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World Brain Day 2021 campaign continue to gain the momentum; join us to stop multiple sclerosis
TissaWijeratneabcWolfgangGrisolddWilliamCarrollfe
doi : 10.1016/j.jns.2021.117547
Volume 427, 15 August 2021, 117547
George Kenneth York III
David A.Steinberg
doi : 10.1016/j.jns.2021.117559
Volume 427, 15 August 2021, 117559
The potential role of miRNA therapies in spinal muscle atrophy
GayatriGandhiaSyahrilAbdullahbcdAgus IwanFoeadeWendy Wai YengYeoa
doi : 10.1016/j.jns.2021.117485
Volume 427, 15 August 2021, 117485
Spinal muscular atrophy (SMA) is a neurodegenerative disease caused by low levels of full-length survival motor neuron (SMN) protein due to the loss of the survival motor neuron 1 (SMN1) gene and inefficient splicing of the survival motor neuron 2 (SMN2) gene, which mostly affects alpha motor neurons of the lower spinal cord. Despite the U.S. Food and Drug Administration (FDA) approved SMN-dependent therapies including Nusinersen, Zolgensma® and Evrysdi™, SMA is still a devastating disease as these existing expensive drugs may not be sufficient and thus, remains a need for additional therapies. The involvement of microRNAs (miRNAs) in SMA is expanding because miRNAs are important mediators of gene expression as each miRNA could target a number of genes. Hence, miRNA-based therapy could be utilized in treating this genetic disorder. However, the delivery of miRNAs into the target cells remains an obstacle in SMA, as there is no effective delivery system to date. This review highlights the potential strategies for intracellular miRNA delivery into target cells and current challenges in miRNA delivery. Furthermore, we provide the future prospects of miRNA-based therapeutic strategies in SMA.
Stroke and stroke prevention in sickle cell anemia in developed and selected developing countries
PallabBhattacharyaaDeepaneetaSarmahaKunjan R.DavebAviragGoswamicMitsuyoshiWatanabedXinWangeKiranKaliaaNikolausPlesnilafDileep R.YavagalgOfeliaAlvarezh
doi : 10.1016/j.jns.2021.117510
Volume 427, 15 August 2021, 117510
This comprehensive review provides an insight into the pathophysiology, epidemiology, evaluation, and treatment of sickle cell anemia (SCA)-related stroke in developed and developing countries. Vascular injury, hypercoagulability and vaso-occlusion play a role in the pathophysiology of stroke in SCA. Transcranial Doppler ultrasound (TCD) has lowered the incidence of ischemic stroke from 11% to 1% as TCD identifies children who are at risk for stroke, providing opportunities for interventions to reduce this risk. Whereas blood exchange is indicated in acute stroke, chronic transfusions (either simple or exchange on a monthly basis) are used for primary as well as secondary stroke prevention in developed countries. Children with abnormally high TCD velocities (? 200 cm/s) are at high risk of stroke and might benefit from hydroxyurea or hydroxycarbamide (HU) after a period of a successful transition from chronic transfusions. Hematopoietic stem cell transplant presents a cure for SCA. Gene therapy is currently investigated and may be offered to patients with SCA who had a stroke or who are at high risk of stroke if proven efficacious and safe. However, gene therapy is not likely to be implemented in low-income countries due to cost. Alternatively, HU is utilized for primary and secondary stroke prevention in developing countries. Further expansion of TCD implementation should be a priority in those settings.
Efficacy of calcitonin gene-related peptide (CGRP) receptor blockers in reducing the number of monthly migraine headache days (MHDs): A network meta-analysis of randomized controlled trials
Ahmed TaherMasoudabMohammed TarekHasanbcAhmedSayedbdHarvey NabilEdwardbeAhmed MohamedAmerbfAbdelrahman ElshahatNagabcMohamedElfilgBadrah S.AlghamdihiAsmaPerveenjGhulam MdAshrafikEshak I.Bahbahbf
doi : 10.1016/j.jns.2021.117505
Volume 427, 15 August 2021, 117505
The Global burden of disease study ranked migraine as the sixth most common disorder worldwide in 2016, with significant social and economic sequelae. In this study, we assessed the efficacy of different Calcitonin gene-related peptide (CGRP) receptor blockers as potential pharmacological approaches and compare them to placebo using the systematic review (SR) and network meta-analysis (NMA) approach. We performed a computerized search of SCOPUS, PubMed, Cochrane central, and Embase databases through January 2019 and included randomized controlled trials (RCTs), which were performed on episodic and chronic migraine patients who used Erenumab, Eptinezumab, Fremanezumab, or Galcanezumab. The combined analysis revealed that after six, eight, and twelve weeks of intervention, the medications with the most potent effects in comparison to placebo were Fremanezumab 900 mg, (SMD = ?0.55, 95% CI [?0.97, ?0.12]); Erenumab 140 mg, (SMD = ?0.51, 95% CI [?0.61, 0.41]); and Erenumab 140 mg, (SMD = ?0.48, 95% CI [?0.571, 0.39]), respectively. For chronic migraine patients, Fremanezumab 900 mg, Erenumab 140 mg, in addition to Erenumab 70 mg, were associated with the highest efficacy after 6, 8, and 12 weeks, correspondingly. The analysis of combined groups data (Chronic and Episodic) showed that Fremanezumab was the most effective drug after six weeks, where Erenumab was the most effective after 8 and 12 weeks. The current evidence retrieved from this NMA suggests that Fremanezumab was the most effective anti-migraine medication in decreasing MHDs per month after six weeks in both chronic and episodic patients.
Clinical review of cerebral venous thrombosis in the context of COVID-19 vaccinations: Evaluation, management, and scientific questions
Kiran T.ThakuraArinaTamborskabcGreta K.WoodcEmilyMcNeillaDavidRohaImo J.AkpandEliza C.MilleraAlyssaBautistaaJanClaassenaCarla Y.KimaAllaGuekhteCarlos A.PardohOlajideWilliamsaDavidGarcía-AzorínkKameshwarPrasadiErichSchmutzhardjBenedict D.MichaelfSherry H.-Y.ChougAndrea S.WinklerlmTomSolomonbMitchell S.Elkinda
doi : 10.1016/j.jns.2021.117532
Volume 427, 15 August 2021, 117532
Vaccine induced immune mediated thrombocytopenia or VITT, is a recent and rare phenomenon of thrombosis with thrombocytopenia, frequently including cerebral venous thromboses (CVT), that has been described following vaccination with adenovirus vaccines ChAdOx1 nCOV-19 (AstraZeneca) and Ad26.COV2·S Johnson and Johnson (Janssen/J&J). The evaluation and management of suspected cases of CVT post COVID-19 vaccination are critical skills for a broad range of healthcare providers.
The impact of student debt on neurological practice
AbhimanyuMahajanaLongDavalosbLoganSchneidercMeaganBaileyaJaffarKhandZacharyLondonb
doi : 10.1016/j.jns.2021.117536
Volume 427, 15 August 2021, 117536
The median cost of attending medical school is rising annually, and with it, student debt. Neurology residents have stepped up during the pandemic to answer the call of a health system at its breaking point. In this article, we outline how this escalating problem of student debt affects the neurology pipeline, the wellbeing and career decisions of current neurology trainees and practicing neurologists and through it, and the gap in healthcare. We describe currently available options for loan repayment and call for advocacy and legislation to address this mounting burden as a means to improve neurological care in the United States.
Systemic mastocytosis: The roles of histamine and its receptors in the central nervous system disorders
PaolaDi MauroaRobertaAnzivinobMarisaDistefanocDavide DomenicoBorzìd
doi : 10.1016/j.jns.2021.117541
Volume 427, 15 August 2021, 117541
Mastocytosis is a rare disease of clonal hematological disorders characterized by a pathological accumulation of Mast Cells (MCs) in different tissues, with variable symptomatology and prognosis.
Classifying epilepsy pragmatically: Past, present, and future
Nathan A.ShlobinaGagandeepSinghbcgCharles R.NewtondeJosemir W.Sandercfg
doi : 10.1016/j.jns.2021.117515
Volume 427, 15 August 2021, 117515
The classification of epilepsy is essential for people with epilepsy and their families, healthcare providers, physicians and researchers. The International League Against Epilepsy proposed updated seizure and epilepsy classifications in 2017, while another four-dimensional epilepsy classification was updated in 2019. An Integrated Epilepsy Classification system was proposed in 2020. Existing classifications, however, lack consideration of important pragmatic factors relevant to the day-to-day life of people with epilepsy and stakeholders. Despite promising developments, consideration of comorbidities in brain development, genetic causes, and environmental triggers of epilepsy remains largely user-dependent in existing classifications. Demographics of epilepsy have changed over time, while existing classification schemes exhibit caveats. A pragmatic classification scheme should incorporate these factors to provide a nuanced classification. Validation across disparate contexts will ensure widespread applicability and ease of use. A team-based approach may simplify communication between healthcare personnel, while an individual-centred perspective may empower people with epilepsy. Together, incorporating these elements into a modern but pragmatic classification scheme may ensure optimal care for people with epilepsy by emphasising cohesiveness among its myriad users. Technological advancements such as 7T MRI, next-generation sequencing, and artificial intelligence may affect future classification efforts.
Development of parkinsonism after long-standing cervical dystonia – A cohort
BettinaBalintabEoinMulroyaFelixGövertcAnnaLatorreadGiuliaDi LazarroefRobertoErrogAmitBatlaaJanice L.HoltonhYasuoMikihThomas T.WarnerhKailash P.Bhatiaa
doi : 10.1016/j.jns.2021.117477
Volume 427, 15 August 2021, 117477
Dystonia occurring in the context of parkinsonism is well-known, e.g. as foot dystonia in young-onset Parkinson's disease (PD), anterocollis in multisystem atrophy (MSA) or blepharospasm (levator inhibition) in progressive supranuclear palsy. We have, however, encountered a series of patients whose phenotype differed from the above described entities.
Impact of revascularization therapies on outcome of posterior circulation ischemic stroke: The Indo-US stroke project
Sruthi S.NairaP.N.SylajaaJeyarajPandianbM.V. PadmaSrivastavacDheerajKhuranadSubhashKauleDeeptiArorabP. SankaraSarmafHimaniKhatterbAneesh B.Singhalg
doi : 10.1016/j.jns.2021.117499
Volume 427, 15 August 2021, 117499
Posterior circulation strokes (PCS) have been less extensively studied than anterior circulation strokes (ACS), especially regarding revascularization therapies. We analyzed the differences in baseline stroke characteristics, revascularization therapy and 3-month outcomes between PCS and ACS in a large prospective multicentre Indian stroke registry.
Sudomotor and cardiovascular autonomic function in de novo Parkinson's disease assessed by sudoscan and cardiovascular reflexes
CamillaRocchiaRoccoCerronibMatteoContibBenedettaLaurettiaNicola BiagioMercuriaAlessandroStefanibMariangelaPierantozzib
doi : 10.1016/j.jns.2021.117502
Volume 427, 15 August 2021, 117502
The prevalence of autonomic involvement in early stage of Parkinson ‘s disease (PD) is still debated. Aim of this study is to assess the autonomic functions in de novo PD patients (dnPD) in comparison with PD patients on therapy (PDot) and healthy controls (HC).
Seizure outcome after epilepsy surgery in tuberous sclerosis complex: Results and analysis of predictors from a multicenter study
C.VannicolaaL.TassibC.BarbacC.BoniverdM.CossubM.de CurtiseL.De PalmafI.D'ErricogG.DidatoeR.GuerrinicF.La BriolaaC.LuisidR.MaibF.MaricC.MarrasfM.MastrangelohA.PeronaijkN.SpecchiofI.ToldodK.TurneraA.VignoliailM.P.Caneviniai
doi : 10.1016/j.jns.2021.117506
Volume 427, 15 August 2021, 117506
Epilepsy surgery is recommended in selected patients with Tuberous Sclerosis Complex (TSC). However, reports on predictive factors of seizure outcome are variable.
Impact of acetylsalicylic acid (ASA) on postoperative hemorrhage in spinal lumbar surgery: Should preoperative ASA be discontinued for elective surgery?
AliRashidiFabianLeinsI. ErolSandalciogluMichaelLuchtmann
doi : 10.1016/j.jns.2021.117508
Volume 427, 15 August 2021, 117508
The application of acetylsalicylic acid (ASA) represents high evidence in the aging society due to primary and secondary prevention in cardiovascular disease and stroke. However, this presents a challenge for neurosurgeons in terms of preoperative and postoperative management of care. This study aimed to analyze the risk of bleeding by applying ASA before lumbar spinal surgery.
Associations between stroke type, stroke severity, and pre-stroke osteoporosis with the risk of post-stroke fracture: A nationwide population-based study
Hung-PingWanga1Sheng-FengSungb1Hsin-YiYangcWan-TingHuangcCheng-YangHsiehde
doi : 10.1016/j.jns.2021.117512
Volume 427, 15 August 2021, 117512
Recognizing the post-stroke fracture risk factors is crucial for targeted intervention and primary fracture prevention. We aimed to investigate whether stroke types, stroke severity, and pre-stroke osteoporosis are associated with post-stroke fracture.
Does an association between cigarette smoking and Parkinson's Disease-related psychosis exist? Insights from a large non-demented cohort
ClaudioTerravecchiaa1GiovanniMostileab1CristinaRascunàaGennarinaArabiacPaoloBaronedRobertoMarconieLetterioMorgantefAndreaQuattronecAlessandraNicolettiaMarioZappiaa
doi : 10.1016/j.jns.2021.117509
Volume 427, 15 August 2021, 117509
Parkinson's Disease-related Psychosis (PDP) encompasses a spectrum of symptoms ranging from “minor” hallucinations to formed hallucinations and delusions. Notably, cognitive impairment has been recognized as the strongest risk factor for PDP. Several evidences suggest a possible role of cigarette smoking in both cognition and psychotic syndromes.
Metabolic complications in myotonic dystrophy type 1: A cross-sectional survey using the National Registry of Japan
ManamiHamaaRihoHorieaTomoyaKubotaaTsuyoshiMatsumurabEnKimuracHarumasaNakamuracMasanori P.TakahashiaHirotoTakadad
doi : 10.1016/j.jns.2021.117511
Volume 427, 15 August 2021, 117511
Myotonic dystrophy type 1 (DM1) is the most common form of muscular dystrophy in adults, affecting multiple organs, including the eyes, heart, endocrine system, and central nervous system. The broad spectrum of DM1 symptoms has been attributed to the aberrant pre-mRNA splicing of various genes due to an abnormal expansion of the CTG repeat in the 3? untranslated region of the DMPK gene. The current challenge in the clinical care of DM1 is the lack of well-established protocols for the management of each organ disorder or symptom. Moreover, the current status of clinical management has not been adequately explored. Metabolic disturbance in DM1 has been less explored among the DM1 manifestations, even though impaired glucose tolerance is a widely known metabolic disorder associated with DM1. We investigated the metabolic disturbance related to DM1 using the national registry of neuromuscular diseases in Japan, Registry of Muscular Dystrophy (Remudy), and assessed the metabolic complications in DM1 and the current treatments. We obtained comprehensive information on the current status of liver dysfunction and dyslipidemia in a sizeable DM1 cohort (~300). We confirmed that the incidence of liver dysfunction and dyslipidemia, particularly hypertriglyceridemia, as well as impaired glucose tolerance, were significantly higher in DM1 patients. Furthermore, the majority of DM1 patients with dyslipidemia were not receiving pharmacotherapy. Our data highlight the current status of DM1 patients in Japan, which can guide the establishment of the standard of care for metabolic issues consequent to DM1.
Contemporary trends in antiepileptic drug treatment among women of childbearing age with epilepsy in the United States: 2004–2015
ShaunAjinkyaa1JonahFoxb1AlainLekoubouc
doi : 10.1016/j.jns.2021.117500
Volume 427, 15 August 2021, 117500
Certain antiepileptic drugs are associated with an increased risk for major congenital malformations (MCM). However, little is known regarding recent patterns of antiepileptic drug (ASM) prescriptions to women of childbearing age with epilepsy (WCE) in the United States.
Use of coffee grounds to test olfaction for predicting cognitive dysfunction and decline
Alexander J.Rajica1Peter S.Pressmanacd1Jonathan H.WoodcockacdHeidi J.ChialadfChristopher M.Filleyabcde
doi : 10.1016/j.jns.2021.117516
Volume 427, 15 August 2021, 117516
Our objective was to determine whether non-standardized testing of olfaction may provide useful information for predicting cognitive dysfunction and decline in patients with neurobehavioral disorders.
Cerebrospinal fluid in COVID-19 neurological complications: Neuroaxonal damage, anti-SARS-Cov2 antibodies but no evidence of cytokine storm
Maria A.GarciaabPaula V.BarrerasaAllieLewisaGabrielPinillacLori J.SokolldThomasKicklerdHebaMostafadMarioCatureglidAbhayMoghekaraKathryn C.FitzgeraldaHopkins Neuro-COVID-19 GroupCarlos A.Pardoad
doi : 10.1016/j.jns.2021.117517
Volume 427, 15 August 2021, 117517
To study in cerebrospinal fluid (CSF) of COVID-19 subjects if a “cytokine storm” or neuroinflammation are implicated in pathogenesis of neurological complications.
Prevalence of carotid web in a French cohort of cryptogenic stroke
C.TurpinataF.L.CollemichebC.ArquizanaN.MolinaricF.CagnazzobI.MourandaP.H.LefèvrebP.HennetondL.CortiaG.GascoubI.DerrazbS.OlindoeV.CostalatbC.DargazanlibN.Gaillarda
doi : 10.1016/j.jns.2021.117513
Volume 427, 15 August 2021, 117513
Carotid webs (CaW) may be an under-recognized cause of anterior circulation cryptogenic ischemic stroke (ACIS). Prevalence is still unknown in European patients with ACIS.
Burden and cost of comorbidities in patients with neuromyelitis optica spectrum disorder
AlexExuzidesaDanielSheinsonaParisSidiropoulosbFabioMagriniaShervinGholizadehaAndySurinachcLawrenceCookdCraig S.MeyeraMichaelYeamanefg
doi : 10.1016/j.jns.2021.117530
Volume 427, 15 August 2021, 117530
Neuromyelitis optica spectrum disorder (NMOSD) is associated with various comorbidities, including non-autoimmune and autoimmune conditions. The burden and cost of illness for NMOSD are unclear, particularly in the context of comorbidities.
Levels of bioactive adrenomedullin in plasma and cerebrospinal fluid in relation to delayed cerebral ischemia in patients after aneurysmal subarachnoid hemorrhage: A prospective observational study
MichaelVeldemanaRabiaDoganaMiriamWeissaChristianStoppebTim PhilippSimoncGernotMarxcHansClusmannaGerrit AlexanderSchubertaWalidAlbannaa
doi : 10.1016/j.jns.2021.117533
Volume 427, 15 August 2021, 117533
Adrenomedullin (ADM) has been identified as a promising biomarker of mortality and outcome in sepsis, heart failure and after major surgery. A recently developed assay specific for bioactive adrenomedullin (bio-ADM) has not yet been assessed in aneurysmal subarachnoid hemorrhage (aSAH). The objective of this prospective trial was to assess the time course of bio-ADM after aSAH in relation to the development of delayed cerebral ischemia (DCI) and its association with clinical outcome.
Clinical correlates of white matter lesions in Parkinson's disease using automated multi-modal segmentation measures
EricFangaMário JoãoFartariabcdChu NingAnneBénédicteMaréchalbcdTobiasKoberbcdJie XinLimfLeon Qi RongOoieCelesteCheneSoo LeeLimaEng KingTanegLing LingChanag
doi : 10.1016/j.jns.2021.117518
Volume 427, 15 August 2021, 117518
Age-related white matter lesions (WML) are common, impact neuronal connectivity, and affect motor function and cognition. In addition to pathological nigrostriatal losses, WML are also common co-morbidities in Parkinson's disease (PD) that affect postural stability and gait. Automated brain volume measures are increasingly incorporated into the clinical reporting workflow to facilitate precision in medicine. Recently, multi-modal segmentation algorithms have been developed to overcome challenges with WML quantification based on single-modality input.
Echocardiographic predictors of cardioembolic stroke due to underlying atrial fibrillation: Reliable left atrial remodeling signs in acute stroke?
KeikoShimamotoabHideakiKanzakiaTomotakaTanakacAtsushiHirayamadMasatoshiKogaeKengo F.KusanoaKazunoriToyodaeSatoshiYasudaafMasafumiIharac
doi : 10.1016/j.jns.2021.117514
Volume 427, 15 August 2021, 117514
Atrial remodeling due to high-burden atrial fibrillation (AF) is associated with cardioembolic stroke (CES). As not all CESs is caused by AF, we analyzed the diagnostic values of each echocardiographic parameter to distinguish likely AF-related CES in acute stroke patients while in non-AF rhythm.
Early detection of cognitive decline in mild cognitive impairment and Alzheimer's disease with a novel eye tracking test
KohTadokoroaToruYamashitaaYusukeFukuiabEmiNomuraaYasuyukiOhtaaSetsukoUenocSayaNishinacKeiichiroTsunodaacYosukeWakutanicYoshikiTakaocTakahiroMiyoshidYasutoHigashidYosukeOsakadaaRyoSasakiaNamikoMatsumotoaYukoKawaharaaYoshioOmoteaMamiTakemotoaNozomiHishikawaaRyutaMoriharaaKojiAbea
doi : 10.1016/j.jns.2021.117529
Volume 427, 15 August 2021, 117529
Due to an increasing number of dementia patients, the development of a rapid and sensitive method for cognitive assessment is awaited. Here, we examined the usefulness of a novel and short (3 min) eye tracking device to evaluate the cognitive function of normal control (NC, n = 52), mild cognitive impairment (MCI, n = 52), and Alzheimer's disease (AD, n = 70) subjects. Eye tracking total score declined significantly in MCI (**p < 0.01 vs NC) and AD (**p < 0.01 vs NC, ##p < 0.01 vs MCI), and correlated well with the mini-mental state examination (MMSE) score (r = 0.57, *p < 0.05). Furthermore, the eye tracking test, especially memory and deductive reasoning tasks, effectively discriminated NC, MCI and AD. The present novel eye tracking test clearly discriminated cognitive functions among NC, MCI, and AD subjects, thereby providing an advantage for the early detection of MCI and AD in screening.
Influence of current and previous smoking on current phenotype in Parkinson's disease
ShuichiroNeshigeabcTomohikoOhshitaaRyujiNeshigebHirofumiMaruyamaac
doi : 10.1016/j.jns.2021.117534
Volume 427, 15 August 2021, 117534
Although an inverse correlation between smoking and Parkinson's disease (PD) has been reported, research into the effect of smoking on current clinical progression remains limited for PD patients. Elucidation of a specific PD population who would benefit from smoking is challenging.
Frequency and factors associated with post-stroke seizures in a large multicenter study in West Africa
Fred S.SarfoaJoshuaAkinyemibAlbertAkpalucKolawoleWahabdJosephYariaeOladimejiAdebayoeMorenikeKomolafefReginaldObiakogLukmanOwolabihGodwin O.OsaigbovoiCarolynJenkinsjYawMensahaGodwinOgbolekBenedictCalys-TagoelPhilipAdebayogLambertAppiahaArtiSinghcAdekunleFakunlemEzinneUveremTiwariHemantnOlayemiBalogunoOsiAdeleyeoBimboFawalefAdeniyiAbdulwasiuoLuqmanOgunjimipOnasanyaAkinolapOyedunniArulogunmArnetteDonnaqOkechukwuOgahrRufusAkinyemiosBruceOvbiageletMayowa O.Owolabisuon behalf of SIREN
doi : 10.1016/j.jns.2021.117535
Volume 427, 15 August 2021, 117535
Post-stroke seizures (PSS) are associated with significant morbidity and mortality across the globe. There is a paucity of data on PSS in Africa.
IDH mutation status and the development of venous thromboembolism in astrocytoma patients
Jacob J.Mandela1MichaelYoussefg1ShlomitYust-KatzcdAkash J.PatelaAliJalaliaZiyiLieJiminWueEthan B.LudmirfJohn F.de Grootb
doi : 10.1016/j.jns.2021.117538
Volume 427, 15 August 2021, 117538
Venous thromboembolism (VTE) is a very common adverse event for astrocytoma patients, but validation of proposed risk biomarkers has been elusive. We examine whether the status of the isocitrate dehydrogenase (IDH) gene is a risk factor for the development of venous thromboembolism (VTE) in astrocytoma patients.
Serum Apo J as a potential marker of conversion from mild cognitive impairment to dementia
TommasoRomagnoliaBeatriceOrtolaniaJuana MariaSanzbdAlessandroTrentinibdDavideSeripacEdoardo DallaNoraaEleonoraCapattiaCarloCervellatiaAngelinaPassaroaGiovanniZulianiaGloriaBromboa
doi : 10.1016/j.jns.2021.117537
Volume 427, 15 August 2021, 117537
Apolipoprotein J (ApoJ) is present in both plasma and tissues, including brain. Growing evidence suggest that this protein may play an early role on the development of the two most common forms of dementia, Alzheimer's disease (AD) and vascular dementia (VD).
Low-dose tirofiban is associated with reduced in-hospital mortality in cardioembolic stroke patients treated with endovascular thrombectomy
WenboZhaoabc1JialiXua1SijieLicGuiyouLiubLongfeiWuaChuanhuiLidChuanjieWuaChanghongRenbJianCheneJiangangDuanfRuixianWanggHaiqingSongaQingfengMaadXunmingJibe
doi : 10.1016/j.jns.2021.117539
Volume 427, 15 August 2021, 117539
Whether tirofiban is safe and effective in cardioembolic stroke patients treated with endovascular thrombectomy (EVT) remains unknown; this study evaluated the safety and efficacy of low-dose tirofiban in this patients population.
Lyme neuroborreliosis-associated cerebrovascular events in the Finnish endemic area
MariaMironovaaElisaKortelabSatuKurkelacMariKanervabSamiCurtzea
doi : 10.1016/j.jns.2021.117544
Volume 427, 15 August 2021, 117544
Neuroborreliosis is a rare cause of cerebral vasculitis and stroke. The incidence of Lyme borreliosis in Finland has been increasing in the last 20 years, so we expect that Lyme neuroborreliosis–associated vasculitis can be a more common cause of stroke in the future.
Status epilepticus: Practice variation and adherence to treatment guideline in a large community hospital
Joni J.SairanenaAnne-MariKantanenaHarri T.HyppöläbReetta K.Kälviäinenac
doi : 10.1016/j.jns.2021.117542
Volume 427, 15 August 2021, 117542
To evaluate the treatment of status epilepticus (SE) and adherence to treatment guideline in a large Finnish community hospital.
Comorbidities worsen the prognosis of generalized myasthenia gravis post-thymectomy
Sini M.Laaksoab1ChrisMyllynena1DanielStrbianabSariAtulaab
doi : 10.1016/j.jns.2021.117549
Volume 427, 15 August 2021, 117549
The effect of comorbidities on the prognosis of myasthenia gravis (MG) remains unclear. In particular, the role of other autoimmune diseases (AD) is controversial.
Clinical effects associated with five-year retinal nerve fiber layer thinning in multiple sclerosis
DejanJakimovskiabRobertZivadinovabcCaila B.VaughnaOsmanOzelaBiancaWeinstock-Guttmana
doi : 10.1016/j.jns.2021.117552
Volume 427, 15 August 2021, 117552
Neurodegenerative changes in multiple sclerosis (MS) are associated with long-term disability progression (DP). Optical coherence tomography (OCT) measures may be used to monitor DP.
Bowel, bladder, and sudomotor symptoms in ALS patients
Verena C.SamaraabPatriciaJerantbSummerGibsonbMarkBrombergb
doi : 10.1016/j.jns.2021.117543
Volume 427, 15 August 2021, 117543
To describe prevalence rates of bowel, bladder, and sudomotor symptoms in patients with amyotrophic lateral sclerosis (ALS) in relation to disease onset and progression. Treatment strategies and efficacies were also assessed.
Mechanical thrombectomy for stroke patients anticoagulated with direct oral anticoagulants versus warfarin
JunpeiKogeaKantaTanakabHiroshiYamagamicTakeshiYoshimotodKazutakaUchidaeTakeshiMorimotofKazunoriToyodaaNobuyukiSakaigShinichiYoshimurae
doi : 10.1016/j.jns.2021.117545
Volume 427, 15 August 2021, 117545
Outcomes after mechanical thrombectomy (MT) for large vessel occlusion (LVO) were compared between stroke patients anticoagulated with direct oral anticoagulants (DOACs) and those anticoagulated with warfarin.
Bevacizumab for stereotactic radiosurgery-induced radiation necrosis in patients with non-small cell lung cancer treated with immune check-point inhibitors
AssafMooreab1ShlomitYust-Katzbc1OdedIchtaRuthEliyahoubdNoaGordonaAharon YehonatanCoheneIris MagdalenaGoldsteineNirPeledeTaliSeigalbcAlexandraAmielcElizabethDudnikbf
doi : 10.1016/j.jns.2021.117556
Volume 427, 15 August 2021, 117556
Bevacizumab was shown to be effective in the treatment of brain radiation necrosis (RN) attributed to the use of stereotactic radiosurgery (SRS). Data on its efficacy and safety in non-small cell lung cancer (NSCLC) patients treated with immune check-point inhibitors (ICI) is lacking.
An amyotrophic lateral sclerosis hot spot in the French Alps associated with genotoxic fungi
LagrangeE.aJ.P.VernouxbJ.ReiscgV.PalmerdW.CamueP.S.Spencerdf
doi : 10.1016/j.jns.2021.117558
Volume 427, 15 August 2021, 117558
Between 1990 and 2018, 14 cases of amyotrophic lateral sclerosis (ALS) were diagnosed in residents of, and in visitors with second homes to, a mountainous hamlet in the French Alps. Systematic investigation revealed a socio-professional network that connected ALS cases. Genetic risk factors for ALS were excluded. Several known environmental factors were scrutinized and eliminated, notably lead and other chemical contaminants in soil, water or home-grown vegetation used for food, radon and electromagnetic fields. Some lifestyle-related behavioral risk factors were identified: Prior to clinical onset of motor neuron disease, some patients had a high degree of athleticism and smoked tobacco. Recent investigations on site, based on a new hypothesis, showed that all patients had ingested wild mushrooms, notably poisonous False Morels. Half of the ALS cohort reported acute illness following Gyromitra gigas mushroom consumption. This finding supports the hypothesis that genotoxins of fungal origin may induce motor neuron degeneration.
Is the weekend effect true in acute stroke patients at tertiary stroke center?
SiljaRätyNicolasMartinez-MajanderOlliSuomalainenGerliSiboltMarjaanaTiainenKatiValkonenTiinaSairanenNinaForssSamiCurtze
doi : 10.1016/j.jns.2021.117557
Volume 427, 15 August 2021, 117557
There is contradicting evidence on the outcome of emergency patients treated during weekends versus weekdays. We studied if outcome of ischemic stroke patients receiving intravenous thrombolysis (IVT) differs according to the treatment time.
A minority of patients with functional seizures have abnormalities on neuroimaging
Wesley T.KerrabJohn K.LeeaAmir H.KarimiaHiroyukiTatekawacL. BrianHickmanadMichaelConnerneyaSiddhika S.SreenivasanaIshitaDubeyaCorinne H.AllasaJena M.SmithaIvankaSavicaeDaniel H.S.SilvermanfLubomir M.HadjiiskigNicholas J.BeimerhiWilliam C.StaceyhjMark S.CohenabckJeromeEngelJrablmJamie D.FeusnerbenopNorikoSalamonacJohn M.Sterna
doi : 10.1016/j.jns.2021.117548
Volume 427, 15 August 2021, 117548
Functional seizures often are managed incorrectly as a diagnosis of exclusion. However, a significant minority of patients with functional seizures may have abnormalities on neuroimaging that typically are associated with epilepsy, leading to diagnostic confusion. We evaluated the rate of epilepsy-associated findings on MRI, FDG-PET, and CT in patients with functional seizures.
Predicting short and long-term mortality after acute ischemic stroke using EHR
VidaAbediPhDacVenkateshAvulaMSaSeyed-MostafaRazaviMDeShreyaBavishiMBBSfDurgeshChaudharyMBBSbShimaShahjoueiMD, MPHbMingWangPhDdChristoph J.GriessenauerMDbgJiangLiMD, PhDaRaminZandMD, MPHb
doi : 10.1016/j.jns.2021.117560
Volume 427, 15 August 2021, 117560
Despite improvements in treatment, stroke remains a leading cause of mortality and long-term disability. In this study, we leveraged administrative data to build predictive models of short- and long-term post-stroke all-cause-mortality.
The costs of care from a US claims database in patients with neuromyelitis optica spectrum disorder
AlexExuzidesaDanielSheinsonaParisSidiropoulosaShervinGholizadehaFabioMagriniaAndySurinachbLawrenceCookcCraig S.MeyeraMichael R.Yeamandef
doi : 10.1016/j.jns.2021.117553
Volume 427, 15 August 2021, 117553
Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease of the central nervous system that often leads to severe disability. Patients with highly active NMOSD have approximately a 10-times higher hospital inpatient admission rate compared with patients without NMOSD. Accurate assessments of the impact of NMOSD treatments on the burdens of illness require quantitative metrics of these burdens, including costs of care.
Self-reported occupational functioning in persons with relapsing-remitting multiple sclerosis: Does personality matter?
K.van der HieleaD.A.M.van GorpbE.E.A.van EgmondabcdP.J.JongenefM.F.RenemangJ.J.L.van der KlinkhiE.P.J.ArnoldusdE.A.C.BeenakkerjJ.J.J.van EijkkS.T.F.M.FrequinlK.de GansmG.J.D.HengstmannE.HoitsmaoO.H.H.GerlachpW.I.M.VerhagenqM.A.P.HeeringsrH.A.M.MiddelkoopasL.H.Visserbd
doi : 10.1016/j.jns.2021.117561
Volume 427, 15 August 2021, 117561
Multiple sclerosis (MS) poses a major threat to sustainable employability. Identifying conditions and factors that promote work participation is of great importance. Our objective was to explore the contribution of personality traits in explaining occupational functioning in MS.
Long-term follow-up of facilitated subcutaneous immunoglobulin therapy in multifocal motor neuropathy
AliAl-ZuhairyaSøren H.SindrupbJohannesJakobsena
doi : 10.1016/j.jns.2021.117495
Volume 427, 15 August 2021, 117495
To assess the feasibility, efficacy and patient satisfaction of long-term facilitated subcutaneous immunoglobulin therapy (fSCIG) in multifocal motor neuropathy (MMN).
Personalizing ocrelizumab treatment in Multiple Sclerosis: What can we learn from Sars-Cov2 pandemic?
F.Tazzaa1C.Lapucciab1M.CellerinoaG.BoffaaG.NovicI.PoirecE.MancusoaN.BruschiaE.SbragiaaA.LaroniacE.CapelloacM.Ingleseac
doi : 10.1016/j.jns.2021.117501
Volume 427, 15 August 2021, 117501
During SARS-CoV-2 pandemic, we adopted a personalized delayed protocol for ocrelizumab infusions in Relapsing Remitting Multiple Sclerosis (RRMS) patients according to the national recommendations. Out of the 83 RRMS patients whose infusion was scheduled between March and December 2020, 56 patients experienced a delay in treatment based on MS severity and SARS-CoV2 infection risk profile. In most cases, the immunophenotype was performed monthly to guide re-infusions. Specifically, B CD19 + cells repopulation rate was monitored. Mean infusion delay was 103,1 [SD 40,6] days, and none of the patients presented relapses or active disease at MRI at the end of the observation period. Treatment naïve status and the interval between immunophenotyping and the last ocrelizumab infusion were predictors of earlier B CD19 + cells repopulation. Two patients contracted SARS-CoV2 with complete recovery. Definitive data about Sars-Cov2 vaccine efficacy in patients treated with ocrelizumab are still lacking. Our findings suggest that a personalized treatment with a delayed infusion schedule does not compromise ocrelizumab short-term efficacy and may help to lengthen the therapeutic window for an effective response to SARS-CoV2 vaccine.
Rare mutations in ATL3, SPTLC2 and SCN9A explaining hereditary sensory neuropathy and congenital insensitivity to pain in a Brazilian cohort
Vivian PedigoneCintraabMaike F.DohrnbcPedro JoséTomaselliaFernanda BarbosaFigueiredoaSandra ElisabeteMarquesaSarah TeixeiraCamargosdLuiz Sergio MagesteBarbosadAdrianaP. RebelobLisaAbreubMattDanzibWilsonMarquesJra1StephanZüchnerb1
doi : 10.1016/j.jns.2021.117498
Volume 427, 15 August 2021, 117498
Hereditary sensory neuropathies (HSN) are a group of rare neurological disorders with heterogeneous clinical and genetic characteristics. Although at least 17 different genes have already been associated with HSN, the epidemiology of the disorder in Brazil is still unknown. Performing whole genome sequencing (WGS) in 23 unrelated Brazilian families diagnosed with HSN, we detected pathogenic variants in ATL3, SPTLC2, and SCN9A in 12 patients belonging to five unrelated families. Clinical features associated with heterozygous mutations in ATL3 (c.575A > G; p.(Tyr192Cys)) and SPTLC2 (c.529A > G; p.(Asn177Asp)) were sensory deficits, neuropathic pain, and recurrent ulcerations. Presenting as congenital insensitivity to pain, three unrelated probands carried biallelic loss-of-function mutations in SCN9A. The so far undescribed stop mutation c.2106G > A (p.(Trp702Ter)) and the likewise novel splicing variant c.3319-1G > A were found in compound-heterozygosity with, respectively, the known pathogenic variants c.2908G > T (p.Trp970Ter) and c.2690G > A (p.Glu897Ter). In total, we identified pathogenic mutations in 21.7% of our families, which suggests that most of the cases could be explained by yet to be discovered genes or unusual alleles. Our study represents the first mutational screen in a Brazilian HSN cohort, enabling additional insights for genotype-phenotype correlations, reducing misdiagnoses, and providing early treatment considerations.
Serum neurofilament light is a sensitive biomarker that reflects grey matter volume in Japanese patients with multiple sclerosis
JuichiFujimoriIchiroNakashima
doi : 10.1016/j.jns.2021.117528
Volume 427, 15 August 2021, 117528
To evaluate the degree of neuroaxonal injury in Japanese multiple sclerosis (MS) patients using serum neurofilament light (sNfL) and to investigate the relationship of sNFL with the degree of brain volume.
Clinical management of chronic inflammatory demyelinating polyneuropathy (CIDP) in Europe and India: An exploratory study
Martin K.R.Sva?inaaMan MohanMehndirattabChristian A.VedelercdYogeshSharmabIljaBobylevaAlinaSprengeraGinaRemkeaHaukeWüstenbergaInesKleinaAbhijeetJoshiaHelmar C.Lehmanna
doi : 10.1016/j.jns.2021.117507
Volume 427, 15 August 2021, 117507
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disorder causing inflammatory demyelination of peripheral nerves and consecutive disability. Diagnostic criteria and treatments are well established, but it is unknown how clinical practice may differ in different geographical regions.
Quantitative and qualitative analyses of herbal medication use among Ghanaian stroke survivors
Fred StephenSarfoabBruceOvbiagelec
doi : 10.1016/j.jns.2021.117540
Volume 427, 15 August 2021, 117540
Herbal medicines are not regulated by regulatory authorities and are often not of proven safety and efficacy. Anecdotal reports suggest widespread use of traditional herbal medicine (THM) for treatment of stroke in Africa, but verifiable data are limited in published literature.
Carotid webs in large vessel occlusion stroke: Clinical, radiological and thrombus histopathological findings
AuroraSemeranoadZakariaMamadouaJean PhilippeDesillesbcCandiceSabbenaMarcoBacigaluppidMichelPiotinbcMickaelMazighibcLucasDi MegliobcMichaelObadiaa
doi : 10.1016/j.jns.2021.117550
Volume 427, 15 August 2021, 117550
Carotid webs are an under-recognized embolic source in patients with cryptogenic stroke. Limited resources currently exist to assist clinicians in stroke prevention for patients with symptomatic carotid webs (SCW). We aimed at analysing the clinical, radiological and procedural features of stroke patients with SCW undergoing endovascular thrombectomy (EVT), and to describe the histopathological composition of their occlusive thrombi.
Treatment of progressive multifocal Leukoencephalopathy associated with idiopathic lymphocytopenia with Nivolumab
LauraFischbachaTobiasBaueraTheodorRüberaMarcusGrobe-EinslerbcAileenSitterdAlexanderRadbrucheOliverKautb
doi : 10.1016/j.jns.2021.117503
Volume 427, 15 August 2021, 117503
Potentials to alleviate coagulopathy and enhance microglial function of beta (?)- glucans, making them worth a clinical study for COVID-19's neurological sequalae
KadalrajaRaghavanaRamesh ShankarKandaswamybNobunaoIkewakicdMasaruIwasakieSamuel J.K.Abrahamefg
doi : 10.1016/j.jns.2021.117554
Volume 427, 15 August 2021, 117554
Comment on “Wernicke-Korsakoff syndrome despite no alcohol abuse: A summary of systematic reports” – A matter of bariatric patients' management
NunzioVelottiaAntonioVitielloaGiovannaBerardiaMarcoMilonebMarioMusellaa
doi : 10.1016/j.jns.2021.117569
Volume 427, 15 August 2021, 117569
Bariatric Wernicke's encephalopathy
ErikOudman
doi : 10.1016/j.jns.2021.117567
Volume 427, 15 August 2021, 117567
Neuroimaging findings in rheumatologic disorders
Sedat GirayKandemirliGirishBathla
doi : 10.1016/j.jns.2021.117531
Volume 427, 15 August 2021, 117531
Patients with rheumatological diseases may present with neurological manifestations of peripheral and/or central nervous system (CNS). Symptoms may be related to underlying rheumatological disease or CNS effects of immune-modulating drugs. Early diagnosis and therapy may help prevent serious complications. Magnetic resonance imaging (MRI), given its excellent soft tissue details, is the preferred imaging modality when evaluating patients with rheumatological disease and suspected CNS involvement. We present a review of the neuroimaging manifestations of various rheumatic diseases with emphasis on the imaging findings on MRI.
Infectious ophthalmoplegias
RajeevRanjanDeependraSinghKarthik VinayMaheshAastha KapilaTakkarSucharitaRayVivekLal
doi : 10.1016/j.jns.2021.117504
Volume 427, 15 August 2021, 117504
Though infections account for a significant proportion of patients with ocular motor palsies, there is surprising paucity of literature on infectious ophthalmoplegias. Almost all types of infectious agents (bacteria, viruses, fungi and parasites) can lead to ocular motor palsies. The causative infectious agent can be diagnosed in most cases using an orderly stepwise approach. In this review we discuss how to approach a patient with ophthalmoplegia with main focus on infectious etiologies.
Neurocysticercosis. A frequent cause of seizures, epilepsy, and other neurological morbidity in most of the world
J.BustosabI.GonzalesaH.SaavedraaS.HandalicH.H.Garciaabfor the Cysticercosis Working Group in Peru
doi : 10.1016/j.jns.2021.117527
Volume 427, 15 August 2021, 117527
Neurocysticercosis is endemic in most of the world and in endemic areas it accounts for approximately 30% of cases of epilepsy. Appropriate diagnosis and management of neurocysticercosis requires understanding the diverse presentations of the disease since these will vary in regards to clinical manifestation, sensitivity of diagnostic tests, and most importantly, therapeutic approach. This review attempts to familiarize tropical neurology practitioners with the diverse types of neurocysticercosis and the more appropriate management approaches for each.
Characterization of adult patients with Guillain–Barré syndrome during the arboviral infection outbreaks in Honduras
Odalis A.SánchezaKendy M.PortilloaSelvin Z.Reyes-GarciabJohn D.EnglandcMarco T.Medinab
doi : 10.1016/j.jns.2021.117551
Volume 427, 15 August 2021, 117551
Arbovirus infections have been associated with a wide spectrum of neurological manifestations. Among these, Guillain-Barré syndrome (GBS) is one of the most common. This study describes the characteristics of GBS associated with arbovirus infections during the outbreak which occurred in Honduras from January 2016 to February 2019. This was an observational retrospective study of adult patients who were diagnosed with GBS during that time. The diagnosis of GBS was based upon the criteria first published by Asbury, et al. and subsequently revised as the Brighton Criteria. A total of 91 patients with GBS constituted the study population. RT-PCR tests for ZIKV, CHIKV, and DENV arboviruses were performed in 47 (52%) of the patients. Of the tested population, 8/47 were positive for one of the arboviruses (5/8 for ZIKV, 3/8 for CHIKV; 0/8 for DENV). The clinical profile of the eight cases with GBS and arboviral infection did not differ significantly from the GBS patients who tested negative for ZIKV and CHIKV. In the cases with GBS and ZIKV, a parainfectious onset of the disease was suggested. Although not a strikingly large number of patients with GBS and arbovirus infection were seen, the close temporal relationship in these eight cases suggests an arbovirus (ZIKV and CHIKV) etiology.
