The integration of multisensory motion stimuli is impaired in vestibular migraine patients
ConclusionsThe comparison of the fHIT results without and with optokinetic stimulation unveils a functional vestibular impairment in VM that is not as large as the one detectable in VN, and that, in contrast with all the other patient groups, mainly impairs the capability to integrate different vestibular stimuli. (Source: Journal of Neurology)
Source: Journal of Neurology - May 24, 2020 Category: Neurology Source Type: research

Association between Helicobacter Pylori infection and stroke: a meta-analysis of 273,135 patients
ConclusionsThis study suggests that HP infection is significantly associated with increased risk of stroke. However, more well-designed studies are required to investigate if early HP eradication might decrease the incidence of stroke. (Source: Journal of Neurology)
Source: Journal of Neurology - May 23, 2020 Category: Neurology Source Type: research

Visual hallucinations and illusions in Parkinson ’s disease: the role of ocular pathology
ConclusionThese findings reinforce the hypothesis that there may be different mechanisms contributing to VH and VI in PD, suggesting that these two entities may also have a different prognosis rather than simply lying along a continuous spectrum.Registration numberClinicaltrials.gov number NCT01114321. (Source: Journal of Neurology)
Source: Journal of Neurology - May 23, 2020 Category: Neurology Source Type: research

Exergames for balance dysfunction in neurological disability: a meta-analysis with meta-regression
AbstractObjectiveTo evaluate systematically the efficacy of exergames for balance dysfunction in neurological conditions and to identify factors of exergaming protocols that may influence their effects.MethodsWe searched electronic databases for randomized clinical trials investigating the effect of commercial exergames versus alternative interventions on balance dysfunction as assessed by standard clinical scales in adults with acquired neurological disabilities. Standardized mean differences (Hedge ’sg) were calculated with random-effects models. Subgroup analyses and meta-regression were run to explore potential m...
Source: Journal of Neurology - May 23, 2020 Category: Neurology Source Type: research

SPG7 mutations in amyotrophic lateral sclerosis: a genetic link to hereditary spastic paraplegia
AbstractAmyotrophic lateral sclerosis (ALS) and hereditary spastic paraplegia (HSP) are motor neuron diseases sharing clinical, pathological, and genetic similarities. While biallelicSPG7 mutations are known to cause recessively inherited HSP, heterozygousSPG7 mutations have repeatedly been identified in HSP and recently also in ALS cases. However, the frequency and clinical impact of rareSPG7 variants have not been studied in a larger ALS cohort. Here, whole-exome (WES) or targetedSPG7 sequencing was done in a cohort of 214 European ALS patients. The consequences of a splice site variant were analyzed on the mRNA level. T...
Source: Journal of Neurology - May 23, 2020 Category: Neurology Source Type: research

Eliazar de Wind (1916 –1987)
(Source: Journal of Neurology)
Source: Journal of Neurology - May 23, 2020 Category: Neurology Source Type: research

Neurofilaments in progressive multiple sclerosis: a systematic review
ConclusionsNeurofilament light has shown consistent utility as a biomarker of neuroinflammation, future brain atrophy and immunosuppressive treatment response at agroup level. Neither neurofilament light or heavy has shown a consistent treatment response to neuroprotective disease-modifying therapies, which will require further data from successful randomised controlled trials. (Source: Journal of Neurology)
Source: Journal of Neurology - May 23, 2020 Category: Neurology Source Type: research

Riluzole prescribing, uptake and treatment discontinuation in people with amyotrophic lateral sclerosis in Scotland
(Source: Journal of Neurology)
Source: Journal of Neurology - May 23, 2020 Category: Neurology Source Type: research

Early immunotherapy is highly effective in IgG1/IgG4 positive IgLON5 disease
(Source: Journal of Neurology)
Source: Journal of Neurology - May 22, 2020 Category: Neurology Source Type: research

A novel frameshift deletion in autosomal recessive SBF1 -related syndromic neuropathy with necklace fibres
ConclusionA novel homozygous frameshift deletion inSBF1 was identified in this family. Sensory-motor axonal neuropathy and necklace fibres in biopsy were the major features expanding the phenotypic spectrum ofSBF1-related recessive syndromic neuropathy. (Source: Journal of Neurology)
Source: Journal of Neurology - May 22, 2020 Category: Neurology Source Type: research

A review of seizures and epilepsy following traumatic brain injury
We describe the epidemiology of early and late seizures following TBI, the significance of intracranial haemorrhage of different types in the risk of later epilepsy and the gaps in current understanding of risk factors contributing to the risk of post-traumatic epilepsy (PTE). The delay from injury to epilepsy presents an opportunity to understand the mechanisms underlying changes in the brain and how they may reveal potential targets for anti-epileptogenic therapy. We review existing treatments, both medical and surgical and conclude that current research is not tailored to differentiate between PTE and other forms of foc...
Source: Journal of Neurology - May 22, 2020 Category: Neurology Source Type: research

Acrophobia and visual height intolerance: advances in epidemiology and mechanisms
AbstractHistorical descriptions of fear at heights date back to Chinese and Roman antiquity. Current definitions distinguish between three different states of responses to height exposure: a physiological height imbalance that results from an impaired visual control of balance, a more or less distressing visual height intolerance, and acrophobia at the severest end of the spectrum. Epidemiological studies revealed a lifetime prevalence of visual height intolerance including acrophobia in 28% of adults (32% in women; 25% in men) and 34% among prepubertal children aged 8 –10 years without gender preponderance. Vis...
Source: Journal of Neurology - May 22, 2020 Category: Neurology Source Type: research

A systematic review and meta-analyses of pregnancy and fetal outcomes in women with multiple sclerosis: a contribution from the IMI2 ConcePTION project
In conclusion, interferons, glatiramer acetate or natalizumab, do not appear to increase the risk for spontaneous abortions, pre-term birth or major congenital malformations. There were very few patients incl uded that were exposed to fingolimod, azathioprine and rituximab; therefore, these results cannot be generalized across drugs. Future studies including internal comparators are needed to enable treating physicians and their patients to decide on the best treatment options. (Source: Journal of Neurology)
Source: Journal of Neurology - May 22, 2020 Category: Neurology Source Type: research

Sonography of optic nerve sheath diameter identifies patients with middle cerebral artery infarction at risk of a malignant course: a pilot prospective observational study
AbstractIntroductionTo assess the value of optic nerve sheath diameter (ONSD) measurements at different time points to predict the malignant evolution in middle cerebral artery (MCA) infarction and to investigate the relationship between ONSD and infarct volume on follow-up computed tomography (CT).MethodsIn a single-center prospective observational study, we recruited patients with MCA infarction and age- and sex-matched controls. Clinical characteristics including NationaI Institutes of Health Stroke Scale (NIHSS) and ONSD measurement were assessed during the first five days after symptom onset. Volumetric analysis of th...
Source: Journal of Neurology - May 22, 2020 Category: Neurology Source Type: research

Cognitive functions in acute unilateral vestibular loss
AbstractCognitive deficits mainly involving visuospatial functions have been defined in patients with bilateral and even unilateral vestibular loss (UVL). We compared the cognitive test results of 21 patients with acute UVL with age- and education-matched healthy controls. The diagnosis of UVL was based on the clinical findings, a normal magnetic resonance imaging with diffusion-weighted sequence and canal paresis on the affected side on caloric testing. Cognitive tests assessing visuospatial functions (Benton ’s Judgment of Line Orientation test, Verbal and non-verbal Cancellation tests, Rey–Osterrieth Complex...
Source: Journal of Neurology - May 21, 2020 Category: Neurology Source Type: research

Pick ’s disease: clinicopathologic characterization of 21 cases
ConclusionsOur study shows that bvFTD and PPA are the most common clinical phenotypes associated with PiD, although rare presentations such as CBS were also seen. Coexisting non-Pick ’s pathology was also present in many cases. Our study highlights the clinical and pathologic heterogeneity in PiD. (Source: Journal of Neurology)
Source: Journal of Neurology - May 21, 2020 Category: Neurology Source Type: research

Dopaminergic function in spinocerebellar ataxia type 6 patients with and without parkinsonism
ConclusionsThe pathology of SCA6 may not be restricted to the cerebellum, but may also be distributed across various regions, including in both presynaptic and postsynaptic dopaminergic neurons to some degree. Patients with SCA6 may show apparent parkinsonism after the progression of neurodegeneration. (Source: Journal of Neurology)
Source: Journal of Neurology - May 21, 2020 Category: Neurology Source Type: research

An update on MSA: premotor and non-motor features open a window of opportunities for early diagnosis and intervention
We describe the progression of clinical features to aid the diagnosis of MSA early in the disease course. We go on to describe the levels of diagnostic certainty and we discuss MSA subtypes that do not fit into the current diagnostic criteria, highlighting the complexity of the disease as well as the need for revised diagnostic tools. Second, we describe the pathology, clinical description, and investigations of cardiovascular autonomic failure, urogenital and sexual dysfunction, orthostatic hypotension, and respiratory and REM-sleep behavior disorders, which may precede the motor presentation by months or years. Their pre...
Source: Journal of Neurology - May 20, 2020 Category: Neurology Source Type: research

When does postural instability appear in monogenic parkinsonisms? An individual-patient meta-analysis
ConclusionsMonogenic parkinsonisms exhibit differential timelines to postural instability, informing prognostic counseling and interpretation of future genotype-specific treatment trials. (Source: Journal of Neurology)
Source: Journal of Neurology - May 20, 2020 Category: Neurology Source Type: research

Expansion of the dimensions in the current management of acute ischemic stroke
AbstractStroke is the fifth leading cause of death in the United States with a huge burden on health care. Acute ischemic stroke (AIS) accounts for 87% of all stroke. The use of thrombolytic agents in AIS treatment is well known since 1950 but no FDA approval until 1996, due to lack of strong evidence showing benefits outweigh the risk of intracranial hemorrhage. The NINDS trial led to the approval of intravenous tissue plasminogen activator treatment (IV recombinant tPA) within 3  h of stroke. Due to this limitation of 3–4.5 h. window, evolution began in the development of effective endovascular therapy (E...
Source: Journal of Neurology - May 20, 2020 Category: Neurology Source Type: research

Stroke in patients with SARS-CoV-2 infection: case series
ConclusionsBoth ischemic and hemorrhagic stroke can complicate the course of COVI-19 infection. In our series, stroke developed mostly in patients with severe pneumonia and multiorgan failure, liver enzymes and LDH were markedly increased in all cases, and the outcome was poor. (Source: Journal of Neurology)
Source: Journal of Neurology - May 20, 2020 Category: Neurology Source Type: research

Neurological manifestations associated with COVID-19: a review and a call for action
AbstractWhile the epidemic of Coronavirus disease 2019 (COVID-19) continues to spread globally, more and more evidences are collected about the presence of neurological manifestations and symptoms associated with it. A systematic review has been performed of papers published until 5 April 2020. 29 papers related to neurological manifestations associated with COVID-19 were examined. The results show presence of central and peripheral nervous system manifestations related to coronavirus. Neurological manifestations, or NeuroCOVID, are part of the COVID-19 clinical picture, but questions remain regarding the frequency and sev...
Source: Journal of Neurology - May 20, 2020 Category: Neurology Source Type: research

Correction to: Optical coherence tomography shows neuroretinal thinning in myelopathy of adrenoleukodystrophy
The original version of this article unfortunately contained a mistake (Source: Journal of Neurology)
Source: Journal of Neurology - May 20, 2020 Category: Neurology Source Type: research

Correction to: Caregiver burden and its related factors in advanced Parkinson ’s disease: data from the PREDICT study
The original version of this article unfortunately contained a mistake. In the Figure (Source: Journal of Neurology)
Source: Journal of Neurology - May 20, 2020 Category: Neurology Source Type: research

Impact of environmental factors and physical activity on disability and quality of life in CIDP
This study adds evidence to the possible role of physical activity in improving symptom severity, disability and QoL in patients with CIDP. None of the other environmental factors investigated appeared to have an impact on the severity and health perception of CIDP. (Source: Journal of Neurology)
Source: Journal of Neurology - May 19, 2020 Category: Neurology Source Type: research

Guillain-Barr é syndrome in the COVID-19 era: just an occasional cluster?
(Source: Journal of Neurology)
Source: Journal of Neurology - May 19, 2020 Category: Neurology Source Type: research

A review of mobile stroke units
AbstractPurpose of reviewMobile stroke units (MSUs) for prehospital treatment and management of patients with acute stroke have been developed more than a decade ago and is currently spreading worldwide. This review discusses the history of MSU and current operations and research.Recent findingsMultiple studies have shown that MSU can significantly reduce treatment time with a tenfold increase of patients treated within the first 60  min of symptom onset. Recent preliminary results from the Berlin Prehospital or Usual Delivery of Acute Stroke Care trial (B-PROUD) showed a positive shift in modified Rankin Scale (mRS) ...
Source: Journal of Neurology - May 18, 2020 Category: Neurology Source Type: research

Do patients with large vessel occlusion ischemic stroke harboring prestroke disability benefit from thrombectomy?
AbstractObjectivesEvidence of endovascular treatment (EVT) for acute large vessel occlusion (LVO) ischemic stroke in patients harboring substantial prestroke disability is lacking due to their exclusion from randomized trials. Here, we used routine care observational data to compare outcomes in patients with and without prestroke disability receiving EVT for LVO ischemic stroke.MethodsConsecutive patients undergoing EVT for acute LVO ischemic stroke at the Sahlgrenska University Hospital from January 1st, 2015 to March 31st, 2018 were registered in the Sahlgrenska Stroke Recanalization Registry. Pre- and poststroke functio...
Source: Journal of Neurology - May 14, 2020 Category: Neurology Source Type: research

Outcomes of a 5-week individualised MDT outpatient (day-patient) treatment programme for functional neurological symptom disorder (FNSD)
ConclusionAn MDT can effectively deliver an outpatient programme for FNSD which can serve as an alternative to costlier inpatient programmes. Early  identification and treatment of co-morbidities is advised. (Source: Journal of Neurology)
Source: Journal of Neurology - May 14, 2020 Category: Neurology Source Type: research

Prevalence and risk factors of silent brain infarcts in patients with AF detected by 3T-MRI
ConclusionsLA enlargement on echocardiogram was associated with SBI in patients with AF and low or moderate embolic risk according to CHADS2 and in the low-risk population according to CHA2DS2VASc. Although further studies are needed, a neuroimaging screening might be justified in these patients to guide medical therapies to improve stroke prevention. (Source: Journal of Neurology)
Source: Journal of Neurology - May 14, 2020 Category: Neurology Source Type: research

The contribution of enhancing lesions in monitoring multiple sclerosis treatment: is gadolinium always necessary?
ConclusionsReactivation of pre-existing lesions is limited to a tiny fraction of MRI studies. Gd  + T1-weighted images could be omitted, in patients treated with DMT for at least 6 months, without relevant loss of information. (Source: Journal of Neurology)
Source: Journal of Neurology - May 12, 2020 Category: Neurology Source Type: research

Screening for genetic mutations in patients with neuropathy without definite etiology is useful
ConclusionScreening for genetic mutations in patients with neuropathy without a definite etiology is useful. While only a minority of patients with unsuspected inherited neuropathy tested positive, the findings altered management in some, improving morbidity and, perhaps, mortality. (Source: Journal of Neurology)
Source: Journal of Neurology - May 12, 2020 Category: Neurology Source Type: research

Tele-health in neurology: an indispensable tool in the management of the SARS-CoV-2 epidemic
(Source: Journal of Neurology)
Source: Journal of Neurology - May 12, 2020 Category: Neurology Source Type: research

Correction to: Routine diagnostics for neural antibodies, clinical correlates, treatment and functional outcome
The original version of this article unfortunately contained a mistake. (Source: Journal of Neurology)
Source: Journal of Neurology - May 12, 2020 Category: Neurology Source Type: research

Acute polyradiculoneuritis with locked-in syndrome in a patient with Covid-19
(Source: Journal of Neurology)
Source: Journal of Neurology - May 12, 2020 Category: Neurology Source Type: research

Posterior circulation stroke: machine learning-based detection of early ischemic changes in acute non-contrast CT scans
AbstractObjectivesTriage of patients with basilar artery occlusion for additional imaging diagnostics, therapy planning, and initial outcome prediction requires assessment of early ischemic changes in early hyperacute non-contrast computed tomography (NCCT) scans. However, accuracy of visual evaluation is impaired by inter- and intra-reader variability, artifacts in the posterior fossa and limited sensitivity for subtle density shifts. We propose a machine learning approach for detecting early ischemic changes in pc-ASPECTS regions (Posterior circulation Alberta Stroke Program Early CT Score) based on admission NCCTs.Metho...
Source: Journal of Neurology - May 11, 2020 Category: Neurology Source Type: research

Asymmetry index of Blink Reflex Recovery Cycle differentiates Parkinson ’s disease from atypical Parkinsonian syndromes
AbstractBackgroundDifferential diagnosis between Parkinson ’s disease (PD) and atypical Parkinsonian syndromes (APS), such as multiple system atrophy (MSA) and progressive supranuclear palsy (PSP), is often difficult because of overlap of common clinical features. We evaluated R2 Blink Reflex Recovery Cycle (R2BRRC) in drug-naive PD patients and in MSA an d PSP patients to differentiate early PD from APS.MethodsWe investigated 43 patients: 15 drug-naive PD patients, 16 MSA patients, and 12 PSP patients. R2BRRC was evaluated bilaterally at interstimulus intervals (ISIs) of 100, 150, 200, 300, 400, 500, and 750  m...
Source: Journal of Neurology - May 11, 2020 Category: Neurology Source Type: research

Bilateral vestibulopathy as an early manifestation of systemic lupus erythematosus
(Source: Journal of Neurology)
Source: Journal of Neurology - May 11, 2020 Category: Neurology Source Type: research

Cerebellar degeneration in adult spinal muscular atrophy patients
ConclusionsNeuroimaging detects cerebellar structural changes in adult SMA patients, suggesting that neurodegeneration is not confined to the lower motor neurons in the disease. (Source: Journal of Neurology)
Source: Journal of Neurology - May 10, 2020 Category: Neurology Source Type: research

Ioflupane 123I (DAT scan) SPECT identifies dopamine receptor dysfunction early in the disease course in progressive apraxia of speech
ConclusionsAbnormal DAT scan is observed early in the disease course in approximately 30% of PAOS patients, with striatal abnormalities similar to those in PSP and CBS. (Source: Journal of Neurology)
Source: Journal of Neurology - May 9, 2020 Category: Neurology Source Type: research

Gadolinium enhancement on cranial MRI in multiple sclerosis is age dependent
ConclusionsOur investigation shows that gadolinium enhancement is strongly associated with age. Since gadolinium enhancement is a marker of inflammatory disease activity, our findings suggest that inflammatory disease activity declines with age, and that IMD treatment may be more beneficial in younger and less useful in older people with MS. (Source: Journal of Neurology)
Source: Journal of Neurology - May 9, 2020 Category: Neurology Source Type: research

Epilepsy in children with leukodystrophies
AbstractBackgroundEpilepsy might be one of the manifestations in children with leukodystrophies, but the incidence of epilepsy in different types of leukodystrophies is unclear yet.MethodsA retrospective observational cohort study was performed on children diagnosed with leukodystrophies in Peking University First Hospital from January 2004 to June 2019, and the patients were followed for 5.5  years (0.4–14.2 years) after the first visit.ResultsA total of 333 patients were included. The overall incidence of epilepsy was 30.6% (102/333). Alexander disease had the highest incidence (77.3%, 51/66), followed by...
Source: Journal of Neurology - May 9, 2020 Category: Neurology Source Type: research

Neurofilament light chain as a potential biomarker of disease status in Friedreich ataxia
AbstractBackgroundThe present study evaluates serum neurofilament light chain (NfL) as a biomarker of disease features in Friedreich ’s ataxia (FRDA).MethodsNfL levels from serum of 117 subjects (85 FRDA patients, 13 carriers, and 19 controls) were assayed and correlated with disease features such as smaller GAA repeat length (GAA1), age, sex, and level of neurological dysfunction.ResultsMean serum NfL levels were higher in FRDA patients than in carriers or unaffected controls in two independent cohorts of subjects. In longitudinal samples from FRDA patients drawn monthly or 1  year apart, values changed minimal...
Source: Journal of Neurology - May 8, 2020 Category: Neurology Source Type: research

Clemente Estable (1894 –1976)
(Source: Journal of Neurology)
Source: Journal of Neurology - May 8, 2020 Category: Neurology Source Type: research

Application of “Mentzer’s PML case definition” to natalizumab-treated patients in the setting of strict MRI-based pharmacovigilance
ConclusionsBoth the AAN PML diagnostic criteria and the “Mentzers case definition” require the positive detection of JC virus DNA in CSF to define patients at a higher degree of suspicion of PML. However, as sensitivity of JC virus PCR in CSF is limited and often returns negative in particular in early cases of PML with a mere MRI-based PML suspicion , both criteria have obvious limitations when frequent MRI is used for pharmacovigilance purposes. Thus, revision of PML diagnostic criteria is needed, including the incorporation of lesion evolution, and longitudinal CSF studies that also assess for the presences ...
Source: Journal of Neurology - May 8, 2020 Category: Neurology Source Type: research

Advantages of timing the duration of a freezing of gait-provoking test in individuals with Parkinson ’s disease
AbstractEvaluating freezing of gait (FOG) and quantifying its severity in patients with Parkinson ’s disease (PD) is challenging; objective assessment is not sufficiently established. We aimed to improve the ability to objectively evaluate FOG severity by investigating the value of measuring the duration of the test and its components. Seventy-one patients with PD and FOG completed a previousl y validated FOG-provoking test. The test was performed under three conditions: (1) usual, single task; (2) dual task (walking while carrying a tray); and (3) triple task (walking while holding a tray and subtracting 7 s). ...
Source: Journal of Neurology - May 7, 2020 Category: Neurology Source Type: research

Overlapping syndrome of MOG-IgG-associated disease and autoimmune GFAP astrocytopathy
AbstractAntibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been considered to be closely relevant to an inflammatory demyelinating disease of the central nervous system (CNS). Glial fibrillary acidic protein (GFAP) immunoglobulin G (IgG) has been identified as a biomarker for a novel autoimmune astrocytopathy. However, coexistence of MOG-IgG and GFAP-IgG is extremely unusual and only one patient has been described with simultaneous presence of MOG-IgG in serum and GFAP-IgG in cerebrospinal fluid (CSF). Herein, we reported the first case of overlapping syndrome of MOG-IgG-associated disease (MOG-AD) and a...
Source: Journal of Neurology - May 7, 2020 Category: Neurology Source Type: research

Progression of grey and white matter brain damage in Parkinson's disease: a critical review of structural MRI literature
AbstractThe current review summarizes the current knowledge on longitudinal cortical and subcortical grey and white matter MRI findings assessed using T1-weighted and one-tensor diffusion-weighted MRI in Parkinson ’s disease (PD) patients. Results were reviewed according to disease duration, disease severity and cognitive impairment. The most consistent findings are those showing a progressive cortical atrophy accumulation in caudate, putamen, temporal/hippocampal, frontal and parietal areas in de novo PD c ases and in the early/middle phase of the disease, with the achievement of a plateau in the later stage. Analyz...
Source: Journal of Neurology - May 6, 2020 Category: Neurology Source Type: research

Correction to: Endovascular stroke treatment ’s impact on malignant type of edema (ESTIMATE)
The original keywords were not sufficiently precise in describing the article content. Therefore, the key words were amended to include hemicraniectomy and malignant edema. (Source: Journal of Neurology)
Source: Journal of Neurology - May 6, 2020 Category: Neurology Source Type: research

A new tetra-plex fluorimetric assay for the quantification of cerebrospinal fluid β-amyloid42, total-tau, phospho-tau and α-synuclein in the differential diagnosis of neurodegenerative dementia
ConclusionsThe new tetra-plex assay coupled with multivariate analytical approaches becomes a valuable asset for the differential diagnosis of neurodegenerative dementia and related applications. (Source: Journal of Neurology)
Source: Journal of Neurology - May 5, 2020 Category: Neurology Source Type: research