StartReact during gait initiation reveals differential control of muscle activation and inhibition in patients with corticospinal degeneration
AbstractCorticospinal lesions cause impairments in voluntary motor control. Recent findings suggest that some degree of voluntary control may be taken over by a compensatory pathway involving the reticulospinal tract. In humans, evidence for this notion mainly comes from StartReact studies. StartReact is the acceleration of reaction times by a startling acoustic stimulus (SAS) simultaneously presented with the imperative stimulus. As previous StartReact studies mainly focused on isolated single-joint movements, the question remains whether the reticulospinal tract can also be utilized for controlling whole-body movements. ...
Source: Journal of Neurology - October 12, 2018 Category: Neurology Source Type: research

Peripheral tumor necrosis factor-a-induced protein 8-like 2 mRNA level for predicting 3-month mortality of patients with acute ischemic stroke
This study aimed to investigate TIPE2 mRNA in peripheral blood mononuclear cells for predicting 3-month functional outcomes and mortality of patients with acute ischemic stroke. A total of 182 consecutive patients were prospective collected, and there were 55 (30.2%) patients with unfavorable outcome and 33 (18.1%) patients died at the end of 3  months. The area under the operating characteristic curve (AUC) for TIPE2 mRNA was 0.810 (95% CI 0.733–0.886) for mortality and 0.740 (95% CI 0.662–0.818) for unfavorable outcome. The model incorporating National Institutes of Health Stroke Scale (NIHSS) plus TIPE2...
Source: Journal of Neurology - October 12, 2018 Category: Neurology Source Type: research

Delayed treatment of MS is associated with high CSF levels of IL-6 and IL-8 and worse future disease course
ConclusionsDelayed diagnosis and treatment initiation are associated with higher CSF levels of IL-6 and IL-8 in RR-MS, leading to worsening disease course and poor response to treatments. (Source: Journal of Neurology)
Source: Journal of Neurology - October 12, 2018 Category: Neurology Source Type: research

Anti-epileptics and pregnancy: an update
(Source: Journal of Neurology)
Source: Journal of Neurology - October 12, 2018 Category: Neurology Source Type: research

Worse endovascular mechanical recanalization results for patients with in-hospital onset acute ischemic stroke
ConclusionThe recognition, assessment and pre-interventional procedures of patients with in-hospital strokes and subsequent mechanical thrombectomy are favorable. Nevertheless, in-hospital stroke patients display inferior recanalization results and poorer clinical outcomes. Furthermore, we find mechanical thrombectomy seems safe for treatment of in-hospital strokes. (Source: Journal of Neurology)
Source: Journal of Neurology - October 12, 2018 Category: Neurology Source Type: research

The Brief International Cognitive Assessment in Multiple Sclerosis (BICAMS): results from the German validation study
AbstractBackgroundRecent research has convincingly shown that the ability to work mainly depends on the cognitive status in multiple sclerosis (MS). An international committee of experts recommended a brief neuropsychological battery to evaluate cognitive performance in MS. BICAMS comprises three tests, the Symbol Digit Modalities Test (SDMT), the learning trials of the California Verbal Learning Test II (CVLT-II), and the Brief Visuospatial Memory Test-Revised (BVMT-R).ObjectiveTo validate BICAMS on a sample of German MS patients and healthy controls (HCs).MethodsAccording to the international guidelines for validation, e...
Source: Journal of Neurology - October 12, 2018 Category: Neurology Source Type: research

The role of clinical and neuroimaging features in the diagnosis of CADASIL
ConclusionsA careful familial history collection and neuroradiological assessment can identify patients in whomNOTCH3 genetic testing has a higher yield. (Source: Journal of Neurology)
Source: Journal of Neurology - October 11, 2018 Category: Neurology Source Type: research

High-sensitivity cardiac troponin T and severity of cerebral white matter lesions in patients with acute ischemic stroke
ConclusionElevated hs-cTnT levels were associated with extent of WML in acute stroke patients. Further studies are needed to assess whether hs-cTnT can be used to identify stroke patients at risk for cognitive decline. (Source: Journal of Neurology)
Source: Journal of Neurology - October 11, 2018 Category: Neurology Source Type: research

Functioning and quality of life in patients with neuropathy associated with anti-MAG antibodies
AbstractAlthough anti-myelin-associated glycoprotein (MAG) antibody neuropathy is reported as a slowly progressive disease, it can lead to significant disability and impairment of health-related quality of life (HR-QoL) and social participation. The aim of this cross-sectional study was to evaluate the functioning and HR-QoL determinants in 67 patients with anti-MAG neuropathy in terms of the International Classification of Functioning, Disability, and Health (ICF). Evaluations included: Medical Research Council (MRC) sum score, Sensory Modality Sum score (SMS), Berg balance scale (BBS), Fatigue Severity Scale (FSS), Visua...
Source: Journal of Neurology - October 10, 2018 Category: Neurology Source Type: research

The clinical features and outcome of scan-negative and scan-positive cases in suspected cauda equina syndrome: a retrospective study of 276 patients
ConclusionsThe data support a model in which scan-negative cauda equina syndrome arises as an end pathway of acute pain, sometimes with partly structural findings and vulnerability to functional disorders. (Source: Journal of Neurology)
Source: Journal of Neurology - October 8, 2018 Category: Neurology Source Type: research

Management of patients with stroke treated with direct oral anticoagulants
AbstractSince their market approval, direct oral anticoagulants (DOACs) are being increasingly used for stroke prevention in patients with atrial fibrillation. However, the management of DOAC-treated patients with stroke poses several challenges for physicians in everyday clinical practice, both in the acute setting and in long-term care. This has spurred extensive research activity in the field over the past few years, which we review here. (Source: Journal of Neurology)
Source: Journal of Neurology - October 6, 2018 Category: Neurology Source Type: research

Short-term and long-term effects of immunoadsorption in refractory chronic inflammatory demyelinating polyneuropathy: a prospective study in 17 patients
AbstractObjectiveChronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, autoimmune-inflammatory disease of the peripheral nervous system. Recently, various immunoglobulin G4 (IgG4) type auto-antibodies have been described in patients with CIDP which can effectively be removed by immunoadsorption (IA). Therefore, we prospectively evaluated the therapeutic effect of IA in 17 patients with progressive CIDP not responding to other treatment.MethodsWe prospectively evaluated the course of disease of 17 patients with CIDP who had insufficiently responded to steroids and/or IVIg previously and who received at least o...
Source: Journal of Neurology - October 6, 2018 Category: Neurology Source Type: research

Cerebral small vessel disease: neuroimaging markers and clinical implication
AbstractCerebral small vessel disease (CSVD) is a broad category of cerebrovascular diseases which primarily affect the perforating arterioles, capillaries and venules with multiple distinct etiologies. In spite of distinctive pathogenesis, CSVD shares similar neuroimaging markers, including recent small subcortical infarct, lacune of presumed vascular origin, white matter hyperintensity of presumed vascular origin, perivascular space and cerebral microbleeds. The radiological features of neuroimaging markers are indicative for etiological analysis. Furthermore, in sporadic arteriosclerotic pathogenesis associated CSVD, th...
Source: Journal of Neurology - October 5, 2018 Category: Neurology Source Type: research

Comparison of outcome in stroke patients admitted during working hours vs. off-hours; a single-center cohort study
AbstractIntroductionWe aimed to disprove an in-hospital off-hour effect in stroke patients by adjusting for disease severity and poor prognostic findings on imaging.Patients and methodsOur study included 5378 patients from a single center prospective stroke registry of a large teaching hospital in the Netherlands, admitted between January 2003 and June 2015. Patients were categorized by admission time, off-hours (OH) or working hours (WH). The in-hospital mortality, 7-day mortality, unfavorable functional outcome (modified Rankin scale  >  2) and discharge to home were analyzed. Results were adjusted for a...
Source: Journal of Neurology - October 5, 2018 Category: Neurology Source Type: research

James Crichton-Browne (1840 –1938)
(Source: Journal of Neurology)
Source: Journal of Neurology - October 4, 2018 Category: Neurology Source Type: research

Vitamin D for the treatment of multiple sclerosis: a meta-analysis
ConclusionThese findings suggest that vitamin D supplementation may have a therapeutic role in the treatment of MS. However, there is uncertainty with regard to the most appropriate dose, with high doses potentially being associated with worse outcomes. There remains the need for further well-performed randomised, dose-ranging, placebo-controlled trials of vitamin D in MS. (Source: Journal of Neurology)
Source: Journal of Neurology - October 3, 2018 Category: Neurology Source Type: research

Spinocerebellar ataxia: an update
AbstractSpinocerebellar ataxia (SCA) is a heterogeneous group of neurodegenerative ataxic disorders with autosomal dominant inheritance. We aim to provide an update on the recent clinical and scientific progresses in SCA where numerous novel genes have been identified with next-generation sequencing techniques. The main disease mechanisms of these SCAs include toxic RNA gain-of-function, mitochondrial dysfunction, channelopathies, autophagy and transcription dysregulation. Recent studies have also demonstrated the importance of DNA repair pathways in modifying SCA with CAG expansions. In addition, we summarise the latest t...
Source: Journal of Neurology - October 3, 2018 Category: Neurology Source Type: research

Vitamin D for the treatment of multiple sclerosis: a meta-analysis
ConclusionThese findings suggest that vitamin D supplementation may have a therapeutic role in the treatment of MS. However, there is uncertainty with regard to the most appropriate dose, with high doses potentially being associated with worse outcomes. There remains the need for further well-performed randomised, dose-ranging, placebo-controlled trials of vitamin D in MS. (Source: Journal of Neurology)
Source: Journal of Neurology - October 3, 2018 Category: Neurology Source Type: research

Spinocerebellar ataxia: an update
AbstractSpinocerebellar ataxia (SCA) is a heterogeneous group of neurodegenerative ataxic disorders with autosomal dominant inheritance. We aim to provide an update on the recent clinical and scientific progresses in SCA where numerous novel genes have been identified with next-generation sequencing techniques. The main disease mechanisms of these SCAs include toxic RNA gain-of-function, mitochondrial dysfunction, channelopathies, autophagy and transcription dysregulation. Recent studies have also demonstrated the importance of DNA repair pathways in modifying SCA with CAG expansions. In addition, we summarise the latest t...
Source: Journal of Neurology - October 3, 2018 Category: Neurology Source Type: research

Cerebellum and cognition in progressive MS patients: functional changes beyond atrophy?
AbstractBackgroundThe cerebellum is a predilection site of pathology in progressive multiple sclerosis (PMS) patients, contributing to cognitive deficits. Aim of this study was to investigate lobular cerebellar functional connectivity (FC) in PMS patients in relation to cognition.MethodsIn this cross-sectional study, resting state fMRI analysis was carried out on 29 PMS patients (11 males, mean age 51.2  ± 11.9 years) and 22 age- and sex-matched healthy controls (HC) (11 males, mean age 49.6 ± 8.8 years). Data were analyzed with a seed-based approach, with four different ...
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Occult paroxysmal atrial fibrillation in non-cryptogenic ischemic stroke
ConclusionLong-term monitoring of non-CIS patients detected PAF in a clinically relevant proportion of patients, resulting in stroke prevention therapy optimization. Further study to confirm these findings and refine the subset that would benefit from long-term cardiac monitoring is warranted. (Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Efficacy of STA –MCA bypass surgery in moyamoya angiopathy: long-term follow-up of the Caucasian Krupp Hospital cohort with 81 procedures
ConclusionDirect STA –MCA bypass is an effective therapy in Caucasian patients with hemodynamically compromised MA. (Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Correction to: The occurrence of seizures after ischemic stroke does not influence long-term mortality; a 26-year follow-up study
The original article can be found online at (Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Subacute encephalopathy with seizures in alcoholics (SESA) syndrome: further evidence supporting that may lie on the ictal-interictal continuum
(Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Multicenter data banking in management of dizzy patients: first results from the DizzyNet registry project
ConclusionsSharing data facilitates research, enhances translation from basic science into clinical applications, and increases transparency. The DizzyNet registry is a first step to data sharing in vestibular research across Europe. (Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Neuropsychiatric symptoms and α-Synuclein profile of patients with Parkinson’s disease dementia, dementia with Lewy bodies and Alzheimer’s disease
This study aims to c ompare the neuropsychiatric and CSF α-Syn profiles in these conditions, and to investigate the possible association between CSF α-Syn levels and neuropsychiatric symptoms.MethodsWe conducted a prospective cross-sectional study, between January 2013 and January 2015, with 16 PDD, 28 DLB and 19 AD patients. All participants underwent a detailed clinical, neuropsychological, neuropsychiatric [Neuropsychiatric Inventory (NPI)] and CSF α-Syn analysis.ResultsSignificantly greater NPI Hallucinations Subitem score was found in the PDD and DLB groups compared to AD (bothp 
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

DIZZYNET 2018: visions and perspectives of future vestibular research
(Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Dizziness in Europe: from licensed fitness to drive to licence without fitness to drive
This article discusses various assessment guidelines for dizzy patients. These range from a total absence of specified binding requirements in Finland or regulations open to clinical interpretation in Switzerland, to inappropriately strict regulations in Germany. We focus on requirements for patients with vestibular disorders in Germany which have been in force since 2014. These guidelines stipulate that for group 1 driving licence (private cars  
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Cerebellum and cognition in progressive MS patients: functional changes beyond atrophy?
AbstractBackgroundThe cerebellum is a predilection site of pathology in progressive multiple sclerosis (PMS) patients, contributing to cognitive deficits. Aim of this study was to investigate lobular cerebellar functional connectivity (FC) in PMS patients in relation to cognition.MethodsIn this cross-sectional study, resting state fMRI analysis was carried out on 29 PMS patients (11 males, mean age 51.2  ± 11.9 years) and 22 age- and sex-matched healthy controls (HC) (11 males, mean age 49.6 ± 8.8 years). Data were analyzed with a seed-based approach, with four different ...
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Scribonius Largus (probably before 1CE –after 48CE)
(Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Moyamoya angiopathy: early postoperative course within 3 months after STA –MCA–bypass surgery in Europe—a retrospective analysis of 64 procedures
ConclusionRevasculating surgery is a safe procedure in Caucasian patients with Moyamoya angiopathy. The observed complications have a good prognosis. (Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Genetic contribution to vestibular diseases
AbstractGrowing evidence supports the contribution of allelic variation to vestibular disorders. Heritability attributed to rare allelic variants is found in familial vestibular syndromes such as enlarged vestibular aqueduct syndrome or familial Meniere disease. However, the involvement of common allelic variants as key regulators of physiological processes in common and rare vestibular diseases is starting to be deciphered, including motion sickness or sporadic Meniere disease. The genetic contribution to most of the vestibular disorders is still largely unknown. This review will outline the role of common and rare varian...
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Gluten neuropathy: prevalence of neuropathic pain and the role of gluten-free diet
AbstractBackground and aimPeripheral neuropathy is a common extraintestinal manifestation of gluten sensitivity (gluten neuropathy). We aimed to establish the prevalence of neuropathic pain in patients with otherwise idiopathic PN and gluten sensitivity (positive antigliadin, endomysial, and/or transglutaminase antibodies, with or without enteropathy) and to describe any contributory factors.MethodsAll consecutive patients with gluten neuropathy (GN) attending a specialist gluten/neurology clinic were invited to participate. Pain was assessed via the DN4 questionnaire and the visual analog scale. Overall Neuropathy Limitat...
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Diagnostic and prognostic power of CSF Tau in amyotrophic lateral sclerosis
This study aims to evaluate the diagnostic and prognostic potential of CSF total Tau (t-Tau), phospho-Tau (p-Tau) and p-Tau/t-Tau ratio in ALS patients using CSF neurofilament light (NFL) as the reference biomarker.MethodsEighty-five incident ALS, 30 ALS-mimicking (AM) diseases and 51 other non-neurodegenerative diseases (ONND) were included in the study.ResultsALS patients had higher levels of CSF t-Tau and lower p-Tau/t-Tau ratio than AM (p = 0.005 andp = 0.006) and ONND (p 
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Vestibular rehabilitation therapy and Nintendo Wii balance board training both improve postural control in bilateral vestibulopathy
(Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Occult paroxysmal atrial fibrillation in non-cryptogenic ischemic stroke
ConclusionLong-term monitoring of non-CIS patients detected PAF in a clinically relevant proportion of patients, resulting in stroke prevention therapy optimization. Further study to confirm these findings and refine the subset that would benefit from long-term cardiac monitoring is warranted. (Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Efficacy of STA –MCA bypass surgery in moyamoya angiopathy: long-term follow-up of the Caucasian Krupp Hospital cohort with 81 procedures
ConclusionDirect STA –MCA bypass is an effective therapy in Caucasian patients with hemodynamically compromised MA. (Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Correction to: Carotid endarterectomy in patients with recurrent symptoms associated with an ipsilateral carotid artery near occlusion with full collapse
The original version of this article unfortunately contained a mistake. (Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Correction to: Myelin oligodendrocyte glycoprotein antibody-associated disease: characterising clinical disease
The original version of this article unfortunately contained a mistake. (Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Correction to: The occurrence of seizures after ischemic stroke does not influence long-term mortality; a 26-year follow-up study
The original article can be found online at (Source: Journal of Neurology)
Source: Journal of Neurology - October 1, 2018 Category: Neurology Source Type: research

Identifying barriers to help-seeking for sexual dysfunction in multiple sclerosis
ConclusionsBarriers to discussing SD are similar between patients and HCPs. The most common barriers are addressable through modifications in the clinic environment, raising awareness and providing training opportunities. (Source: Journal of Neurology)
Source: Journal of Neurology - September 19, 2018 Category: Neurology Source Type: research

36-Months follow-up assessment after cessation and resuming of enzyme replacement therapy in late onset Pompe disease: data from the Swiss Pompe Registry
ConclusionsOur data suggests that long-term interruption of ERT in LOPD may lead to deterioration of clinical meaningful parameters and quality of life. In addition, a clinical restoration after ERT cessation is possible for most of the LOPD patients within a 36 months follow-up. (Source: Journal of Neurology)
Source: Journal of Neurology - September 19, 2018 Category: Neurology Source Type: research

Migraine prophylactic management in neurology and primary care (2006 –2015)
(Source: Journal of Neurology)
Source: Journal of Neurology - September 19, 2018 Category: Neurology Source Type: research

Generalization after ocular onset in myasthenia gravis: a case series in Germany
ConclusionsThe conversion rate in ocular myasthenia was high in our series, predicted by the presence of a thymoma. Our findings suggest that corticosteroids can prevent secondary generalization in ocular myasthenia patients with thymic hyperplasia, which requires further research. (Source: Journal of Neurology)
Source: Journal of Neurology - September 17, 2018 Category: Neurology Source Type: research

Ethical challenges in tracheostomy-assisted ventilation in amyotrophic lateral sclerosis
AbstractThe special nature of amyotrophic lateral sclerosis (ALS) and tracheostomy with invasive ventilation (TIV) leads to challenges that can be difficult in two senses: not only to handle well, but also to discuss with patients and other involved stakeholders. Because of the delicate nature of interpersonal relations and communication in ALS, some of the downsides to TIV may almost take on a nature of taboo, making them difficult to raise for open discussion. Yet these ethical challenges are important to be aware of, not only for health professionals and managers but, arguably, also for patients and next of kin. They ar...
Source: Journal of Neurology - September 14, 2018 Category: Neurology Source Type: research

Ciclosporin and chorea: a new association?
(Source: Journal of Neurology)
Source: Journal of Neurology - September 14, 2018 Category: Neurology Source Type: research

Differential effects of propranolol on head and upper limb tremor in patients with essential tremor and dystonia
AbstractPropranolol is used as the first-line treatment in essential tremor and it has also been proposed as a treatment for tremor in dystonia. However, several issues remain uncertain. For example, it is still not clear whether propranolol exerts a beneficial effect on head tremor. Moreover, no studies have investigated whether the effect of propranolol on head and upper limb tremor in essential tremor differs from that in dystonia. We aimed to assess the effects of propranolol on tremor in different body parts in essential tremor and in patients with tremor and dystonia. Twenty-nine patients with head and upper limb tre...
Source: Journal of Neurology - September 12, 2018 Category: Neurology Source Type: research

Early ophthalmologic features of Parkinson ’s disease: a review of preceding clinical and diagnostic markers
AbstractNon-motor symptoms in Parkinson ’s disease are an important cause of morbidity and may even precede the onset of the motor features of the disease. Visual abnormalities are among the most frequent non-motor symptoms observed during the early stages of the disease. Some of the visual symptoms of Parkinson’s disease can likely b e explained by the presence of dopaminergic neurons within the retina, where the progressive loss of dopamine and the accumulation of α-synuclein within the retinal layers leads to visual dysfunction, while some are caused by abnormalities in cortical visual processing. Many...
Source: Journal of Neurology - September 12, 2018 Category: Neurology Source Type: research

Quality of life in a German cohort of Parkinson ’s patients assessed with three different measures
ConclusionsThe associations between clinical aspects of PD and QoL vary substantially among all three measures. Importantly, depression as a frequent comorbidity in PD is underestimated by the EQ-5D, but not by the PDQ-39 and the WHOQOL-100/WHOQOL-BREF. In turn, motor impairments are underestimated by the latter and associated strongest with QoL in the EQ-5D. (Source: Journal of Neurology)
Source: Journal of Neurology - September 12, 2018 Category: Neurology Source Type: research