from PID and died of a pre-existing immune dysregulation, possibly exacerbated by pembrolizumab. The long-term follow-up of the first patient provides support for therapeutic decisions during this therapy and is the longest published clinical course of a patient with checkpoint inhibition for PML. We conclude that pembrolizumab can control PML symptoms long term in a subgroup of patients with PID, in our cases for 21 and 36Â months. However, therapy must be started early because symptoms are only partially reversible. In light of severe adverse events, application of pembrolizumab is only justified if the prognosis for the individual patient is very poor.
Diagnosis and follow-up evaluation of central nervous system vasculitis: an evaluation of vessel-wall MRI findings
Maximilian Patzig, Robert Forbrig, Clemens Küpper, Ozan Eren, Tobias Saam, Lars Kellert, Thomas Liebig & Florian Schöberl
doi : 10.1007/s00415-021-10683-7
Journal of Neurology volume 269, pages 982–996 (2022)
To approach the clinical value of MRI with vessel wall imaging (VWI) in patients with central nervous system vasculitis (CNSV), we analyzed patterns of VWI findings both at the time of initial presentation and during follow-up.
The angiographic presentation of European Moyamoya angiopathy
Sara Pilgram-Pastor, René Chapot & Markus Kraemer
doi : 10.1007/s00415-021-10684-6
Journal of Neurology volume 269, pages 997–1006 (2022)
Little is known about the angiographic presentation of Moyamoya angiopathy (MMA) in non-Asian patients.
Blink reflex recovery cycle distinguishes patients with idiopathic normal pressure hydrocephalus from elderly subjects
Alessandro Mechelli, Andrea Quattrone, Rita Nisticò, Marianna Crasà , Domenico La Torre, Basilio Vescio & Aldo Quattrone
doi : 10.1007/s00415-021-10687-3
Journal of Neurology volume 269, pages 1007–1012 (2022)
The R2 component of blink reflex recovery cycle (R2BRrc) is a simple neurophysiological tool to detect the brainstem hyperexcitability commonly occurring in several neurological diseases such as Parkinson’s disease and atypical parkinsonisms. In our study, we investigated for the first time the usefulness of R2BRrc to assess brainstem excitability in patients with idiopathic Normal Pressure Hydrocephalus (iNPH) in comparison with healthy subjects.
Association of the Careggi Collateral Score with 3-month modified Rankin Scale score after thrombectomy for stroke with occlusion of the middle cerebral artery
Manuel Cappellari, Valentina Saia, Giovanni Pracucci, Fainardi Enrico, Arturo Consoli, Sergio Nappini, Lucio Castellan, Sandra Bracco, Mauro Bergui, Mirco Cosottini, Alessandra Briatico Vangosa, Sergio Vinci, Maria Ruggiero, Edoardo Puglielli, Luigi Chiumarulo, Giacomo Cester, Chiara Comelli, Umberto Silvagni, Daniele Morosetti, Valentina Caldiera, Nicola Cavasin, Valeria Ledda, Giuseppina Sanfilippo, Andrea Saletti, Pietro Filauri, Ivan Gallesio, Nunzio Paolo Nuzzi, Pitero Amistá, Cecilia Zivelonghi, Mauro Plebani, Marco Pavia, Daniele Romano, Francesco Biraschi, Roberto Menozzi, Roberto Gasparotti, Andrea Giorgianni, Andrea Zini, Domenico Inzitari, Danilo Toni, Salvatore Mangiafico on behalf of The IRETAS Group
doi : 10.1007/s00415-021-10898-8
Journal of Neurology volume 269, pages 1013–1023 (2022)
The Careggi Collateral Score (CCS) (qualitative–quantitative evaluation) was developed from a single-centre cohort as an angiographic score to describe both the extension and effectiveness of the pial collateral circulation in stroke patients with occlusion of the anterior circulation. We aimed to examine the association between CCS (quantitative evaluation) and 3-month modified Rankin Scale (mRS) score in a large multi-center cohort of patients receiving thrombectomy for stroke with occlusion of middle cerebral artery (MCA).
Correction to: Association of the Careggi Collateral Score with 3-month modified Rankin Scale score after thrombectomy for stroke with occlusion of the middle cerebral artery
Manuel Cappellari, Valentina Saia, Giovanni Pracucci, Enrico Fainardi, Arturo Consoli, Sergio Nappini, Lucio Castellan, Sandra Bracco, Mauro Bergui, Mirco Cosottini, Alessandra Briatico Vangosa, Sergio Vinci, Maria Ruggiero, Edoardo Puglielli, Luigi Chiumarulo, Giacomo Cester, Chiara Comelli, Umberto Silvagni, Daniele Morosetti, Valentina Caldiera, Nicola Cavasin, Valeria Ledda, Giuseppina Sanfilippo, Andrea Saletti, Pietro Filauri, Ivan Gallesio, Nunzio Paolo Nuzzi, Pitero Amistá, Cecilia Zivelonghi, Mauro Plebani, Marco Pavia, Daniele Romano, Francesco Biraschi, Roberto Menozzi, Roberto Gasparotti, Andrea Giorgianni, Andrea Zini, Domenico Inzitari, Danilo Toni, Salvatore Mangiafico on behalf of The IRETAS Group
doi : 10.1007/s00415-021-10941-8
Journal of Neurology volume 269, pages 1024–1025 (2022)
Ocular flutter is a rare and treatment-responsive symptom in Lyme neuroborreliosis: a case report
Fiona Jäger, Stefan Greisenegger, Felix K. Schwarz & Gerald Wiest
doi : 10.1007/s00415-021-10769-2
Journal of Neurology volume 269, pages 1026–1027 (2022)
Paroxysmal central positional nystagmus responsive to clonazepam
Eun Hye Oh, Hyunsung Kim, Seo-Young Choi, Kwang-Dong Choi & Jae-Hwan Choi
doi : 10.1007/s00415-021-10770-9
Journal of Neurology volume 269, pages 1028–1031 (2022)
When should we consider chronic patients as non-responders to monoclonal antibodies targeting the CGRP pathway?
Claudia Altamura, Sabina Cevoli, Nicoletta Brunelli, Cinzia Aurilia, Luisa Fofi, Gabriella Egeo, Piero Barbanti, Fabrizio Vernieri
doi : 10.1007/s00415-021-10772-7
Journal of Neurology volume 269, pages 1032–1034 (2022)
Pathogenic events in very early Guillain–Barré syndrome: neither demyelination nor axonal degeneration but endoneurial inflammatory oedema
José BercianoÂ
doi : 10.1007/s00415-021-10773-6
Journal of Neurology volume 269, pages 1035–1037 (2022)
Alzheimer’s disease clinical trial update 2019–2021
Joseph Pleen & Ryan Townley
doi : 10.1007/s00415-021-10790-5
Journal of Neurology volume 269, pages 1038–1051 (2022)
The current clinical trial landscape targeting Alzheimer’s disease (AD) is reviewed in the context of studies completed from 2019 to 2021. This review focuses on available data for observational and phase II/III clinical trial results, which will have the most impact on the field. ClinicalTrials.gov, the United States (US) comprehensive federal registry, was queried to identify completed trials. There are currently 226 interventional clinical trials and 51 observational studies completed, suspended, terminated, or withdrawn within our selected time frame. This review reveals that the role of biomarkers is expanding and although many lessons have been learned, many challenges remain when targeting disease modification of AD through amyloid and tau. In addition, to halt or slow clinical progression of AD, new clinical and observational trials are focusing on prevention as well as the role of more diverse biological processes known to influence AD pathology.
Effects on cognition of DMTs in multiple sclerosis: moving beyond the prevention of inflammatory activity
Paolo Preziosa, Lorenzo Conti, Maria A. Rocca & Massimo Filippi
doi : 10.1007/s00415-021-10832-y
Journal of Neurology volume 269, pages 1052–1064 (2022)
In this review, we critically summarize recent findings derived from randomized controlled trials (RCTs), observational studies and meta-analyses that have been published in the last 3Â years and that included the effects of DMTs on cognitive performances among their outcomes.
Advances in the use of stem cell transplants in the treatment of multiple sclerosis
Rachel Thomas, Ray Wynford-Thomas & Neil P. Robertson
doi : 10.1007/s00415-021-10927-6
Journal of Neurology volume 269, pages 1065–1067 (2022)
Carlo Francesco Giuseppe Bellingeri (1785–1843)
Lorenzo Lorusso, Stefano Zago & Stefano Sandrone
doi : 10.1007/s00415-021-10732-1
Journal of Neurology volume 269, pages 1068–1069 (2022)
