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SARS-CoV-2 infection and acute ischemic stroke in Lombardy, Italy
Alessandro Pezzini ,Mario Grassi
doi : 10.1007/s00415-021-10620-8
Journal of Neurology volume 269, pages 1–11 (2022)
To characterize patients with acute ischemic stroke related to SARS-CoV-2 infection and assess the classification performance of clinical and laboratory parameters in predicting in-hospital outcome of these patients.
Altered mental status in COVID-19
Daniel Antoniello, Mark J. Milstein, Joseph Dardick, Jenelys Fernandez-Torres, Jenny Lu, Nikunj Patel & Charles Esenwa
doi : 10.1007/s00415-021-10623-5
Journal of Neurology volume 269, pages 12–18 (2022)
Altered mental status (AMS) is a common neurological manifestation of COVID-19 infection in hospitalized patients. The principal causes of AMS have yet to be determined. We aimed to identify the common causes of AMS in patients with COVID-19 presenting to the emergency department with AMS on arrival.
Impact of the COVID-19 lockdown on the adherence of stroke patients to direct oral anticoagulants: a secondary analysis from the MAAESTRO study
Fine Dietrich, Alexandros A. Polymeris, Melina Verbeek, Stefan T. Engelter, Kurt E. Hersberger, Sabine Schaedelin, Isabelle Arnet & Philippe A. Lyrer
doi : 10.1007/s00415-021-10631-5
Journal of Neurology volume 269, pages 19–25 (2022)
The negative impact of the COVID-19 outbreak on stroke care has been reported, but no data exist on the influence of the lockdown on medication adherence to antithrombotic treatment for stroke prevention. We present a comparison of electronic adherence data of stroke patients treated with direct oral anticoagulants (DOAC) prior to and during the COVID-19 lockdown in spring 2020 in Switzerland.
Global survey on disruption and mitigation of neurological services during COVID-19: the perspective of global international neurological patients and scientific associations
Chahnez Charfi Triki, Matilde Leonardi, Salma Zouari Mallouli, Martina Cacciatore, Kimberly Coard Karlshoej, Francesca Giulia Magnani, Charles R. Newton, Andrea Pilotto, Deanna Saylor, Erica Westenberg, Donna Walsh, Andrea Sylvia Winkler, Kiran T. Thakur, Njideka U. Okubadejo & David Garcia-Azorin
doi : 10.1007/s00415-021-10641-3
Journal of Neurology volume 269, pages 26–38 (2022)
The COVID-19 pandemic outbreak has dramatically disrupted healthcare systems. Two rapid WHO pulse surveys studied disruptions in mental health services, but did not particularly focus on neurology. Here, a global survey was conducted and addresses the impact of the pandemic on neurology services.
Serological response to SARS-CoV-2 vaccination in multiple sclerosis patients treated with fingolimod or ocrelizumab: an initial real-life experience
Guerrieri S., Lazzarin S., Zanetta C., Nozzolillo A., Filippi M. & Moiola L.
doi : 10.1007/s00415-021-10663-x
Journal of Neurology volume 269, pages 39–43 (2022)
Recent observations suggest a lack of humoral response after SARS-CoV-2 vaccination in multiple sclerosis (MS) patients treated with fingolimod or ocrelizumab
Long COVID: cognitive complaints (brain fog) and dysfunction of the cingulate cortex
Jacques Hugon, Eva-Flore Msika, Mathieu Queneau, Karim Farid & Claire Paquet
doi : 10.1007/s00415-021-10655-x
Journal of Neurology volume 269, pages 44–46 (2022)
Many patients who have suffered from acute COVID infections have long-lasting symptoms affecting several organs including the brain. This long COVID status can include “brain fog� and cognitive deficits that can disturb activities of daily living and can delay complete recovery. Here, we report two cases of neurological long COVID with abnormal FDG PET findings marked by hypometabolic regions of the cingulate cortex.
Bell’s palsy following a single dose of mRNA SARS-CoV-2 vaccine: a case report
C. Martin-Villares, A. Vazquez-Feito, M. J. Gonzalez-Gimeno & B. de la Nogal-Fernandez
doi : 10.1007/s00415-021-10617-3
Journal of Neurology volume 269, pages 47–48 (2022)
Acute bilateral optic/chiasm neuritis with longitudinal extensive transverse myelitis in longstanding stable multiple sclerosis following vector-based vaccination against the SARS-CoV-2
Christoph Helmchen, Gesine M. Buttler, Robert Markewitz, Katja Hummel, Heinz Wiendl & Tobias Boppel
doi : 10.1007/s00415-021-10647-x
Journal of Neurology volume 269, pages 49–54 (2022)
First manifestation of multiple sclerosis after immunization with the Pfizer-BioNTech COVID-19 vaccine
Joachim Havla, Yannick Schultz, Hanna Zimmermann, Reinhard Hohlfeld, Adrian Danek & Tania Kümpfel
doi : 10.1007/s00415-021-10648-w
Journal of Neurology volume 269, pages 55–58 (2022)
COVID-19 and Parkinson’s disease: a casual association or a possible second hit in neurodegeneration?
Francesco Cavallieri, Valentina Fioravanti, Giulia Toschi, Sara Grisanti, Manuela Napoli, Claudio Moratti, Rosario Pascarella, Annibale Versari, Alessandro Fraternali, Massimiliano Casali, Jefri J. Paul, Elena Moro, Peter Bauer, Marialuisa Zedde & Franco Valzania
doi : 10.1007/s00415-021-10694-4
Journal of Neurology volume 269, pages 59–61 (2022)
Placebo response in degenerative cerebellar ataxias: a descriptive review of randomized, placebo-controlled trials
Ji-Hyun Choi, Chaewon Shin, Han-Joon Kim & Beomseok Jeon
doi : 10.1007/s00415-020-10306-7
Journal of Neurology volume 269, pages 62–71 (2022)
Placebo response in degenerative cerebellar ataxias (CAs) has never been studied despite the large number of randomized controlled trials (RCTs) that have been conducted. In this descriptive review, we aimed to examine the placebo response in patients with CAs. We performed a literature search on PubMed for RCTs on CAs that were published from 1977 to January 2020 and collected data on the changes from the baseline to the endpoint on various objective ataxia-associated clinical rating scales. We reviewed 56 clinical trials, finally including 35 parallel-group studies and excluding 21 cross-over studies. The included studies were categorized as follows: (1) studies showing significant improvements in one or more ataxia scales in the placebo groups (n = 3); (2) studies reporting individual placebo responders with improvements in one or more ataxia scales in the placebo groups (n = 5)—the overall proportion of placebo responders was 31.9%; (3) studies showing mean changes in the direction of improvement in at least one ataxia scale in the placebo groups, though not statistically significant (n = 19); (4) studies showing no placebo response in any of the ataxia scales in the placebo groups (n = 4); (5) studies where data on the placebo groups were unavailable (n = 9). This review demonstrated the placebo response in patients with CAs on various objective ataxia scales. Our study emphasizes that the placebo response should be considered when designing, analyzing, and interpreting clinical trials and in clinical practice in CA patients.
Effects of intratympanic gentamicin and intratympanic glucocorticoids in Ménière’s disease: a network meta-analysis
Weiming Hao, Huiqian Yu & Huawei Li
doi : 10.1007/s00415-020-10320-9
Journal of Neurology volume 269, pages 72–86 (2022)
Intratympanic therapies, usually including glucocorticoid and gentamicin, are becoming worldwide used in clinical practice of Ménière’s disease today. However, clinical efficacy and safety of these two therapies are still in controversial.
Hearing loss versus vestibular loss as contributors to cognitive dysfunction
Paul F. SmithÂ
doi : 10.1007/s00415-020-10343-2
Journal of Neurology volume 269, pages 87–99 (2022)
In the last 5Â years, there has been a surge in evidence that hearing loss (HL) may be a risk factor for cognitive dysfunction, including dementia. At the same time, there has been an increase in the number of studies implicating vestibular loss in cognitive dysfunction. Due to the fact that vestibular disorders often present with HL and other auditory disorders such as tinnitus, it has been suggested that, in many cases, what appears to be vestibular-related cognitive dysfunction may be due to HL (e.g., Dobbels et al. Front Neurol 11:710, 2020). This review analyses the studies of vestibular-related cognitive dysfunction which have controlled HL. It is suggested that despite the fact that many studies in the area have not controlled HL, many other studies have (~ 19/44 studies or 43%). Therefore, although there is certainly a need for further studies controlling HL, there is evidence to suggest that vestibular loss is associated with cognitive dysfunction, especially related to spatial memory. This is consistent with the overwhelming evidence from animal studies that the vestibular system transmits specific types of information about self-motion to structures such as the hippocampus.
Wearables in the home-based assessment of abnormal movements in Parkinson’s disease: a systematic review of the literature
Stefania Ancona, Francesca D. Faraci, Elina Khatab, Luigi Fiorillo, Oriella Gnarra, Tobias Nef, Claudio L. A. Bassetti & Panagiotis Bargiotas
doi : 10.1007/s00415-020-10350-3
Journal of Neurology volume 269, pages 100–110 (2022)
At present, the standard practices for home-based assessments of abnormal movements in Parkinson’s disease (PD) are based either on subjective tools or on objective measures that often fail to capture day-to-day fluctuations and long-term information in real-life conditions in a way that patient’s compliance and privacy are secured. The employment of wearable technologies in PD represents a great paradigm shift in healthcare remote diagnostics and therapeutics monitoring. However, their applicability in everyday clinical practice seems to be still limited. We carried out a systematic search across the Medline Database. In total, 246 publications, published until 1 June 2020, were identified. Among them, 26 reports met the inclusion criteria and were included in the present review. We focused more on clinically relevant aspects of wearables’ application including feasibility and efficacy of the assessment, the number, type and body position of the wearable devices, type of PD motor symptom, environment and duration of assessments and validation methodology. The aim of this review is to provide a systematic overview of the current knowledge and state-of-the-art of the home-based assessment of motor symptoms and fluctuations in PD patients using wearable technology, highlighting current problems and laying foundations for future works.
The changing landscape of optic neuritis: a narrative review
Lindsey B. De Lott, Jeffrey L. Bennett & Fiona Costello
doi : 10.1007/s00415-020-10352-1
Journal of Neurology volume 269, pages 111–124 (2022)
Optic neuritis (ON) is an inflammatory optic neuropathy that is often a harbinger of central nervous system (CNS) demyelinating disorders. ON is frequently misdiagnosed in the clinical arena, leading to either inappropriate management or diagnostic delays. As a result, patients may fail to achieve optimal recovery. The treatment response to corticosteroids and long term risk of multiple sclerosis was established in the first clinical trials conducted roughly 30Â years ago. Spontaneous resolution was observed in the vast majority of patients and intravenous high-dose corticosteroids hastened recovery; half of the patients eventually developed multiple sclerosis. Over the ensuing decades, the number of inflammatory conditions associated with ON has significantly expanded exposing substantial variability in the prognosis, treatment, and management of ON patients. ON subtypes can frequently be distinguished by distinct clinical, serological, and radiological profiles allowing expedited and specialized treatment. Guided by an increased understanding of the immunopathology underlying optic nerve and associated CNS injuries, novel disease management strategies are emerging to minimize vision loss, improve long-term surveillance strategies, and minimize CNS injury and disability. Knowledge regarding the clinical signs and symptoms of different ON subtypes is essential to guide acute therapy, prognosticate recovery, accurately identify underlying CNS inflammatory disorders, and facilitate study design for the next generation of clinical and translational trials.
A critical review of the pharmacological treatment of REM sleep behavior disorder in adults: time for more and larger randomized placebo-controlled trials
Moran Gilat, Nathaniel S. Marshall, Dries Testelmans, Bertien Buyse & Simon J. G. Lewis
doi : 10.1007/s00415-020-10353-0
Journal of Neurology volume 269, pages 125–148 (2022)
Rapid Eye Movement sleep behavior disorder (RBD) is a parasomnia causing sufferers to physically act out their dreams. These behaviors can disrupt sleep and sometimes lead to injuries in patients and their bed-partners. Clonazepam and melatonin are the first-line pharmacological treatment options for RBD based on direct uncontrolled clinical observations and very limited double-blind placebo-controlled trials. Given the risk for adverse outcomes, especially in older adults, it is of great importance to assess the existing level of evidence for the use of these treatments. In this update, we therefore critically review the clinical and scientific evidence on the pharmacological management of RBD in people aged over 50. We focus on the first-line treatments, and provide an overview of all other alternative pharmacological agents trialed for RBD we could locate as supplementary materials. By amalgamating all clinical observations, our update shows that 66.7% of 1,026 RBD patients reported improvements from clonazepam and 32.9% of 137 RBD patients reported improvements from melatonin treatment on various outcome measures in published accounts. Recently, however, three relatively small randomized placebo-controlled trials did not find these agents to be superior to placebo. Given clonazepam and melatonin are clinically assumed to majorly modify or eliminate RBD in nearly all patients—there is an urgent need to test whether this magnitude of treatment effect remains intact in larger placebo-controlled trials.
Strokectomy for malignant middle cerebral artery infarction: experience and meta-analysis of current evidence
Saad Moughal, Sarah Trippier, Alaa AL-Mousa, Atticus H. Hainsworth, Anthony C. Pereira, Pawanjit S Minhas & Anan Shtaya
doi : 10.1007/s00415-020-10358-9
Journal of Neurology volume 269, pages 149–158 (2022)
Strokectomy means surgical excision of infarcted brain tissue post-stroke with preservation of skull integrity, distinguishing it from decompressive hemicraniectomy. Both can mitigate malignant middle cerebral artery (MCA) syndrome but evidence regarding strokectomy is sparse. Here, we report our data and meta-analysis of strokectomy compared to hemicraniectomy for malignant MCA infarction. All malignant MCA stroke cases requiring surgical intervention in a large tertiary centre (January 2012–December 2017, N = 24) were analysed for craniotomy diameter, complications, length of follow-up and outcome measured using the modified Rankin score (mRS). Good outcome was defined as mRS 0–3 at 12 months. In a meta-analysis, outcome from strokectomy (pooled from our cohort and published strokectomy studies) was compared with hemicraniectomy (our cohort pooled with published DECIMAL, DESTINY and HAMLET clinical trial data). In our series (N = 24, 12/12 F/M; mean age: 45.83 ± 8.91, range 29–63 years), 4 patients underwent strokectomy (SC) and 20 hemicraniectomy (HC). Among SC patients, craniotomy diameter was smaller, relative to HC patients (86 ± 13.10 mm, 120 ± 4.10 mm, respectively; p = 0.003), complications were less common (25%, 55%) and poor outcomes were less common (25%, 70%). In the pooled data (N = 41 SC, 71 HC), strokectomy tended towards good outcome more than hemicraniectomy (OR 2.2, 95% CI 0.99–4.7; p = 0.051). In conclusion, strokectomy may be non-inferior, lower risk and cost saving relative to hemicraniectomy sufficiently to be worthy of further investigation and maybe a randomised trial.
Rituximab for the treatment of multiple sclerosis: a review
Clara Grazia Chisari, Eleonora Sgarlata, Sebastiano Arena, Simona Toscano, Maria Luca & Francesco Patti
doi : 10.1007/s00415-020-10362-z
Journal of Neurology volume 269, pages 159–183 (2022)
In the last decades, evidence suggesting the direct or indirect involvement of B cells on multiple sclerosis (MS) pathogenesis has accumulated. The increased amount of data on the efficacy and safety of B-cell-depleting therapies from several studies has suggested the addition of these drugs as treatment options to the current armamentarium of disease modifying therapies (DMTs) for MS. Particularly, rituximab (RTX), a chimeric monoclonal antibody directed at CD20 positive B lymphocytes resulting in cell-mediated apoptosis, has been demonstrated to reduce inflammatory activity, incidence of relapses and new brain lesions on magnetic resonance imaging (MRI) in patients with relapsing–remitting MS (RRMS). Additional evidence also demonstrated that patients with progressive MS (PMS) may benefit from RTX, which also showed to be well tolerated, with acceptable safety risks and favorable cost-effectiveness profile.
Migraine in transient global amnesia: a meta-analysis of observational studies
Ioannis Liampas, Athanasios S. Siouras, Vasileios Siokas, Zisis Tsouris, Dimitrios Rikos, Alexandros Brotis, Athina-Maria Aloizou, Metaxia Dastamani & Efthimios Dardiotis
doi : 10.1007/s00415-020-10363-y
Journal of Neurology volume 269, pages 184–196 (2022)
Although many studies have investigated the relationship between transient global amnesia (TGA) and migraine, to date, no meta-analysis has confirmed the existence and size of their association.
Parameters for subthalamic deep brain stimulation in patients with dystonia: a systematic review
Yuhan Wang, Chencheng Zhang, Bomin Sun, Dianyou Li & Yiwen Wu
doi : 10.1007/s00415-020-10372-x
Journal of Neurology volume 269, pages 197–204 (2022)
Deep brain stimulation (DBS) is used for treating dystonia, commonly targeting the subthalamic nucleus (STN). Optimal stimulation parameters are required to achieve satisfying results. However, recommended parameters for STN-DBS remain to be identified. In this review, we aimed to assess the optimal stimulation parameters by analyzing previously published STN-DBS data of patients with dystonia.
Effect of melatonin supplementation on sleep quality: a systematic review and meta-analysis of randomized controlled trials
Gholami Fatemeh, Moradi Sajjad, Rasaei Niloufar, Soveid Neda, Setayesh Leila & Mirzaei Khadijeh
doi : 10.1007/s00415-020-10381-w
Journal of Neurology volume 269, pages 205–216 (2022)
The Present study was conducted to systematically review the effect of the melatonin on sleep quality. We summarized evidence from randomized clinical trials (RCTs) that investigated the effects of melatonin on sleep quality as assessed by the Pittsburgh Sleep Quality Index (PSQI) in adults with various diseases.
Bedside video-ophthalmoscopy as an aid in the diagnosis of central vestibular syndromes
Victor Rodrigues Pereira Evangelista, Sofia Abreu Mermelstein, Marcos Martins da Silva & Diego Kaski
doi : 10.1007/s00415-020-10389-2
Journal of Neurology volume 269, pages 217–220 (2022)
The evaluation of central vestibular syndromes, especially in the acute setting, can pose a challenge even for the most experienced clinician. Of particular importance is the evaluation of ocular torsion and nystagmus, which can be sensitive for central vestibular pathology, but easily missed by the untrained eye. We present two cases of acute vestibular syndrome of central origin in which the use of magnified fundoscopy at the bedside aided the precise anatomical diagnosis to inform appropriate further management. We also review aspects of the pathophysiology and anatomy of vestibular roll plane disorders. In case 1, the finding of position-dependent ocular torsion facilitated a rapid distinction between central skew deviation and a trochlear nerve palsy. In case 2, the fundoscopic magnification identified a pure rotatory nystagmus indicative of a central vestibular disorder. Ophthalmoscopy remains a useful bedside technique in acute vertigo, but the use of inexpensive magnification with a smartphone can provide objective and recordable evidence of central vestibular pathology, aiding therapeutic decisions.
Functional connectivity of hippocampus in temporal lobe epilepsy depends on hippocampal dominance: a systematic review of the literature
Camille K. Milton, Christen M. O’Neal & Andrew K. Conner
doi : 10.1007/s00415-020-10391-8
Journal of Neurology volume 269, pages 221–232 (2022)
Lateralized alterations in hippocampal function in the resting-state have been demonstrated for patients with temporal lobe epilepsy (TLE). However, resting-state fMRI of the hippocampus has yet to be substantiated as an adjunct to standard pre-operative assessments of the seizure focus.
Multiverse to inform neurological research: an example using recovery outcome of neglect
Margaret J. Moore & Nele Demeyere
doi : 10.1007/s00415-021-10612-8
Journal of Neurology volume 269, pages 233–242 (2022)
Multiverse analysis provides an ideal tool for understanding how inherent, yet ultimately arbitrary methodological choices impact the conclusions of individual studies. With this investigation, we aimed to demonstrate the utility of multiverse analysis for evaluating generalisability and identifying potential sources of bias within studies employing neurological populations.
Periorbital injections of botulinum toxin a: a novel therapeutic option for convergence spasm in neuropsychiatric disorders
Kristina Hess, Moritz Schmitt & Bettina Wabbels
doi : 10.1007/s00415-021-10613-7
Journal of Neurology volume 269, pages 243–250 (2022)
Convergence spasm (CS, spasm of near reflex) is characterized by transient attacks of convergence, miosis and accommodation, often associated with functional neurological disorders. To date, no simple and efficient treatment option is available for CS. This study investigates whether periorbital botulinum toxin injections as used in essential blepharospasm are also a treatment option in these patients.
Music to move persons with Parkinson’s disease: a personalized approach
Susanne Ten Holter, Jorik Nonnekes & Bastiaan Bloem
doi : 10.1007/s00415-021-10615-5
Journal of Neurology volume 269, pages 251–252 (2022)
Subthalamic deep brain stimulation affects heading perception in Parkinson’s disease
Sinem Balta Beylergil, Angela M. Noecker, Mikkel Petersen, Palak Gupta, Sarah Ozinga, Mark F. Walker, Camilla Kilbane, Cameron C. McIntyre & Aasef G. Shaikh
doi : 10.1007/s00415-021-10616-4
Journal of Neurology volume 269, pages 253–268 (2022)
Parkinson’s disease (PD) presents with visuospatial impairment and falls. It is critical to understand how subthalamic deep brain stimulation (STN DBS) modulates visuospatial perception. We hypothesized that DBS has different effects on visual and vestibular perception of linear motion (heading), a critical aspect of visuospatial navigation; and such effects are specific to modulated STN location. Two-alternative forced-choice experiments were performed in 14 PD patients with bilateral STN DBS and 19 age-matched healthy controls (HC) during passive en bloc linear motion and 3D optic-flow in immersive virtual reality measured vestibular and visual heading. Objective measure of perception with Weibull psychometric function revealed that PD has significantly lower accuracy [L: 60.71 (17.86)%, R: 74.82 (17.44)%] and higher thresholds [L: 16.68 (12.83), R: 10.09 (7.35)] during vestibular task in both directions compared to HC (p < 0.05). DBS significantly improved vestibular discrimination accuracy [81.40 (14.36)%] and threshold [4.12 (5.87), p < 0.05] in the rightward direction. There were no DBS effects on the slopes of vestibular psychometric curves. Visual heading perception was better than vestibular and it was comparable to HC. There was no significant effect of DBS on visual heading response accuracy or discrimination threshold (p > 0.05). Patient-specific DBS models revealed an association between change in vestibular heading perception and the modulation of the dorsal STN. In summary, DBS may have different effects on vestibular and visual heading perception in PD. These effects may manifest via dorsal STN putatively by its effects on the cerebellum.
Elucidating distinct clinico-radiologic signatures in the borderland between neuromyelitis optica and multiple sclerosis
Maciej Jurynczyk, Elzbieta Klimiec-Moskal, Yazhuo Kong, Samuel Hurley, Silvia Messina, Tianrong Yeo, Mark Jenkinson, Maria Isabel Leite & Jacqueline Palace
doi : 10.1007/s00415-021-10619-1
Journal of Neurology volume 269, pages 269–279 (2022)
Separating antibody-negative neuromyelitis optica spectrum disorders (NMOSD) from multiple sclerosis (MS) in borderline cases is extremely challenging due to lack of biomarkers. Elucidating different pathologies within the likely heterogenous antibody-negative NMOSD/MS overlap syndrome is, therefore, a major unmet need which would help avoid disability from inappropriate treatment.
Anti-HMGCR myopathy overlaps with dermatomyositis-like rash: a distinct subtype of idiopathic inflammatory myopathy
Ying Hou, Kai Shao, Yaping Yan, Tingjun Dai, Wei Li, Yuying Zhao, Duoling Li, Jian-Qiang Lu, Gary L. Norman & Chuanzhu Yan
doi : 10.1007/s00415-021-10621-7
Journal of Neurology volume 269, pages 280–293 (2022)
To characterize the clinical and pathological features of anti-HMGCR myopathy.
Capturing vertigo in the emergency room: three tools to double the rate of diagnosis
Benjamin Nham, Nicole Reid, Kendall Bein, Andrew P. Bradshaw, Leigh A. McGarvie, Emma C. Argaet, Allison S. Young, Shaun R. Watson, G. Michael Halmagyi, Deborah A. Black & Miriam S. Welgampola
doi : 10.1007/s00415-021-10627-1
Journal of Neurology volume 269, pages 294–306 (2022)
Many patients attending the emergency room (ER) with vertigo, leave without a diagnosis. We assessed whether the three tools could improve ER diagnosis of vertigo.
Sex-specific differences in presentations and determinants of outcomes after endovascular thrombectomy for large vessel occlusion stroke
Robert W. Regenhardt, Ashby C. Turner, Joshua A. Hirsch, Michael J. Young, Naif M. Alotaibi, Christopher J. Stapleton, Aman B. Patel, Thabele M. Leslie-Mazwi, Natalia S. Rost & Mark R. Etherton
doi : 10.1007/s00415-021-10628-0
Journal of Neurology volume 269, pages 307–315 (2022)
Sex-specific differences in ischemic stroke outcomes are prevalent. We sought to investigate sex differences in the determinants of reperfusion and functional outcomes after endovascular thrombectomy (EVT) for emergent large vessel occlusion ischemic stroke (ELVO).
Alterations of the structural covariance network in the hypothalamus of patients with cluster headache
Dong Ah Lee, Ho-Joon Lee, Hyung Chan Kim & Kang Min Park
doi : 10.1007/s00415-021-10629-z
Journal of Neurology volume 269, pages 316–322 (2022)
The hypothalamus is one of the key structures involved in the pathophysiology of cluster headaches. This study aimed to analyze the volume of hypothalamic subunits and structural covariance networks in the hypothalamus of patients with cluster headache.
Responder analysis for neuropathic impairment and quality-of-life assessment in patients with hereditary transthyretin amyloidosis with polyneuropathy in the NEURO-TTR study
Aaron Yarlas, Andrew Lovley, Duncan Brown, Mark Kosinski & Montserrat Vera-Llonch
doi : 10.1007/s00415-021-10635-1
Journal of Neurology volume 269, pages 323–335 (2022)
Hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN) is a rare disease characterized by rapid neuropathic progression. In pivotal studies of gene-silencing treatments, the modified Neuropathy Impairment Score + 7 tests (mNIS + 7) and Norfolk-Quality of Life (QOL)-Diabetic Neuropathy (DN) questionnaire assessed treatment impact on neuropathic progression. Establishing responder definition (RD) thresholds for these measures would enable evaluation of clinically meaningful treatment benefit.
Characteristic differences between vestibular migraine and migraine only patients
Pinar Özçelik, Koray Koçoglu, Vesile Öztürk, Pembe Keskinoglu & Gülden Akdal
doi : 10.1007/s00415-021-10636-0
Journal of Neurology volume 269, pages 336–341 (2022)
Vestibular migraine (VM) is one of the most common causes of vertigo in clinical practice but it is not always easy to make the correct diagnosis. Our aims were to find out how VM patients differ from migraine only (MO) patients, to evaluate co-morbid depression in these two groups and to determine if their disease has an effect on their quality of life.
The Clock Drawing Test as a predictor of cognitive decline in non-demented stroke patients
Ilaria Cova, Francesco Mele, Federica Zerini, Laura Maggiore, Silvia Rosa, Valentina Cucumo, Michela Brambilla, Alessia Nicotra, Giorgia Maestri, Pierluigi Bertora, Simone Pomati & Leonardo Pantoni
doi : 10.1007/s00415-021-10637-z
Journal of Neurology volume 269, pages 342–349 (2022)
The early detection of patients at risk of post-stroke cognitive impairment (PSCI) may help planning subacute and long-term care. We aimed to determine the predictivity of two screening cognitive tests on the occurrence of mild cognitive impairment or dementia in acute stroke patients.
MRI radiomic features-based machine learning approach to classify ischemic stroke onset time
Yi-Qun Zhang, Ao-Fei Liu, Feng-Yuan Man, Ying-Ying Zhang, Chen Li, Yun-E. Liu, Ji Zhou, Ai-Ping Zhang, Yang-Dong Zhang, Jin Lv & Wei-Jian Jiang
doi : 10.1007/s00415-021-10638-y
Journal of Neurology volume 269, pages 350–360 (2022)
We aimed to investigate the ability of MRI radiomics features-based machine learning (ML) models to classify the time since stroke onset (TSS), which could aid in stroke assessment and treatment options.
Chronobiology of transient global amnesia
Carolin Hoyer, Kyoko Higashida, Fabio Fabbian, Alfredo De Giorgi, Vesile Sandikci, Anne Ebert, Michael Platten, Shuhei Okazaki, Roberto Manfredini & Kristina Szabo
doi : 10.1007/s00415-021-10639-x
Journal of Neurology volume 269, pages 361–367 (2022)
The etiology of transient global amnesia (TGA) is still a matter of debate. Based, among others, on the observation of a close temporal relation between certain events and subsequent TGA episodes, recent proposals discuss the relevance of stress-associated processes impacting on hippocampal functioning. Circadian, infra- and ultradian rhythmicity has been found to play a relevant role in the multifactorial pathomechanisms of various disorders but has not been thoroughly studied in TGA.
Frequency and phenotype of thalamic aphasia
Ida Rangus, Merve Fritsch, Matthias Endres, Birgit Udke & Christian H. Nolte
doi : 10.1007/s00415-021-10640-4
Journal of Neurology volume 269, pages 368–376 (2022)
Aphasia is a recognized presenting symptom of thalamic lesions. Little is known regarding its frequency and phenotype. We examined the frequency of thalamic aphasia following Isolated Acute unilateral ischemic Lesions in the Thalamus (IALT) with respect to lesion location. Furthermore, we characterized thalamic aphasia according to affected language domains and severity.
Core cerebrospinal fluid biomarker profile in anti-LGI1 encephalitis
Pierre Lardeux, Anthony Fourier, Elise Peter, Aline Dorey, Sergio Muñiz-Castrillo, Alberto Vogrig, Géraldine Picard, Véronique Rogemond, Mathieu Verdurand, Maité Formaglio, Bastien Joubert, Caroline Froment Tilikete, Jérôme Honnorat, Isabelle Quadrio & Virginie Desestret
doi : 10.1007/s00415-021-10642-2
Journal of Neurology volume 269, pages 377–388 (2022)
To compare CSF biomarkers’ levels in patients suffering from anti-Leucine-rich Glioma-Inactivated 1 (LGI1) encephalitis to neurodegenerative [Alzheimer’s disease (AD), Creutzfeldt–Jakob’s disease (CJD)] and primary psychiatric (PSY) disorders.
Progressive cortical and sub-cortical alterations in patients with anti-N-methyl-d-aspartate receptor encephalitis
Jinping Xu, Yuanyuan Guo, Jiaying Li, Xinyi Lv, Juanjuan Zhang, Jinhuan Zhang, Qingmao Hu, Kai Wang & Yanghua Tian
doi : 10.1007/s00415-021-10643-1
Journal of Neurology volume 269, pages 389–398 (2022)
Advanced structural analyses are increasingly being highly valued to uncover pathophysiological understanding of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis. Therefore, we aimed to explore whether and how antibody-mediated NMDAR dysfunction affected cortical and sub-cortical brain morphology and their relationship with clinical symptoms.
Morning light therapy in adults with Tourette’s disorder
Emily J. Ricketts, Helen J. Burgess, Gabrielle E. Montalbano, Meredith E. Coles, Joseph F. McGuire, Hardian Thamrin, Dana L. McMakin, James T. McCracken, Mary A. Carskadon, John Piacentini & Christopher S. Colwell
doi : 10.1007/s00415-021-10645-z
Journal of Neurology volume 269, pages 399–410 (2022)
Sleep disturbance is common among individuals with Tourette’s Disorder (TD). Given that sleep is influenced by the circadian system, this study examined circadian rhythms and sleep in adults with TD, and explored the possible benefit of short-wavelength wearable morning light therapy.
A systematic review of non-motor symptom evaluation in clinical trials for amyotrophic lateral sclerosis
Emily Beswick, Deborah Forbes, Zack Hassan, Charis Wong, Judith Newton, Alan Carson, Sharon Abrahams, Siddharthan Chandran & Suvankar Pal
doi : 10.1007/s00415-021-10651-1
Journal of Neurology volume 269, pages 411–426 (2022)
Amyotrophic lateral sclerosis (ALS) is increasingly recognised as a multi-system disorder, presenting with common and impactful non-motor symptoms, such as neuropsychiatric symtpoms, cognitive and behavioural changes, pain, disordered sleep, fatigue and problematic saliva.
Evaluation of a new body-focused group therapy versus a guided self-help group program for adults with psychogenic non-epileptic seizures (PNES): a pilot randomized controlled feasibility study
Philine Senf-Beckenbach, Matthias Hoheisel, Janine Devine, Arnina Frank, Laura Obermann, Matthias Rose & Kim Hinkelmann
doi : 10.1007/s00415-021-10652-0
Journal of Neurology volume 269, pages 427–436 (2022)
Psychogenic non-epileptic seizures (PNES), a common phenomenon in neurological settings, are regarded as a paroxysmal type of functional neurological disorder (FND). In a substantial proportion, PNES are disabling with poor long-term outcomes and high economic costs. Despite the clinical and financial consequences of PNES, there is still a lack of controlled clinical trials on the treatment of this challenging disorder. The study aims to evaluate the feasibility and collect first evidence of the efficacy of a group based-intervention in PNES-patients.
Monoallelic KIF1A-related disorders: a multicenter cross sectional study and systematic literature review
Stefania Della Vecchia, Alessandra Tessa, Claudia Dosi, Jacopo Baldacci, Rosa Pasquariello, Antonella Antenora, Guja Astrea, Maria Teresa Bassi, Roberta Battini, Carlo Casali, Ettore Cioffi, Greta Conti, Giovanna De Michele, Anna Rita Ferrari, Alessandro Filla, Chiara Fiorillo, Carlo Fusco, Salvatore Gallone, Chiara Germiniasi, Renzo Guerrini, Shalom Haggiag, Diego Lopergolo, Andrea Martinuzzi, Federico Melani, Andrea Mignarri, Elena Panzeri, Antonella Pini, Anna Maria Pinto, Francesca Pochiero, Guido Primiano, Elena Procopio, Alessandra Renieri, Romina Romaniello, Cristina Sancricca, Serenella Servidei, Carlotta Spagnoli, Chiara Ticci, Anna Rubegni & Filippo Maria Santorelli
doi : 10.1007/s00415-021-10792-3
Journal of Neurology volume 269, pages 437–450 (2022)
Monoallelic variants in the KIF1A gene are associated with a large set of clinical phenotypes including neurodevelopmental and neurodegenerative disorders, underpinned by a broad spectrum of central and peripheral nervous system involvement.
Correction to: Monoallelic KIF1A-related disorders: a multicenter cross sectional study and systematic literature review
Stefania Della Vecchia, Alessandra Tessa, Claudia Dosi, Jacopo Baldacci, Rosa Pasquariello, Antonella Antenora, Guja Astrea, Maria Teresa Bassi, Roberta Battini, Carlo Casali, Ettore Cioffi, Greta Conti, Giovanna De Michele, Anna Rita Ferrari, Alessandro Filla, Chiara Fiorillo, Carlo Fusco, Salvatore Gallone, Chiara Germiniasi, Renzo Guerrini, Shalom Haggiag, Diego Lopergolo, Andrea Martinuzzi, Federico Melani, Andrea Mignarri, Elena Panzeri, Antonella Pini, Anna Maria Pinto, Francesca Pochiero, Guido Primiano, Elena Procopio, Alessandra Renieri, Romina Romaniello, Cristina Sancricca, Serenella Servidei, Carlotta Spagnoli, Chiara Ticci, Anna Rubegni & Filippo Maria Santorelli
doi : 10.1007/s00415-021-10839-5
Journal of Neurology volume 269, page 451 (2022)
Treatment response scoring systems to assess long-term prognosis in self-injectable DMTs relapsing–remitting multiple sclerosis patients
Jordi RÃo, Àlex Rovira, Claudio Gasperini, Mar Tintoré, Luca Prosperini, Susana Otero-Romero, Manuel Comabella, Ã�ngela Vidal-Jordana, Ingrid Galán, Luciana Midaglia, Breogán Rodriguez-Acevedo, Ana Zabalza, Joaquim Castilló, Georgina Arrambide, Carlos Nos, Ã�lvaro Cobo, Carmen Tur, Cristina Auger, Jaume Sastre-Garriga & Xavier Montalban
doi : 10.1007/s00415-021-10823-z
Journal of Neurology volume 269, pages 452–459 (2022)
Different treatment response scoring systems in treated MS patients exist. The objective was to assess the long-term predictive value of these systems in RRMS patients treated with self-injectable DMTs.
Correction to: Treatment response scoring systems to assess long-term prognosis in self-injectable DMTs relapsing–remitting multiple sclerosis patients
Jordi RÃo, Àlex Rovira, Claudio Gasperini, Mar Tintoré, Luca Prosperini, Susana Otero-Romero, Manuel Comabella, Ã�ngela Vidal-Jordana, Ingrid Galán, Luciana Midaglia, Breogán Rodriguez-Acevedo, Ana Zabalza, Joaquim Castilló, Georgina Arrambide, Carlos Nos, Ã�lvaro Cobo-Calvo, Carmen Tur, Cristina Auger, Jaume Sastre-Garriga & Xavier Montalban
doi : 10.1007/s00415-021-10906-x
Journal of Neurology volume 269, page 460 (2022)
FLAIR2 post-processing: improving MS lesion detection in standard MS imaging protocols
Tobias Zrzavy, Alice Wielandner, Lukas Haider, Sophie Bartsch, Fritz Leutmezer, Thomas Berger, Karl Heinz Nenning, Alexander Rauscher, Paulus Rommer & Gregor Kasprian
doi : 10.1007/s00415-021-10833-x
Journal of Neurology volume 269, pages 461–467 (2022)
Technical improvements in magnetic resonance imaging (MRI) acquisition, such as higher field strength and optimized sequences, lead to better multiple sclerosis (MS) lesion detection and characterization. Multiplication of 3D-FLAIR with 3D-T2 sequences (FLAIR2) results in isovoxel images with increased contrast-to-noise ratio, increased white–gray-matter contrast, and improved MS lesion visualization without increasing MRI acquisition time. The current study aims to assess the potential of 3D-FLAIR2 in detecting cortical/leucocortical (LC), juxtacortical (JC), and white matter (WM) lesions.
Correction to: FLAIR2 post-processing: improving MS lesion detection in standard MS imaging protocols
Tobias Zrzavy, Alice Wielandner, Lukas Haider, Sophie Bartsch, Fritz Leutmezer, Thomas Berger, Karl Heinz Nenning, Alexander Rauscher, Paulus Rommer & Gregor Kasprian
doi : 10.1007/s00415-021-10855-5
Journal of Neurology volume 269, pages 468–469 (2022)
Off-label-dosing of non-vitamin K-dependent oral antagonists in AF patients before and after stroke: results of the prospective multicenter Berlin Atrial Fibrillation Registry
Serdar Tütüncü, Manuel Olma, Claudia Kunze, Joanna Dietzel, Johannes Schurig, Cornelia Fiessler, Carolin Malsch, Tobias Eberhard Haas, Boris Dimitrijeski, Wolfram Doehner, Georg Hagemann, Frank Hamilton, Martin Honermann, Gerhard Jan Jungehulsing, Andreas Kauert, Hans-Christian Koennecke, Bruno-Marcel Mackert, Darius Nabavi, Christian H. Nolte, Joschua Mirko Reis, Ingo Schmehl, Paul Sparenberg, Robert Stingele, Enrico Völzke, Carolin Waldschmidt, Daniel Zeise-Wehry, Peter U. Heuschmann, Matthias Endress & Karl Georg Haeusler
doi : 10.1007/s00415-021-10866-2
Journal of Neurology volume 269, pages 470–480 (2022)
We aimed to analyze prevalence and predictors of NOAC off-label under-dosing in AF patients before and after the index stroke.
Correction to: Off-label-dosing of non-vitamin K-dependent oral antagonists in AF patients before and after stroke: results of the prospective multicenter Berlin Atrial Fibrillation Registry
Serdar Tütüncü, Manuel Olma, Claudia Kunze, Joanna Dietzel, Johannes Schurig, Cornelia Fiessler, Carolin Malsch, Tobias Eberhard Haas, Boris Dimitrijeski, Wolfram Doehner, Georg Hagemann, Frank Hamilton, Martin Honermann, Gerhard Jan Jungehulsing, Andreas Kauert, Hans-Christian Koennecke, Bruno-Marcel Mackert, Darius Nabavi, Christian H. Nolte, Joschua Mirko Reis, Ingo Schmehl, Paul Sparenberg, Robert Stingele, Enrico Völzke, Carolin Waldschmidt, Daniel Zeise-Wehry, Peter U. Heuschmann, Matthias Endres & Karl Georg Haeusler
doi : 10.1007/s00415-021-10907-w
Journal of Neurology volume 269, pages 481–482 (2022)
Radiation-induced intracranial vasculitis on high-resolution vessel wall MRI
Wenjie Yang, John W. Krakauer & Bruce A. Wasserman
doi : 10.1007/s00415-021-10742-z
Journal of Neurology volume 269, pages 483–485 (2022)
Asymmetric rapidly progressive idiopathic normal-pressure hydrocephalus: description of a case
Paride Schito, Francesca Caso, Giuseppe Magnani, Lina Raffaella Barzaghi, Alessandra Barbieri, Maria Antonietta Volontè, Vittorio Martinelli, Luigia Brugliera, Sandro Iannaccone & Massimo Filippi
doi : 10.1007/s00415-021-10746-9
Journal of Neurology volume 269, pages 486–489 (2022)
The visually enhanced vestibulo-ocular reflex in CANVAS
Gábor M. Halmágyi, Kishore Kumar & Leigh A. McGarvie
doi : 10.1007/s00415-021-10755-8
Journal of Neurology volume 269, pages 490–492 (2022)
Interpretation of vaccine associated neurological adverse events: a methodological and historical review
Marija Cauchi, Harriet Ball, Yoav Ben-Shlomo & Neil Robertson
doi : 10.1007/s00415-021-10747-8
Journal of Neurology volume 269, pages 493–503 (2022)
As a result of significant recent scientific investment, the range of vaccines available for COVID-19 prevention continues to expand and uptake is increasing globally. Although initial trial safety data have been generally reassuring, a number of adverse events, including vaccine induced thrombosis and thrombocytopenia (VITT), have come to light which have the potential to undermine the success of the vaccination program. However, it can be difficult to interpret available data and put these into context and to communicate this effectively. In this review, we discuss contemporary methodologies employed to investigate possible associations between vaccination and adverse neurological outcomes and why determining causality can be challenging. We demonstrate these issues by discussing relevant historical exemplars and explore the relevance for the current pandemic and vaccination program. We also discuss challenges in understanding and communicating such risks to clinicians and the general population within the context of the ‘infodemic’ facilitated by the Internet and other media.
Autosomal recessive adult onset ataxia
Nataša Dragaševic-Miškovic, Iva Stankovic, Andona Milovanovic & Vladimir S. Kostic
doi : 10.1007/s00415-021-10763-8
Journal of Neurology volume 269, pages 504–533 (2022)
Autosomal recessive ataxias (ARCA) represent a complex group of diseases ranging from primary ataxias to rare and complex metabolic disorders in which ataxia is a part of the clinical picture. Small number of ARCA manifest exclusively in adulthood, while majority of typical childhood onset ARCA may also start later with atypical clinical presentation. We have systematically searched the literature for ARCA with adult onset, both in the group of primary ataxias including those that are less frequently described in isolated or in a small number of families, and also in the group of complex and metabolic diseases in which ataxia is only part of the clinical picture. We propose an algorithm that could be used when encountering a patient with adult onset sporadic or recessive ataxia in whom the acquired causes are excluded. ARCA are frequently neglected in the differential diagnosis of adult-onset ataxias. Rising awareness of their clinical significance is important, not only because some of these disorders may be potentially treatable, but also for prognostic implications and inclusion of patients to future clinical trials with disease modifying agents.
Intravenous immunoglobulin for inflammatory demyelinating polyneuropathy
M. Talaei & N. P. Robertson
doi : 10.1007/s00415-021-10908-9
Journal of Neurology volume 269, pages 534–536 (2022)
Gabriel Anton (1858–1933)
Andrzej Grzybowski & Joanna Zolnierz
doi : 10.1007/s00415-021-10662-y
Journal of Neurology volume 269, pages 537–538 (2022)
Correction to: Role of complement and potential of complement inhibitors in myasthenia gravis and neuromyelitis optica spectrum disorders: a brief review
Jayne L. Chamberlain, Saif Huda, Daniel H. Whittam, Marcelo Matiello, B. Paul Morgan & Anu Jacob
doi : 10.1007/s00415-021-10896-w
Journal of Neurology volume 269, page 539 (2022)
Correction to: Long-term effects of bilateral pallidal deep brain stimulation in dystonia: a follow-up between 8 and 16Â years
P. Krause, S. Völzmann, S. Ewert, A. Kupsch, G. H. Schneider & Andrea A. Kühn
doi : 10.1007/s00415-021-10863-5
Journal of Neurology volume 269, page 540 (2022)
SARS-CoV-2 infection and acute ischemic stroke in Lombardy, Italy
Alessandro Pezzini ,Mario Grassi
doi : 10.1007/s00415-021-10620-8
Journal of Neurology volume 269, pages 1–11 (2022)
To characterize patients with acute ischemic stroke related to SARS-CoV-2 infection and assess the classification performance of clinical and laboratory parameters in predicting in-hospital outcome of these patients.
Altered mental status in COVID-19
Daniel Antoniello, Mark J. Milstein, Joseph Dardick, Jenelys Fernandez-Torres, Jenny Lu, Nikunj Patel & Charles Esenwa
doi : 10.1007/s00415-021-10623-5
Journal of Neurology volume 269, pages 12–18 (2022)
Altered mental status (AMS) is a common neurological manifestation of COVID-19 infection in hospitalized patients. The principal causes of AMS have yet to be determined. We aimed to identify the common causes of AMS in patients with COVID-19 presenting to the emergency department with AMS on arrival.
Impact of the COVID-19 lockdown on the adherence of stroke patients to direct oral anticoagulants: a secondary analysis from the MAAESTRO study
Fine Dietrich, Alexandros A. Polymeris, Melina Verbeek, Stefan T. Engelter, Kurt E. Hersberger, Sabine Schaedelin, Isabelle Arnet & Philippe A. Lyrer
doi : 10.1007/s00415-021-10631-5
Journal of Neurology volume 269, pages 19–25 (2022)
The negative impact of the COVID-19 outbreak on stroke care has been reported, but no data exist on the influence of the lockdown on medication adherence to antithrombotic treatment for stroke prevention. We present a comparison of electronic adherence data of stroke patients treated with direct oral anticoagulants (DOAC) prior to and during the COVID-19 lockdown in spring 2020 in Switzerland.
Global survey on disruption and mitigation of neurological services during COVID-19: the perspective of global international neurological patients and scientific associations
Chahnez Charfi Triki, Matilde Leonardi, Salma Zouari Mallouli, Martina Cacciatore, Kimberly Coard Karlshoej, Francesca Giulia Magnani, Charles R. Newton, Andrea Pilotto, Deanna Saylor, Erica Westenberg, Donna Walsh, Andrea Sylvia Winkler, Kiran T. Thakur, Njideka U. Okubadejo & David Garcia-Azorin
doi : 10.1007/s00415-021-10641-3
Journal of Neurology volume 269, pages 26–38 (2022)
The COVID-19 pandemic outbreak has dramatically disrupted healthcare systems. Two rapid WHO pulse surveys studied disruptions in mental health services, but did not particularly focus on neurology. Here, a global survey was conducted and addresses the impact of the pandemic on neurology services.
Serological response to SARS-CoV-2 vaccination in multiple sclerosis patients treated with fingolimod or ocrelizumab: an initial real-life experience
Guerrieri S., Lazzarin S., Zanetta C., Nozzolillo A., Filippi M. & Moiola L.
doi : 10.1007/s00415-021-10663-x
Journal of Neurology volume 269, pages 39–43 (2022)
Recent observations suggest a lack of humoral response after SARS-CoV-2 vaccination in multiple sclerosis (MS) patients treated with fingolimod or ocrelizumab
Long COVID: cognitive complaints (brain fog) and dysfunction of the cingulate cortex
Jacques Hugon, Eva-Flore Msika, Mathieu Queneau, Karim Farid & Claire Paquet
doi : 10.1007/s00415-021-10655-x
Journal of Neurology volume 269, pages 44–46 (2022)
Many patients who have suffered from acute COVID infections have long-lasting symptoms affecting several organs including the brain. This long COVID status can include “brain fog” and cognitive deficits that can disturb activities of daily living and can delay complete recovery. Here, we report two cases of neurological long COVID with abnormal FDG PET findings marked by hypometabolic regions of the cingulate cortex.
Bell’s palsy following a single dose of mRNA SARS-CoV-2 vaccine: a case report
C. Martin-Villares, A. Vazquez-Feito, M. J. Gonzalez-Gimeno & B. de la Nogal-Fernandez
doi : 10.1007/s00415-021-10617-3
Journal of Neurology volume 269, pages 47–48 (2022)
Acute bilateral optic/chiasm neuritis with longitudinal extensive transverse myelitis in longstanding stable multiple sclerosis following vector-based vaccination against the SARS-CoV-2
Christoph Helmchen, Gesine M. Buttler, Robert Markewitz, Katja Hummel, Heinz Wiendl & Tobias Boppel
doi : 10.1007/s00415-021-10647-x
Journal of Neurology volume 269, pages 49–54 (2022)
First manifestation of multiple sclerosis after immunization with the Pfizer-BioNTech COVID-19 vaccine
Joachim Havla, Yannick Schultz, Hanna Zimmermann, Reinhard Hohlfeld, Adrian Danek & Tania Kümpfel
doi : 10.1007/s00415-021-10648-w
Journal of Neurology volume 269, pages 55–58 (2022)
COVID-19 and Parkinson’s disease: a casual association or a possible second hit in neurodegeneration?
Francesco Cavallieri, Valentina Fioravanti, Giulia Toschi, Sara Grisanti, Manuela Napoli, Claudio Moratti, Rosario Pascarella, Annibale Versari, Alessandro Fraternali, Massimiliano Casali, Jefri J. Paul, Elena Moro, Peter Bauer, Marialuisa Zedde & Franco Valzania
doi : 10.1007/s00415-021-10694-4
Journal of Neurology volume 269, pages 59–61 (2022)
Placebo response in degenerative cerebellar ataxias: a descriptive review of randomized, placebo-controlled trials
Ji-Hyun Choi, Chaewon Shin, Han-Joon Kim & Beomseok Jeon
doi : 10.1007/s00415-020-10306-7
Journal of Neurology volume 269, pages 62–71 (2022)
Placebo response in degenerative cerebellar ataxias (CAs) has never been studied despite the large number of randomized controlled trials (RCTs) that have been conducted. In this descriptive review, we aimed to examine the placebo response in patients with CAs. We performed a literature search on PubMed for RCTs on CAs that were published from 1977 to January 2020 and collected data on the changes from the baseline to the endpoint on various objective ataxia-associated clinical rating scales. We reviewed 56 clinical trials, finally including 35 parallel-group studies and excluding 21 cross-over studies. The included studies were categorized as follows: (1) studies showing significant improvements in one or more ataxia scales in the placebo groups (n?=?3); (2) studies reporting individual placebo responders with improvements in one or more ataxia scales in the placebo groups (n?=?5)—the overall proportion of placebo responders was 31.9%; (3) studies showing mean changes in the direction of improvement in at least one ataxia scale in the placebo groups, though not statistically significant (n?=?19); (4) studies showing no placebo response in any of the ataxia scales in the placebo groups (n?=?4); (5) studies where data on the placebo groups were unavailable (n?=?9). This review demonstrated the placebo response in patients with CAs on various objective ataxia scales. Our study emphasizes that the placebo response should be considered when designing, analyzing, and interpreting clinical trials and in clinical practice in CA patients.
Effects of intratympanic gentamicin and intratympanic glucocorticoids in Ménière’s disease: a network meta-analysis
Weiming Hao, Huiqian Yu & Huawei Li
doi : 10.1007/s00415-020-10320-9
Journal of Neurology volume 269, pages 72–86 (2022)
Intratympanic therapies, usually including glucocorticoid and gentamicin, are becoming worldwide used in clinical practice of Ménière’s disease today. However, clinical efficacy and safety of these two therapies are still in controversial.
Hearing loss versus vestibular loss as contributors to cognitive dysfunction
Paul F. Smith
doi : 10.1007/s00415-020-10343-2
Journal of Neurology volume 269, pages 87–99 (2022)
In the last 5 years, there has been a surge in evidence that hearing loss (HL) may be a risk factor for cognitive dysfunction, including dementia. At the same time, there has been an increase in the number of studies implicating vestibular loss in cognitive dysfunction. Due to the fact that vestibular disorders often present with HL and other auditory disorders such as tinnitus, it has been suggested that, in many cases, what appears to be vestibular-related cognitive dysfunction may be due to HL (e.g., Dobbels et al. Front Neurol 11:710, 2020). This review analyses the studies of vestibular-related cognitive dysfunction which have controlled HL. It is suggested that despite the fact that many studies in the area have not controlled HL, many other studies have (~ 19/44 studies or 43%). Therefore, although there is certainly a need for further studies controlling HL, there is evidence to suggest that vestibular loss is associated with cognitive dysfunction, especially related to spatial memory. This is consistent with the overwhelming evidence from animal studies that the vestibular system transmits specific types of information about self-motion to structures such as the hippocampus.
Wearables in the home-based assessment of abnormal movements in Parkinson’s disease: a systematic review of the literature
Stefania Ancona, Francesca D. Faraci, Elina Khatab, Luigi Fiorillo, Oriella Gnarra, Tobias Nef, Claudio L. A. Bassetti & Panagiotis Bargiotas
doi : 10.1007/s00415-020-10350-3
Journal of Neurology volume 269, pages 100–110 (2022)
At present, the standard practices for home-based assessments of abnormal movements in Parkinson’s disease (PD) are based either on subjective tools or on objective measures that often fail to capture day-to-day fluctuations and long-term information in real-life conditions in a way that patient’s compliance and privacy are secured. The employment of wearable technologies in PD represents a great paradigm shift in healthcare remote diagnostics and therapeutics monitoring. However, their applicability in everyday clinical practice seems to be still limited. We carried out a systematic search across the Medline Database. In total, 246 publications, published until 1 June 2020, were identified. Among them, 26 reports met the inclusion criteria and were included in the present review. We focused more on clinically relevant aspects of wearables’ application including feasibility and efficacy of the assessment, the number, type and body position of the wearable devices, type of PD motor symptom, environment and duration of assessments and validation methodology. The aim of this review is to provide a systematic overview of the current knowledge and state-of-the-art of the home-based assessment of motor symptoms and fluctuations in PD patients using wearable technology, highlighting current problems and laying foundations for future works.
The changing landscape of optic neuritis: a narrative review
Lindsey B. De Lott, Jeffrey L. Bennett & Fiona Costello
doi : 10.1007/s00415-020-10352-1
Journal of Neurology volume 269, pages 111–124 (2022)
Optic neuritis (ON) is an inflammatory optic neuropathy that is often a harbinger of central nervous system (CNS) demyelinating disorders. ON is frequently misdiagnosed in the clinical arena, leading to either inappropriate management or diagnostic delays. As a result, patients may fail to achieve optimal recovery. The treatment response to corticosteroids and long term risk of multiple sclerosis was established in the first clinical trials conducted roughly 30 years ago. Spontaneous resolution was observed in the vast majority of patients and intravenous high-dose corticosteroids hastened recovery; half of the patients eventually developed multiple sclerosis. Over the ensuing decades, the number of inflammatory conditions associated with ON has significantly expanded exposing substantial variability in the prognosis, treatment, and management of ON patients. ON subtypes can frequently be distinguished by distinct clinical, serological, and radiological profiles allowing expedited and specialized treatment. Guided by an increased understanding of the immunopathology underlying optic nerve and associated CNS injuries, novel disease management strategies are emerging to minimize vision loss, improve long-term surveillance strategies, and minimize CNS injury and disability. Knowledge regarding the clinical signs and symptoms of different ON subtypes is essential to guide acute therapy, prognosticate recovery, accurately identify underlying CNS inflammatory disorders, and facilitate study design for the next generation of clinical and translational trials.
A critical review of the pharmacological treatment of REM sleep behavior disorder in adults: time for more and larger randomized placebo-controlled trials
Moran Gilat, Nathaniel S. Marshall, Dries Testelmans, Bertien Buyse & Simon J. G. Lewis
doi : 10.1007/s00415-020-10353-0
Journal of Neurology volume 269, pages 125–148 (2022)
Rapid Eye Movement sleep behavior disorder (RBD) is a parasomnia causing sufferers to physically act out their dreams. These behaviors can disrupt sleep and sometimes lead to injuries in patients and their bed-partners. Clonazepam and melatonin are the first-line pharmacological treatment options for RBD based on direct uncontrolled clinical observations and very limited double-blind placebo-controlled trials. Given the risk for adverse outcomes, especially in older adults, it is of great importance to assess the existing level of evidence for the use of these treatments. In this update, we therefore critically review the clinical and scientific evidence on the pharmacological management of RBD in people aged over 50. We focus on the first-line treatments, and provide an overview of all other alternative pharmacological agents trialed for RBD we could locate as supplementary materials. By amalgamating all clinical observations, our update shows that 66.7% of 1,026 RBD patients reported improvements from clonazepam and 32.9% of 137 RBD patients reported improvements from melatonin treatment on various outcome measures in published accounts. Recently, however, three relatively small randomized placebo-controlled trials did not find these agents to be superior to placebo. Given clonazepam and melatonin are clinically assumed to majorly modify or eliminate RBD in nearly all patients—there is an urgent need to test whether this magnitude of treatment effect remains intact in larger placebo-controlled trials.
Strokectomy for malignant middle cerebral artery infarction: experience and meta-analysis of current evidence
Saad Moughal, Sarah Trippier, Alaa AL-Mousa, Atticus H. Hainsworth, Anthony C. Pereira, Pawanjit S Minhas & Anan Shtaya
doi : 10.1007/s00415-020-10358-9
Journal of Neurology volume 269, pages 149–158 (2022)
Strokectomy means surgical excision of infarcted brain tissue post-stroke with preservation of skull integrity, distinguishing it from decompressive hemicraniectomy. Both can mitigate malignant middle cerebral artery (MCA) syndrome but evidence regarding strokectomy is sparse. Here, we report our data and meta-analysis of strokectomy compared to hemicraniectomy for malignant MCA infarction. All malignant MCA stroke cases requiring surgical intervention in a large tertiary centre (January 2012–December 2017, N?=?24) were analysed for craniotomy diameter, complications, length of follow-up and outcome measured using the modified Rankin score (mRS). Good outcome was defined as mRS 0–3 at 12 months. In a meta-analysis, outcome from strokectomy (pooled from our cohort and published strokectomy studies) was compared with hemicraniectomy (our cohort pooled with published DECIMAL, DESTINY and HAMLET clinical trial data). In our series (N?=?24, 12/12 F/M; mean age: 45.83?±?8.91, range 29–63 years), 4 patients underwent strokectomy (SC) and 20 hemicraniectomy (HC). Among SC patients, craniotomy diameter was smaller, relative to HC patients (86?±?13.10 mm, 120?±?4.10 mm, respectively; p?=?0.003), complications were less common (25%, 55%) and poor outcomes were less common (25%, 70%). In the pooled data (N?=?41 SC, 71 HC), strokectomy tended towards good outcome more than hemicraniectomy (OR 2.2, 95% CI 0.99–4.7; p?=?0.051). In conclusion, strokectomy may be non-inferior, lower risk and cost saving relative to hemicraniectomy sufficiently to be worthy of further investigation and maybe a randomised trial.
Rituximab for the treatment of multiple sclerosis: a review
Clara Grazia Chisari, Eleonora Sgarlata, Sebastiano Arena, Simona Toscano, Maria Luca & Francesco Patti
doi : 10.1007/s00415-020-10362-z
Journal of Neurology volume 269, pages 159–183 (2022)
In the last decades, evidence suggesting the direct or indirect involvement of B cells on multiple sclerosis (MS) pathogenesis has accumulated. The increased amount of data on the efficacy and safety of B-cell-depleting therapies from several studies has suggested the addition of these drugs as treatment options to the current armamentarium of disease modifying therapies (DMTs) for MS. Particularly, rituximab (RTX), a chimeric monoclonal antibody directed at CD20 positive B lymphocytes resulting in cell-mediated apoptosis, has been demonstrated to reduce inflammatory activity, incidence of relapses and new brain lesions on magnetic resonance imaging (MRI) in patients with relapsing–remitting MS (RRMS). Additional evidence also demonstrated that patients with progressive MS (PMS) may benefit from RTX, which also showed to be well tolerated, with acceptable safety risks and favorable cost-effectiveness profile.
Migraine in transient global amnesia: a meta-analysis of observational studies
Ioannis Liampas, Athanasios S. Siouras, Vasileios Siokas, Zisis Tsouris, Dimitrios Rikos, Alexandros Brotis, Athina-Maria Aloizou, Metaxia Dastamani & Efthimios Dardiotis
doi : 10.1007/s00415-020-10363-y
Journal of Neurology volume 269, pages 184–196 (2022)
Although many studies have investigated the relationship between transient global amnesia (TGA) and migraine, to date, no meta-analysis has confirmed the existence and size of their association.
Parameters for subthalamic deep brain stimulation in patients with dystonia: a systematic review
Yuhan Wang, Chencheng Zhang, Bomin Sun, Dianyou Li & Yiwen Wu
doi : 10.1007/s00415-020-10372-x
Journal of Neurology volume 269, pages 197–204 (2022)
Deep brain stimulation (DBS) is used for treating dystonia, commonly targeting the subthalamic nucleus (STN). Optimal stimulation parameters are required to achieve satisfying results. However, recommended parameters for STN-DBS remain to be identified. In this review, we aimed to assess the optimal stimulation parameters by analyzing previously published STN-DBS data of patients with dystonia.
Effect of melatonin supplementation on sleep quality: a systematic review and meta-analysis of randomized controlled trials
Gholami Fatemeh, Moradi Sajjad, Rasaei Niloufar, Soveid Neda, Setayesh Leila & Mirzaei Khadijeh
doi : 10.1007/s00415-020-10381-w
Journal of Neurology volume 269, pages 205–216 (2022)
The Present study was conducted to systematically review the effect of the melatonin on sleep quality. We summarized evidence from randomized clinical trials (RCTs) that investigated the effects of melatonin on sleep quality as assessed by the Pittsburgh Sleep Quality Index (PSQI) in adults with various diseases.
Bedside video-ophthalmoscopy as an aid in the diagnosis of central vestibular syndromes
Victor Rodrigues Pereira Evangelista, Sofia Abreu Mermelstein, Marcos Martins da Silva & Diego Kaski
doi : 10.1007/s00415-020-10389-2
Journal of Neurology volume 269, pages 217–220 (2022)
The evaluation of central vestibular syndromes, especially in the acute setting, can pose a challenge even for the most experienced clinician. Of particular importance is the evaluation of ocular torsion and nystagmus, which can be sensitive for central vestibular pathology, but easily missed by the untrained eye. We present two cases of acute vestibular syndrome of central origin in which the use of magnified fundoscopy at the bedside aided the precise anatomical diagnosis to inform appropriate further management. We also review aspects of the pathophysiology and anatomy of vestibular roll plane disorders. In case 1, the finding of position-dependent ocular torsion facilitated a rapid distinction between central skew deviation and a trochlear nerve palsy. In case 2, the fundoscopic magnification identified a pure rotatory nystagmus indicative of a central vestibular disorder. Ophthalmoscopy remains a useful bedside technique in acute vertigo, but the use of inexpensive magnification with a smartphone can provide objective and recordable evidence of central vestibular pathology, aiding therapeutic decisions.
Functional connectivity of hippocampus in temporal lobe epilepsy depends on hippocampal dominance: a systematic review of the literature
Camille K. Milton, Christen M. O’Neal & Andrew K. Conner
doi : 10.1007/s00415-020-10391-8
Journal of Neurology volume 269, pages 221–232 (2022)
Lateralized alterations in hippocampal function in the resting-state have been demonstrated for patients with temporal lobe epilepsy (TLE). However, resting-state fMRI of the hippocampus has yet to be substantiated as an adjunct to standard pre-operative assessments of the seizure focus.
Multiverse to inform neurological research: an example using recovery outcome of neglect
Margaret J. Moore & Nele Demeyere
doi : 10.1007/s00415-021-10612-8
Journal of Neurology volume 269, pages 233–242 (2022)
Multiverse analysis provides an ideal tool for understanding how inherent, yet ultimately arbitrary methodological choices impact the conclusions of individual studies. With this investigation, we aimed to demonstrate the utility of multiverse analysis for evaluating generalisability and identifying potential sources of bias within studies employing neurological populations.
Periorbital injections of botulinum toxin a: a novel therapeutic option for convergence spasm in neuropsychiatric disorders
Kristina Hess, Moritz Schmitt & Bettina Wabbels
doi : 10.1007/s00415-021-10613-7
Journal of Neurology volume 269, pages 243–250 (2022)
Convergence spasm (CS, spasm of near reflex) is characterized by transient attacks of convergence, miosis and accommodation, often associated with functional neurological disorders. To date, no simple and efficient treatment option is available for CS. This study investigates whether periorbital botulinum toxin injections as used in essential blepharospasm are also a treatment option in these patients.
Music to move persons with Parkinson’s disease: a personalized approach
Susanne Ten Holter, Jorik Nonnekes & Bastiaan Bloem
doi : 10.1007/s00415-021-10615-5
Journal of Neurology volume 269, pages 251–252 (2022)
Subthalamic deep brain stimulation affects heading perception in Parkinson’s disease
Sinem Balta Beylergil, Angela M. Noecker, Mikkel Petersen, Palak Gupta, Sarah Ozinga, Mark F. Walker, Camilla Kilbane, Cameron C. McIntyre & Aasef G. Shaikh
doi : 10.1007/s00415-021-10616-4
Journal of Neurology volume 269, pages 253–268 (2022)
Parkinson’s disease (PD) presents with visuospatial impairment and falls. It is critical to understand how subthalamic deep brain stimulation (STN DBS) modulates visuospatial perception. We hypothesized that DBS has different effects on visual and vestibular perception of linear motion (heading), a critical aspect of visuospatial navigation; and such effects are specific to modulated STN location. Two-alternative forced-choice experiments were performed in 14 PD patients with bilateral STN DBS and 19 age-matched healthy controls (HC) during passive en bloc linear motion and 3D optic-flow in immersive virtual reality measured vestibular and visual heading. Objective measure of perception with Weibull psychometric function revealed that PD has significantly lower accuracy [L: 60.71 (17.86)%, R: 74.82 (17.44)%] and higher thresholds [L: 16.68 (12.83), R: 10.09 (7.35)] during vestibular task in both directions compared to HC (p?<?0.05). DBS significantly improved vestibular discrimination accuracy [81.40 (14.36)%] and threshold [4.12 (5.87), p?<?0.05] in the rightward direction. There were no DBS effects on the slopes of vestibular psychometric curves. Visual heading perception was better than vestibular and it was comparable to HC. There was no significant effect of DBS on visual heading response accuracy or discrimination threshold (p?>?0.05). Patient-specific DBS models revealed an association between change in vestibular heading perception and the modulation of the dorsal STN. In summary, DBS may have different effects on vestibular and visual heading perception in PD. These effects may manifest via dorsal STN putatively by its effects on the cerebellum.
Elucidating distinct clinico-radiologic signatures in the borderland between neuromyelitis optica and multiple sclerosis
Maciej Jurynczyk, Elzbieta Klimiec-Moskal, Yazhuo Kong, Samuel Hurley, Silvia Messina, Tianrong Yeo, Mark Jenkinson, Maria Isabel Leite & Jacqueline Palace
doi : 10.1007/s00415-021-10619-1
Journal of Neurology volume 269, pages 269–279 (2022)
Separating antibody-negative neuromyelitis optica spectrum disorders (NMOSD) from multiple sclerosis (MS) in borderline cases is extremely challenging due to lack of biomarkers. Elucidating different pathologies within the likely heterogenous antibody-negative NMOSD/MS overlap syndrome is, therefore, a major unmet need which would help avoid disability from inappropriate treatment.
Anti-HMGCR myopathy overlaps with dermatomyositis-like rash: a distinct subtype of idiopathic inflammatory myopathy
Ying Hou, Kai Shao, Yaping Yan, Tingjun Dai, Wei Li, Yuying Zhao, Duoling Li, Jian-Qiang Lu, Gary L. Norman & Chuanzhu Yan
doi : 10.1007/s00415-021-10621-7
Journal of Neurology volume 269, pages 280–293 (2022)
To characterize the clinical and pathological features of anti-HMGCR myopathy.
Capturing vertigo in the emergency room: three tools to double the rate of diagnosis
Benjamin Nham, Nicole Reid, Kendall Bein, Andrew P. Bradshaw, Leigh A. McGarvie, Emma C. Argaet, Allison S. Young, Shaun R. Watson, G. Michael Halmagyi, Deborah A. Black & Miriam S. Welgampola
doi : 10.1007/s00415-021-10627-1
Journal of Neurology volume 269, pages 294–306 (2022)
Many patients attending the emergency room (ER) with vertigo, leave without a diagnosis. We assessed whether the three tools could improve ER diagnosis of vertigo.
Sex-specific differences in presentations and determinants of outcomes after endovascular thrombectomy for large vessel occlusion stroke
Robert W. Regenhardt, Ashby C. Turner, Joshua A. Hirsch, Michael J. Young, Naif M. Alotaibi, Christopher J. Stapleton, Aman B. Patel, Thabele M. Leslie-Mazwi, Natalia S. Rost & Mark R. Etherton
doi : 10.1007/s00415-021-10628-0
Journal of Neurology volume 269, pages 307–315 (2022)
Sex-specific differences in ischemic stroke outcomes are prevalent. We sought to investigate sex differences in the determinants of reperfusion and functional outcomes after endovascular thrombectomy (EVT) for emergent large vessel occlusion ischemic stroke (ELVO).
Alterations of the structural covariance network in the hypothalamus of patients with cluster headache
Dong Ah Lee, Ho-Joon Lee, Hyung Chan Kim & Kang Min Park
doi : 10.1007/s00415-021-10629-z
Journal of Neurology volume 269, pages 316–322 (2022)
The hypothalamus is one of the key structures involved in the pathophysiology of cluster headaches. This study aimed to analyze the volume of hypothalamic subunits and structural covariance networks in the hypothalamus of patients with cluster headache.
Responder analysis for neuropathic impairment and quality-of-life assessment in patients with hereditary transthyretin amyloidosis with polyneuropathy in the NEURO-TTR study
Aaron Yarlas, Andrew Lovley, Duncan Brown, Mark Kosinski & Montserrat Vera-Llonch
doi : 10.1007/s00415-021-10635-1
Journal of Neurology volume 269, pages 323–335 (2022)
Hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN) is a rare disease characterized by rapid neuropathic progression. In pivotal studies of gene-silencing treatments, the modified Neuropathy Impairment Score?+?7 tests (mNIS?+?7) and Norfolk-Quality of Life (QOL)-Diabetic Neuropathy (DN) questionnaire assessed treatment impact on neuropathic progression. Establishing responder definition (RD) thresholds for these measures would enable evaluation of clinically meaningful treatment benefit.
Characteristic differences between vestibular migraine and migraine only patients
Pinar Özçelik, Koray Koçoglu, Vesile Öztürk, Pembe Keskinoglu & Gülden Akdal
doi : 10.1007/s00415-021-10636-0
Journal of Neurology volume 269, pages 336–341 (2022)
Vestibular migraine (VM) is one of the most common causes of vertigo in clinical practice but it is not always easy to make the correct diagnosis. Our aims were to find out how VM patients differ from migraine only (MO) patients, to evaluate co-morbid depression in these two groups and to determine if their disease has an effect on their quality of life.
The Clock Drawing Test as a predictor of cognitive decline in non-demented stroke patients
Ilaria Cova, Francesco Mele, Federica Zerini, Laura Maggiore, Silvia Rosa, Valentina Cucumo, Michela Brambilla, Alessia Nicotra, Giorgia Maestri, Pierluigi Bertora, Simone Pomati & Leonardo Pantoni
doi : 10.1007/s00415-021-10637-z
Journal of Neurology volume 269, pages 342–349 (2022)
The early detection of patients at risk of post-stroke cognitive impairment (PSCI) may help planning subacute and long-term care. We aimed to determine the predictivity of two screening cognitive tests on the occurrence of mild cognitive impairment or dementia in acute stroke patients.
MRI radiomic features-based machine learning approach to classify ischemic stroke onset time
Yi-Qun Zhang, Ao-Fei Liu, Feng-Yuan Man, Ying-Ying Zhang, Chen Li, Yun-E. Liu, Ji Zhou, Ai-Ping Zhang, Yang-Dong Zhang, Jin Lv & Wei-Jian Jiang
doi : 10.1007/s00415-021-10638-y
Journal of Neurology volume 269, pages 350–360 (2022)
We aimed to investigate the ability of MRI radiomics features-based machine learning (ML) models to classify the time since stroke onset (TSS), which could aid in stroke assessment and treatment options.
Chronobiology of transient global amnesia
Carolin Hoyer, Kyoko Higashida, Fabio Fabbian, Alfredo De Giorgi, Vesile Sandikci, Anne Ebert, Michael Platten, Shuhei Okazaki, Roberto Manfredini & Kristina Szabo
doi : 10.1007/s00415-021-10639-x
Journal of Neurology volume 269, pages 361–367 (2022)
The etiology of transient global amnesia (TGA) is still a matter of debate. Based, among others, on the observation of a close temporal relation between certain events and subsequent TGA episodes, recent proposals discuss the relevance of stress-associated processes impacting on hippocampal functioning. Circadian, infra- and ultradian rhythmicity has been found to play a relevant role in the multifactorial pathomechanisms of various disorders but has not been thoroughly studied in TGA.
Frequency and phenotype of thalamic aphasia
Ida Rangus, Merve Fritsch, Matthias Endres, Birgit Udke & Christian H. Nolte
doi : 10.1007/s00415-021-10640-4
Journal of Neurology volume 269, pages 368–376 (2022)
Aphasia is a recognized presenting symptom of thalamic lesions. Little is known regarding its frequency and phenotype. We examined the frequency of thalamic aphasia following Isolated Acute unilateral ischemic Lesions in the Thalamus (IALT) with respect to lesion location. Furthermore, we characterized thalamic aphasia according to affected language domains and severity.
Core cerebrospinal fluid biomarker profile in anti-LGI1 encephalitis
Pierre Lardeux, Anthony Fourier, Elise Peter, Aline Dorey, Sergio Muñiz-Castrillo, Alberto Vogrig, Géraldine Picard, Véronique Rogemond, Mathieu Verdurand, Maité Formaglio, Bastien Joubert, Caroline Froment Tilikete, Jérôme Honnorat, Isabelle Quadrio & Virginie Desestret
doi : 10.1007/s00415-021-10642-2
Journal of Neurology volume 269, pages 377–388 (2022)
To compare CSF biomarkers’ levels in patients suffering from anti-Leucine-rich Glioma-Inactivated 1 (LGI1) encephalitis to neurodegenerative [Alzheimer’s disease (AD), Creutzfeldt–Jakob’s disease (CJD)] and primary psychiatric (PSY) disorders.
Progressive cortical and sub-cortical alterations in patients with anti-N-methyl-d-aspartate receptor encephalitis
Jinping Xu, Yuanyuan Guo, Jiaying Li, Xinyi Lv, Juanjuan Zhang, Jinhuan Zhang, Qingmao Hu, Kai Wang & Yanghua Tian
doi : 10.1007/s00415-021-10643-1
Journal of Neurology volume 269, pages 389–398 (2022)
Advanced structural analyses are increasingly being highly valued to uncover pathophysiological understanding of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis. Therefore, we aimed to explore whether and how antibody-mediated NMDAR dysfunction affected cortical and sub-cortical brain morphology and their relationship with clinical symptoms.
Morning light therapy in adults with Tourette’s disorder
Emily J. Ricketts, Helen J. Burgess, Gabrielle E. Montalbano, Meredith E. Coles, Joseph F. McGuire, Hardian Thamrin, Dana L. McMakin, James T. McCracken, Mary A. Carskadon, John Piacentini & Christopher S. Colwell
doi : 10.1007/s00415-021-10645-z
Journal of Neurology volume 269, pages 399–410 (2022)
Sleep disturbance is common among individuals with Tourette’s Disorder (TD). Given that sleep is influenced by the circadian system, this study examined circadian rhythms and sleep in adults with TD, and explored the possible benefit of short-wavelength wearable morning light therapy.
A systematic review of non-motor symptom evaluation in clinical trials for amyotrophic lateral sclerosis
Emily Beswick, Deborah Forbes, Zack Hassan, Charis Wong, Judith Newton, Alan Carson, Sharon Abrahams, Siddharthan Chandran & Suvankar Pal
doi : 10.1007/s00415-021-10651-1
Journal of Neurology volume 269, pages 411–426 (2022)
Amyotrophic lateral sclerosis (ALS) is increasingly recognised as a multi-system disorder, presenting with common and impactful non-motor symptoms, such as neuropsychiatric symtpoms, cognitive and behavioural changes, pain, disordered sleep, fatigue and problematic saliva.
Evaluation of a new body-focused group therapy versus a guided self-help group program for adults with psychogenic non-epileptic seizures (PNES): a pilot randomized controlled feasibility study
Philine Senf-Beckenbach, Matthias Hoheisel, Janine Devine, Arnina Frank, Laura Obermann, Matthias Rose & Kim Hinkelmann
doi : 10.1007/s00415-021-10652-0
Journal of Neurology volume 269, pages 427–436 (2022)
Psychogenic non-epileptic seizures (PNES), a common phenomenon in neurological settings, are regarded as a paroxysmal type of functional neurological disorder (FND). In a substantial proportion, PNES are disabling with poor long-term outcomes and high economic costs. Despite the clinical and financial consequences of PNES, there is still a lack of controlled clinical trials on the treatment of this challenging disorder. The study aims to evaluate the feasibility and collect first evidence of the efficacy of a group based-intervention in PNES-patients.
Monoallelic KIF1A-related disorders: a multicenter cross sectional study and systematic literature review
Stefania Della Vecchia, Alessandra Tessa, Claudia Dosi, Jacopo Baldacci, Rosa Pasquariello, Antonella Antenora, Guja Astrea, Maria Teresa Bassi, Roberta Battini, Carlo Casali, Ettore Cioffi, Greta Conti, Giovanna De Michele, Anna Rita Ferrari, Alessandro Filla, Chiara Fiorillo, Carlo Fusco, Salvatore Gallone, Chiara Germiniasi, Renzo Guerrini, Shalom Haggiag, Diego Lopergolo, Andrea Martinuzzi, Federico Melani, Andrea Mignarri, Elena Panzeri, Antonella Pini, Anna Maria Pinto, Francesca Pochiero, Guido Primiano, Elena Procopio, Alessandra Renieri, Romina Romaniello, Cristina Sancricca, Serenella Servidei, Carlotta Spagnoli, Chiara Ticci, Anna Rubegni & Filippo Maria Santorelli
doi : 10.1007/s00415-021-10792-3
Journal of Neurology volume 269, pages 437–450 (2022)
Monoallelic variants in the KIF1A gene are associated with a large set of clinical phenotypes including neurodevelopmental and neurodegenerative disorders, underpinned by a broad spectrum of central and peripheral nervous system involvement.
Correction to: Monoallelic KIF1A-related disorders: a multicenter cross sectional study and systematic literature review
Stefania Della Vecchia, Alessandra Tessa, Claudia Dosi, Jacopo Baldacci, Rosa Pasquariello, Antonella Antenora, Guja Astrea, Maria Teresa Bassi, Roberta Battini, Carlo Casali, Ettore Cioffi, Greta Conti, Giovanna De Michele, Anna Rita Ferrari, Alessandro Filla, Chiara Fiorillo, Carlo Fusco, Salvatore Gallone, Chiara Germiniasi, Renzo Guerrini, Shalom Haggiag, Diego Lopergolo, Andrea Martinuzzi, Federico Melani, Andrea Mignarri, Elena Panzeri, Antonella Pini, Anna Maria Pinto, Francesca Pochiero, Guido Primiano, Elena Procopio, Alessandra Renieri, Romina Romaniello, Cristina Sancricca, Serenella Servidei, Carlotta Spagnoli, Chiara Ticci, Anna Rubegni & Filippo Maria Santorelli
doi : 10.1007/s00415-021-10839-5
Journal of Neurology volume 269, page 451 (2022)
Treatment response scoring systems to assess long-term prognosis in self-injectable DMTs relapsing–remitting multiple sclerosis patients
Jordi Río, Àlex Rovira, Claudio Gasperini, Mar Tintoré, Luca Prosperini, Susana Otero-Romero, Manuel Comabella, Ángela Vidal-Jordana, Ingrid Galán, Luciana Midaglia, Breogán Rodriguez-Acevedo, Ana Zabalza, Joaquim Castilló, Georgina Arrambide, Carlos Nos, Álvaro Cobo, Carmen Tur, Cristina Auger, Jaume Sastre-Garriga & Xavier Montalban
doi : 10.1007/s00415-021-10823-z
Journal of Neurology volume 269, pages 452–459 (2022)
Different treatment response scoring systems in treated MS patients exist. The objective was to assess the long-term predictive value of these systems in RRMS patients treated with self-injectable DMTs.
Correction to: Treatment response scoring systems to assess long-term prognosis in self-injectable DMTs relapsing–remitting multiple sclerosis patients
Jordi Río, Àlex Rovira, Claudio Gasperini, Mar Tintoré, Luca Prosperini, Susana Otero-Romero, Manuel Comabella, Ángela Vidal-Jordana, Ingrid Galán, Luciana Midaglia, Breogán Rodriguez-Acevedo, Ana Zabalza, Joaquim Castilló, Georgina Arrambide, Carlos Nos, Álvaro Cobo-Calvo, Carmen Tur, Cristina Auger, Jaume Sastre-Garriga & Xavier Montalban
doi : 10.1007/s00415-021-10906-x
Journal of Neurology volume 269, page 460 (2022)
FLAIR2 post-processing: improving MS lesion detection in standard MS imaging protocols
Tobias Zrzavy, Alice Wielandner, Lukas Haider, Sophie Bartsch, Fritz Leutmezer, Thomas Berger, Karl Heinz Nenning, Alexander Rauscher, Paulus Rommer & Gregor Kasprian
doi : 10.1007/s00415-021-10833-x
Journal of Neurology volume 269, pages 461–467 (2022)
Technical improvements in magnetic resonance imaging (MRI) acquisition, such as higher field strength and optimized sequences, lead to better multiple sclerosis (MS) lesion detection and characterization. Multiplication of 3D-FLAIR with 3D-T2 sequences (FLAIR2) results in isovoxel images with increased contrast-to-noise ratio, increased white–gray-matter contrast, and improved MS lesion visualization without increasing MRI acquisition time. The current study aims to assess the potential of 3D-FLAIR2 in detecting cortical/leucocortical (LC), juxtacortical (JC), and white matter (WM) lesions.
Correction to: FLAIR2 post-processing: improving MS lesion detection in standard MS imaging protocols
Tobias Zrzavy, Alice Wielandner, Lukas Haider, Sophie Bartsch, Fritz Leutmezer, Thomas Berger, Karl Heinz Nenning, Alexander Rauscher, Paulus Rommer & Gregor Kasprian
doi : 10.1007/s00415-021-10855-5
Journal of Neurology volume 269, pages 468–469 (2022)
Off-label-dosing of non-vitamin K-dependent oral antagonists in AF patients before and after stroke: results of the prospective multicenter Berlin Atrial Fibrillation Registry
Serdar Tütüncü, Manuel Olma, Claudia Kunze, Joanna Dietzel, Johannes Schurig, Cornelia Fiessler, Carolin Malsch, Tobias Eberhard Haas, Boris Dimitrijeski, Wolfram Doehner, Georg Hagemann, Frank Hamilton, Martin Honermann, Gerhard Jan Jungehulsing, Andreas Kauert, Hans-Christian Koennecke, Bruno-Marcel Mackert, Darius Nabavi, Christian H. Nolte, Joschua Mirko Reis, Ingo Schmehl, Paul Sparenberg, Robert Stingele, Enrico Völzke, Carolin Waldschmidt, Daniel Zeise-Wehry, Peter U. Heuschmann, Matthias Endress & Karl Georg Haeusler
doi : 10.1007/s00415-021-10866-2
Journal of Neurology volume 269, pages 470–480 (2022)
We aimed to analyze prevalence and predictors of NOAC off-label under-dosing in AF patients before and after the index stroke.
Correction to: Off-label-dosing of non-vitamin K-dependent oral antagonists in AF patients before and after stroke: results of the prospective multicenter Berlin Atrial Fibrillation Registry
Serdar Tütüncü, Manuel Olma, Claudia Kunze, Joanna Dietzel, Johannes Schurig, Cornelia Fiessler, Carolin Malsch, Tobias Eberhard Haas, Boris Dimitrijeski, Wolfram Doehner, Georg Hagemann, Frank Hamilton, Martin Honermann, Gerhard Jan Jungehulsing, Andreas Kauert, Hans-Christian Koennecke, Bruno-Marcel Mackert, Darius Nabavi, Christian H. Nolte, Joschua Mirko Reis, Ingo Schmehl, Paul Sparenberg, Robert Stingele, Enrico Völzke, Carolin Waldschmidt, Daniel Zeise-Wehry, Peter U. Heuschmann, Matthias Endres & Karl Georg Haeusler
doi : 10.1007/s00415-021-10907-w
Journal of Neurology volume 269, pages 481–482 (2022)
Radiation-induced intracranial vasculitis on high-resolution vessel wall MRI
Wenjie Yang, John W. Krakauer & Bruce A. Wasserman
doi : 10.1007/s00415-021-10742-z
Journal of Neurology volume 269, pages 483–485 (2022)
Asymmetric rapidly progressive idiopathic normal-pressure hydrocephalus: description of a case
Paride Schito, Francesca Caso, Giuseppe Magnani, Lina Raffaella Barzaghi, Alessandra Barbieri, Maria Antonietta Volontè, Vittorio Martinelli, Luigia Brugliera, Sandro Iannaccone & Massimo Filippi
doi : 10.1007/s00415-021-10746-9
Journal of Neurology volume 269, pages 486–489 (2022)
The visually enhanced vestibulo-ocular reflex in CANVAS
Gábor M. Halmágyi, Kishore Kumar & Leigh A. McGarvie
doi : 10.1007/s00415-021-10755-8
Journal of Neurology volume 269, pages 490–492 (2022)
Interpretation of vaccine associated neurological adverse events: a methodological and historical review
Marija Cauchi, Harriet Ball, Yoav Ben-Shlomo & Neil Robertson
doi : 10.1007/s00415-021-10747-8
Journal of Neurology volume 269, pages 493–503 (2022)
As a result of significant recent scientific investment, the range of vaccines available for COVID-19 prevention continues to expand and uptake is increasing globally. Although initial trial safety data have been generally reassuring, a number of adverse events, including vaccine induced thrombosis and thrombocytopenia (VITT), have come to light which have the potential to undermine the success of the vaccination program. However, it can be difficult to interpret available data and put these into context and to communicate this effectively. In this review, we discuss contemporary methodologies employed to investigate possible associations between vaccination and adverse neurological outcomes and why determining causality can be challenging. We demonstrate these issues by discussing relevant historical exemplars and explore the relevance for the current pandemic and vaccination program. We also discuss challenges in understanding and communicating such risks to clinicians and the general population within the context of the ‘infodemic’ facilitated by the Internet and other media.
Autosomal recessive adult onset ataxia
Nataša Dragaševic-Miškovic, Iva Stankovic, Andona Milovanovic & Vladimir S. Kostic
doi : 10.1007/s00415-021-10763-8
Journal of Neurology volume 269, pages 504–533 (2022)
Autosomal recessive ataxias (ARCA) represent a complex group of diseases ranging from primary ataxias to rare and complex metabolic disorders in which ataxia is a part of the clinical picture. Small number of ARCA manifest exclusively in adulthood, while majority of typical childhood onset ARCA may also start later with atypical clinical presentation. We have systematically searched the literature for ARCA with adult onset, both in the group of primary ataxias including those that are less frequently described in isolated or in a small number of families, and also in the group of complex and metabolic diseases in which ataxia is only part of the clinical picture. We propose an algorithm that could be used when encountering a patient with adult onset sporadic or recessive ataxia in whom the acquired causes are excluded. ARCA are frequently neglected in the differential diagnosis of adult-onset ataxias. Rising awareness of their clinical significance is important, not only because some of these disorders may be potentially treatable, but also for prognostic implications and inclusion of patients to future clinical trials with disease modifying agents.
Intravenous immunoglobulin for inflammatory demyelinating polyneuropathy
M. Talaei & N. P. Robertson
doi : 10.1007/s00415-021-10908-9
Journal of Neurology volume 269, pages 534–536 (2022)
Gabriel Anton (1858–1933)
Andrzej Grzybowski & Joanna Zolnierz
doi : 10.1007/s00415-021-10662-y
Journal of Neurology volume 269, pages 537–538 (2022)
Correction to: Role of complement and potential of complement inhibitors in myasthenia gravis and neuromyelitis optica spectrum disorders: a brief review
Jayne L. Chamberlain, Saif Huda, Daniel H. Whittam, Marcelo Matiello, B. Paul Morgan & Anu Jacob
doi : 10.1007/s00415-021-10896-w
Journal of Neurology volume 269, page 539 (2022)
Correction to: Long-term effects of bilateral pallidal deep brain stimulation in dystonia: a follow-up between 8 and 16 years
P. Krause, S. Völzmann, S. Ewert, A. Kupsch, G. H. Schneider & Andrea A. Kühn
doi : 10.1007/s00415-021-10863-5
Journal of Neurology volume 269, page 540 (2022)
