Risk factors for in-hospital and follow-up mortality after childhood arterial ischemic stroke
ConclusionsThis study identified groups with greater acute and long-term  mortality after a first episode of AIS in childhood. Specific interventions focused on these risk groups may decrease mortality rates. Further studies need to confirm our findings by adding children from other centers. (Source: Journal of Neurology)
Source: Journal of Neurology - May 14, 2019 Category: Neurology Source Type: research

Prognostic factors in ALS: a comparison between Germany and China
AbstractObjectiveSeveral independent prognostic factors, such as age of onset, type of onset, body mass index (BMI), and progression rate have been identified for amyotrophic lateral sclerosis (ALS) in Caucasians. The aim of this study was to identify such factors in Chinese patients and to compare their impact with German patients.MethodsComparison of prognostic factors was based on two hospital-based registries. The registry of the German Network for Motor Neuron Diseases contains 3100 patients with ALS. The Chinese registry comprises 2101 patients who were collected between 2003 and 2015 in the metropolitan area of Beij...
Source: Journal of Neurology - May 14, 2019 Category: Neurology Source Type: research

A cluster of disseminated small cortical lesions in MELAS: its distinctive clinical and neuroimaging features
AbstractObjectivesTo investigate a diversity of stroke-like episodes (SLEs) in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), and report a disseminated form of SLEs (D-SLEs) attributed to a cluster of disseminated small cortical lesions.MethodsWe retrospectively reviewed the clinical information of 27 MELAS patients seen at Kitasato University Hospital between January 1990 and April 2018. Among those, we selected 13 patients with m.3243A>G mutation [median age at onset, 35  years (11–68 years), two pediatric onset 
Source: Journal of Neurology - May 14, 2019 Category: Neurology Source Type: research

Chronic headache with vasospasm treated with nimodipine
(Source: Journal of Neurology)
Source: Journal of Neurology - May 14, 2019 Category: Neurology Source Type: research

Gerald M. Edelman (1929 –2014)
(Source: Journal of Neurology)
Source: Journal of Neurology - May 14, 2019 Category: Neurology Source Type: research

Thrombus hallmarks reveal atherothrombotic stroke aetiology
(Source: Journal of Neurology)
Source: Journal of Neurology - May 14, 2019 Category: Neurology Source Type: research

Acute posterior multifocal placoid pigment epitheliopathy after alemtuzumab treatment for relapsing –remitting multiple sclerosis
(Source: Journal of Neurology)
Source: Journal of Neurology - May 14, 2019 Category: Neurology Source Type: research

What is the role of the cerebellum in the pathophysiology of dystonia?
(Source: Journal of Neurology)
Source: Journal of Neurology - May 14, 2019 Category: Neurology Source Type: research

Neurological adverse events post allogeneic hematopoietic cell transplantation: major determinants of morbidity and mortality
ConclusionsOur large study with long-term follow-up highlights the wide spectrum of neurological complications in alloHCT. Accurate recognition is required for adequate management, a major determinant of survival. Thus, long-term increased awareness and collaboration between expert physicians is warranted. (Source: Journal of Neurology)
Source: Journal of Neurology - May 13, 2019 Category: Neurology Source Type: research

Survey of motion sickness susceptibility in children and adolescents aged 3  months to 18 years
AbstractWe conducted two representative, cross-sectional, population-based surveys on the susceptibility to motion sickness (MSS) in childhood: One was targeted at parents with children aged 6  months to 18 years (7569 households approached) and the other at parents with children aged 3 months to 5 years (12,720 households approached). In both surveys 3285 parents provided information on 5041 children. The main findings in the first survey were: 369 children (9.2%) were susceptible to motion sickness with a slight female preponderance, and in the second study 16 (1.2%) were susceptible; first occurrence...
Source: Journal of Neurology - May 13, 2019 Category: Neurology Source Type: research

Amyotrophy, cerebellar impairment and psychiatric disease are the main symptoms in a cohort of 14 Czech patients with the late-onset form of Tay –Sachs disease
ConclusionLOTS seems to be an underdiagnosed cause of progressive distal motor neuron disease, with variably expressed cerebellar impairment and psychiatric symptomatology in our group of adolescent and adult patients. The enzyme assay of β-hexosaminidase A in serum/plasma is a rapid and reliable tool to verify clinical suspicions. (Source: Journal of Neurology)
Source: Journal of Neurology - May 10, 2019 Category: Neurology Source Type: research

Real-world pharmacological treatment patterns of patients with young-onset Parkinson ’s disease in Japan: a medical claims database analysis
ConclusionsDopamine agonists were most frequently prescribed during the study period and were the initial treatment of choice. We also observed a change in levodopa equivalent daily dose over the disease course. This study provides a descriptive overview of real-world prescribing patterns in young-onset Parkinson ’s disease patients. (Source: Journal of Neurology)
Source: Journal of Neurology - May 10, 2019 Category: Neurology Source Type: research

Neuronal loss or dysfunction in patients with early Lyme neuroborreliosis: a proton magnetic resonance spectroscopy study of the brain
ConclusionA significant reduction in NAA/Cr ratio in comparison with the controls suggests the presence of diffuse neuronal loss in patients with early LNB. (Source: Journal of Neurology)
Source: Journal of Neurology - May 10, 2019 Category: Neurology Source Type: research

Electrical brain stimulation of the parietal lobe impairs the perception of verticality
(Source: Journal of Neurology)
Source: Journal of Neurology - May 10, 2019 Category: Neurology Source Type: research

Does the MDS-UPDRS provide the precision to assess progression in early Parkinson ’s disease? Learnings from the Parkinson’s progression marker initiative cohort
ConclusionsThe MDS-UPDRS-II and -III have psychometric limitations which limits the precision of measurement of motor symptoms and impact in early PD. This can lead to insensitivity in detecting differences and clinical change. Importantly, the diagnostic psychometric evidence provided by the RMT analysis provides a clear starting point for how to improve the quantification of clinically relevant concepts to characterize the course of early PD. (Source: Journal of Neurology)
Source: Journal of Neurology - May 9, 2019 Category: Neurology Source Type: research

Selective suppression of the vestibulo-ocular reflex during human locomotion
ConclusionThe vertical aVOR appears to be suppressed during faster walking and running, whereas at the same time, the predictability of resultant head movements increases. This suggests that during stereotyped human locomotion, internal feed-forward commands supplement or even suppress sensory feedback to mediate gaze stabilization in the vertical plane. (Source: Journal of Neurology)
Source: Journal of Neurology - May 9, 2019 Category: Neurology Source Type: research

Intact vestibular function is relevant for anxiety related to vertigo
(Source: Journal of Neurology)
Source: Journal of Neurology - May 9, 2019 Category: Neurology Source Type: research

Multi-gene testing in neurological disorders showed an improved diagnostic yield: data from over 1000 Indian patients
ConclusionIn our study, we observed an improved performance of multi-gene panel testing, with an overall diagnostic yield of 40%. Furthermore, we show that NGS (next-generation sequencing)-based testing is comprehensive and can detect all types of variants including structural variants. It can be considered as a single-platform genetic test for neurological disorders that can provide a swift and definitive diagnosis in a cost-effective manner. (Source: Journal of Neurology)
Source: Journal of Neurology - May 8, 2019 Category: Neurology Source Type: research

Seizures and epilepsy in multiple sclerosis: epidemiology and prognosis in a large tertiary referral center
ConclusionOur study shows a lower number of seizures and epilepsy in MS than previously reported. While a single seizure in MS usually has a good prognosis, relapse-associated seizures and established epilepsy in MS may not be as benign as previously assumed. (Source: Journal of Neurology)
Source: Journal of Neurology - May 8, 2019 Category: Neurology Source Type: research

Video head impulse test contributes to Susac syndrome diagnosis
(Source: Journal of Neurology)
Source: Journal of Neurology - May 7, 2019 Category: Neurology Source Type: research

Idiopathic acute high-tone sensorineural hearing loss accompanied by vertigo: vestibulo-cochlear artery syndrome? Consideration based on VEMP and vHIT
(Source: Journal of Neurology)
Source: Journal of Neurology - May 7, 2019 Category: Neurology Source Type: research

Insufficient image quality
(Source: Journal of Neurology)
Source: Journal of Neurology - May 6, 2019 Category: Neurology Source Type: research

Can histologically normal epileptogenic zone share common electrophysiological phenotypes with focal cortical dysplasia? SEEG-based study in MRI-negative epileptic patients
ConclusionsHistologically normal and FCD-associated epileptogenic zones share distinct interictal and ictal electrophysiological phenotypes, with common patterns between FCD subtypes and between dysplastic and apparently normal brain.SignificanceSome specific seizure-onset patterns seem to be predictive of the underlying histology and may help to detect an MRI-invisible FCD. (Source: Journal of Neurology)
Source: Journal of Neurology - May 4, 2019 Category: Neurology Source Type: research

Season of birth and multiple sclerosis: a systematic review and multivariate meta-analysis
AbstractSeason of birth is considered to be associated with multiple sclerosis (MS) although some findings opposing to this assumption raise doubts about the seasonality pattern in MS births. The present work synthesizes the evidence of previous published studies aiming at examining whether the month of birth is associated with a higher number of MS births. Pubmed and Scopus were systematically searched and a multivariate meta-analysis of case –control studies was conducted. Data of healthy controls births were retrieved from census reports when not included in the studies. For comparisons, October was set as a refer...
Source: Journal of Neurology - May 4, 2019 Category: Neurology Source Type: research

Screening performance of abbreviated versions of the UPSIT smell test
ConclusionUsing abbreviated smell tests could provide a cost-effective means of large-scale hyposmia screening, allowing more targeted UPSIT administration in general and PD-related settings. (Source: Journal of Neurology)
Source: Journal of Neurology - May 3, 2019 Category: Neurology Source Type: research

Treatment of cervical dystonia with abo- and onabotulinumtoxinA: long-term safety and efficacy in daily clinical practice
ConclusionWe show that treatment of cervical dystonia with the two most frequently used botulinum toxin A preparations is a safe and effective therapy even over a long treatment duration of up to 27  years. (Source: Journal of Neurology)
Source: Journal of Neurology - May 2, 2019 Category: Neurology Source Type: research

Approach to an experimental model of Mal de Debarquement Syndrome
ConclusionSubsequent neurophysiological and imaging examinations are required to investigate whether the model of transient, experimental MdDS actually shares a common substrate with the enduring pathological condition of MdDS. (Source: Journal of Neurology)
Source: Journal of Neurology - May 2, 2019 Category: Neurology Source Type: research

Cortical microinfarcts in patients with multiple lobar microbleeds on 3  T MRI
AbstractThe pathogenesis of cortical microinfarcts (CMIs) is considered to be heterogeneous including cerebral small vessel disease (SVD) such as hypertensive vasculopathy (HV) and cerebral amyloid angiopathy (CAA). Recent advances in MRI have enabled the detection of CMIs in vivo. To investigate the characteristics of CMIs in advanced cerebral SVD, we performed a retrospective analysis of 85 patients with cognitive impairment who had multiple lobar cerebral microbleeds (CMBs) on 3  T MRI. Among them, 41 (48.2%) patients were classified into the strictly lobar CMB group (i.e. probable-CAA group), and 44 (51.8%) patien...
Source: Journal of Neurology - May 2, 2019 Category: Neurology Source Type: research

The observation period after clinical brain death diagnosis according to ancillary tests: differences between supratentorial and infratentorial brain injury
ConclusionsWe found important differences in the confirmation of BD diagnosis between primary supratentorial and infratentorial lesion, and identified an optimal OBP of 2  h in patients with supratentorial lesions. By contrast, in primary posterior fossa/infratentorial lesions, the determination of an optimal OPB remains less accurate and hence more challenging. (Source: Journal of Neurology)
Source: Journal of Neurology - May 2, 2019 Category: Neurology Source Type: research

Specific mechanisms of subarachnoid hemorrhage accompanied by ischemic stroke in essential thrombocythemia: two case reports and a literature review
ConclusionsThe current study describes rare cases of SAH accompanied by ischemic stroke secondary to ET along with a review of the current literature, implying specific mechanisms for cerebral artery disorders associated withJAK2 V617F mutation. (Source: Journal of Neurology)
Source: Journal of Neurology - May 2, 2019 Category: Neurology Source Type: research

Color perception impairment following optic neuritis and its association with retinal atrophy
ConclusionsImpaired chromatic discrimination thresholds quantitatively document persistent functional complaints after ON. There is evidence of dysfunction in both the affected eye and the fellow eye. (Source: Journal of Neurology)
Source: Journal of Neurology - April 16, 2019 Category: Neurology Source Type: research

Crossed aphasia confirmed by fMRI in a case with nonfluent variant of primary progressive aphasia carrying a GRN mutation
We report a case of a 58-year-old woman with nfvPPA diagnosis (age at onset  = 55) previously described as a crossed aphasia case with progranulin mutation. At 2 years from the first visit, patient underwent 3DT1-weighted and a task-based functional MRI (fMRI). During the fMRI task, she was asked to perform a letter fluency test as the task of interest and to count for ward as the control condition. Image processing and data analysis were performed using SPM12 and the effect of each task was tested atp 
Source: Journal of Neurology - April 16, 2019 Category: Neurology Source Type: research

Postural control during galvanic vestibular stimulation in patients with persistent perceptual –postural dizziness
AbstractOver the past years galvanic vestibular stimulation (GVS) has been increasingly applied to stimulate the vestibular system in health and disease, but not in patients with persistent postural –perceptual dizziness (PPPD) yet. We functionally tested motion perception thresholds and postural responses to imperceptible noisy (nGVS) and perceptible bimastoidal GVS intensities in patients with PPPD with normal vestibulo-ocular reflexes. We hypothesized that GVS destabilizes PPPD patients un der simple postural conditions stronger compared to healthy controls. They were compared to healthy subjects under several con...
Source: Journal of Neurology - April 16, 2019 Category: Neurology Source Type: research

Predicting cognitive decline with non-clinical markers in Parkinson ’s disease (PRECODE-2)
AbstractObjectivesTo investigate whether baseline [123I]FP-CIT SPECT and CSF markers can predict cognitive impairment (CI) in PD patients, and provide a profile of those most at risk.Methods262 de novo PD patients from the Parkinson ’s Progression Markers Initiative database were stratified into two CI groups at the 36-month follow-up: MoCA-defined diagnosis: PD patients who had a MoCA score 
Source: Journal of Neurology - April 16, 2019 Category: Neurology Source Type: research

Ocrelizumab efficacy in subgroups of patients with relapsing multiple sclerosis
ConclusionsThe treatment effect of ocrelizumab versus IFN β-1a, measured by clinical and MRI outcomes, was maintained across most of the subgroups and strata of interest, and the pattern of treatment benefit across all subgroups was consistent with that from the pooled OPERA studies. (Source: Journal of Neurology)
Source: Journal of Neurology - April 16, 2019 Category: Neurology Source Type: research

Writing, reading, and speaking in blepharospasm
AbstractThe aim of the study was to evaluate the effects of writing, reading, and speaking on orbiculari oculi (OO) muscle spasms and on the blink rate in patients with blepharospasm (BSP). Patients with hemifacial spasm (HFS) and healthy subjects (HS) acted as control subjects. Thirty patients with BSP, 20 patients with primary HFS and 20 age-matched healthy subjects were videotaped according to a standardized procedure: at rest with eyes open; while writing a standard sentence on paper; while writing a standard sentence on a blackboard keeping the head straight; during a conversation based on a simple topic (speaking tas...
Source: Journal of Neurology - April 16, 2019 Category: Neurology Source Type: research

Misdiagnoses and delay of diagnoses in Moyamoya angiopathy —a large Caucasian case series
ConclusionsThis is the first systematic report which shows that patients with MMA are at high risk to be falsely diagnosed and treated. Depiction of typical vascular abnormalities in angiopathy is essential. Normal CSF cell counts, negative oligoclonal bands, and lack of infratentorial lesions as well as gadolinium-positive T1 lesions on MRI may be red flags differentiating this vasculopathy from vasculitis and MS. (Source: Journal of Neurology)
Source: Journal of Neurology - April 16, 2019 Category: Neurology Source Type: research

Resolution of apathy after dorsal instead of ventral subthalamic deep brain stimulation for Parkinson ’s disease
(Source: Journal of Neurology)
Source: Journal of Neurology - April 16, 2019 Category: Neurology Source Type: research

Two cases of diabetes mellitus type 1 after alemtuzumab treatment for multiple sclerosis: another probable secondary autoimmune disease
(Source: Journal of Neurology)
Source: Journal of Neurology - April 16, 2019 Category: Neurology Source Type: research

Motor, cognitive and behavioral differences in MDS PSP phenotypes
ConclusionAvailable clinical assessments hardly capture differences between PSP phenotypes. The cognitive testing differentiating the PSP phenotypes were semantic fluency and ideomotor apraxia. In PSP, mild cognitive impairment likely represents an intermediate step from normal cognition to dementia. The only marker of PSP non-Richardson ’s syndrome phenotype was better performance in visuo-spatial testing. (Source: Journal of Neurology)
Source: Journal of Neurology - April 15, 2019 Category: Neurology Source Type: research

Hereditary neuropathy with liability to pressure palsies
AbstractHereditary neuropathy with liability to pressure palsies (HNPP) is characterized by recurrent sensory and motor neuropathy in individual nerves starting in adolescence or young adulthood, focal conduction abnormalities at entrapment sites on nerve conduction studies, and sausage-like swellings (tomacula) of the myelin sheaths by nerve biopsy. It is characterized genetically by the deletion of the chromosome 17p11.2 –p12 region including the peripheral myelin protein-22 gene in the overwhelming majority of cases. HNPP may be frequently underdiagnosed or misdiagnosed owing to the heterogeneity of clinical and e...
Source: Journal of Neurology - April 15, 2019 Category: Neurology Source Type: research

Prevalence and risk factors of stroke in the elderly in Northern China: data from the National Stroke Screening Survey
AbstractBackgroundThe overall global burden of stroke is considerable and increasing. In China, stroke is the leading cause of death and disability.MethodsFor this study, we used data from the National Stroke Screening Survey in 2012 and the 2010 Chinese population from sixth National Census of Populations to calculate a standardized (by age, gender, and education) stroke prevalence. Prevalence, risk factors, and management of stroke were compared by gender, age, and site.FindingsThe standardized prevalence rate of survival stroke patients in study population aged 60 and older was 4.94% in total. Hypertension was the most ...
Source: Journal of Neurology - April 15, 2019 Category: Neurology Source Type: research

Vernon B. Mountcastle (1918 –2015)
(Source: Journal of Neurology)
Source: Journal of Neurology - April 15, 2019 Category: Neurology Source Type: research

Occurrence of cerebral small vessel disease at diagnosis of MPO-ANCA-associated vasculitis
ConclusionInflammatory events are associated with the occurrence of cerebral SVD before clinical diagnosis of MPO-ANCA-positive AAV. The patients may be continuously exposed to the risk of cerebral SVD after immunosuppressive therapy. (Source: Journal of Neurology)
Source: Journal of Neurology - April 13, 2019 Category: Neurology Source Type: research

Retraction Note to: Juvenile-onset myasthenia gravis: autoantibody status, clinical characteristics and genetic polymorphisms
The Joint Editors-in-Chief have retracted this article [1] at the request of the University of Bergen and the Norwegian Board of Health Supervision. (Source: Journal of Neurology)
Source: Journal of Neurology - April 11, 2019 Category: Neurology Source Type: research

Balanced sex distribution in patients with Meni ère’s disease
(Source: Journal of Neurology)
Source: Journal of Neurology - April 10, 2019 Category: Neurology Source Type: research

Musical hallucinations and their relation with epilepsy
AbstractMusical hallucinations are poorly understood phenomena. Their relation with epilepsy was first described over a century ago, but never systematically explored. We, therefore, reviewed the literature, and assessed all descriptions of musical hallucinations attributed to epileptic activity. Our search yielded 191 articles, which together describe 983 unique patients, with 24 detailed descriptions of musical hallucinations related to epilepsy. We also describe six of our own patients. Based on the phenomenological descriptions and neurophysiological data, we distinguish four subgroups of epilepsy-related musical hallu...
Source: Journal of Neurology - April 10, 2019 Category: Neurology Source Type: research

Association between sleep disturbance in Alzheimer ’s disease patients and burden on and health status of their caregivers
ConclusionThis study demonstrated that sleep disturbance in AD patients was associated with an increased burden and poorer health status of caregivers. Our findings highlight the importance of sleep management in AD patients. (Source: Journal of Neurology)
Source: Journal of Neurology - April 9, 2019 Category: Neurology Source Type: research

Risk of traffic accidents after onset of vestibular disease assessed with a surrogate marker
ConclusionsAlthough these data were not originally collected to address the research question, they provide a valid body of evidence. There is no rationale for driving restrictions, which substantially interfere with the individuals ’ quality of life, in patients with incident MD and VN. (Source: Journal of Neurology)
Source: Journal of Neurology - April 9, 2019 Category: Neurology Source Type: research

Correction to: A cluster of disseminated small cortical lesions in MELAS: its distinctive clinical and neuroimaging features
The author would like to correct the errors in the publication of the original article. The corrected details are given below for your reading. (Source: Journal of Neurology)
Source: Journal of Neurology - April 9, 2019 Category: Neurology Source Type: research