European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies

BACKGROUND: Antibodies to myelin oligodendrocyte glycoprotein (MOG) have been identified in both children and adults with demyelination, with a strong association with bilateral or recurrent optic neuritis (ON). However, the full clinical spectrum of this newly described condition is unknown. We sought to describe non-ON inflammatory ophthalmological presentations such as uveitis and optic perineuritis in the context of MOG antibody seropositivity. METHODS: Using a live cell-based assay analysed by flow cytometry, we identified seropositive patients referred for MOG antibody testing in Australasia between 2014-2017...

BACKGROUND: Acute endovascular reperfusion treatment (aERT) of stroke patients with large vessel occlusions (LVO) is efficacious and safe according to several clinical trials. Data on outcome and safety of aERT in daily clinical routine is warranted, and in this study we present national data from Denmark 2011 -2017. METHODS: National data for Denmark from 2011 through 2017 on all aERT procedures in patients with acute ischemic stroke and CTA/MRA verified LVO. Data were derived from the Danish Stroke Registry (DRS), a national clinical quality registry to which reporting is mandatory for all hospitals treating stroke patients...

BACKGROUND AND PURPOSE: The aim of this study was to examine the association among remote diffusion-weighted imaging lesions (R-DWILs), imaging markers of cerebral small vessel disease (cSVD), and total cSVD burden in patients with primary intracerebral hemorrhage (ICH). METHODS: Three hundred and forty-four consecutive primary ICH patients were enrolled prospectively. R-DWILs on magnetic resonance imaging (MRI), as well as four imaging markers of cSVD, including cerebral microbleeds (CMBs), white matter hyperintensities (WMHs), lacunes, and enlarged perivascular spaces (EPVS) were rated with validated scales...

BACKGROUND: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly-progressive neurodegenerative disease caused by an abnormal isoform of the human prion protein. Structural MRI in pathologically-confirmed sCJD patients was compared to cognitively-normal individuals to identify a cortical thickness signature of sCJD. METHODS: This retrospective cross-sectional study compared autopsy-confirmed sCJD patients with dementia (n=11) to age- and sex-matched cognitively normal individuals (n=22)...

BACKGROUND: Multiple sclerosis is a demyelinating and neurodegenerative disease of the central nervous system that can be tracked through biomarkers of disease status. We investigated the effects of exercise on MS biomarkers associated with central nervous system status including imaging, blood-brain barrier function and neurotrophic factors. METHODS: We conducted open-dated searches of Scopus, Medline, EMBASE and the Cochrane Library. We included studies written in English describing interventions of exercise that measured one or more of the biomarkers associated with multiple sclerosis published until October 2018...

Corticobasal degeneration (CBD) is a neurodegenerative condition characterized by 4R-tau protein deposition in several brain regions that clinically manifests itself as a heterogeneous atypical parkinsonism typically expressing in the adulthood. The prototypical clinical phenotype of CBD is corticobasal syndrome (CBS). Important insights into the pathophysiological mechanisms underlying motor and higher cortical symptoms in CBS have been gained by using advanced neuroimaging and neurophysiological techniques...

OBJECTIVE: Analyzing cerebrospinal fluid (CSF) is crucial in the diagnostic workup of epileptic seizures to rule out autoimmunity or infections as underlying cause. Therefore, the description of postictal changes in CSF is essential to differentiate between negligible and etiopathologically relevant changes in CSF profile. METHODS: A retrospective analysis of 247 patients newly diagnosed with epileptic seizures and CSF analysis during diagnostic workup was conducted...

BACKGROUND: Acquired neuromyotonia can occur in patients with thymoma, alone or in association with myasthenia gravis (MG), but the clinic-prognostic significance of such comorbidity is largely unknown. We investigated clinic-pathologic features, along with the occurrence of neuromyotonia as predictors of tumor recurrence in patients with thymoma-associated myasthenia. METHODS: We retrospectively studied 268 patients with thymomatous MG. Patients with symptoms of spontaneous muscle overactivity were selected for autoantibody testing using immunohistology for neuronal cell-surface proteins and cell-based assays for CASPR2, LGI1, Glycine receptor, and Netrin-1 receptors antibodies...

INTRODUCTION: The unanticipated magnetic resonance imaging (MRI) detection in the brain of asymptomatic subjects of white matter lesions suggestive of multiple sclerosis (MS) has been named as radiologically isolated syndrome (RIS). As the difference between early MS (i.e., clinically isolated syndrome [CIS]) and RIS is the occurrence of a clinical event, it should be logical to improve detection of subclinical form without interfering with MRI as there are radiological diagnostic criteria for that...

We examined the existence of a prodrome in relapsing-onset multiple sclerosis (RMS) and primary progressive MS (PPMS) by comparing the number of physician encounters (via International Classification of Diseases diagnoses and physician speciality) in the five years before symptom onset in 1,887 RMS, 171 PPMS cases, and 9,837 matched population controls. Negative binomial regression models were sex, age, socioeconomic status and calendar year adjusted. Compared to RMS cases, PPMS cases had more nervous system-related encounters (adjusted rate ratio [aRR]=3...

Dysphagia is a common neurological, often underdiagnosed symptom of an acute stroke, particularly, in patients suffering from a stroke in the posterior circulation, particularly brainstem infarction or a malign anterior circulation stroke, with altered consciousness. Up to 80% of acute stroke patients showed a dysfunction in swallowing, whereas the determined rate of dysphasia is depending on the stroke's severity, the type and timing of the dysphasia screening techniques. A quarter of the dysphasia patients developed a chronic swallowing dysfunction which severely affects the quality of life in addition to the malnutrition and dehydration risk ...

BACKGROUND: Individuals with GBA (glucocerebrosidase) mutations are at increased risk of Parkinson's disease (PD). It is still debated, however, whether this increased risk results from impaired glucocerebrosidase activity leading to substrate accumulation. Comparing the presence of prodromal PD marker in GBA mutation carriers and in Gaucher patients (in which substrate accumulation is extensive) can assist in clarifying this issue. METHODS: In this cross sectional study we compared the hyperechogenic area of the substantia nigra, a prodromal PD marker, in large cohorts of GBA mutation carriers (n=71) and Gaucher patients (n=145)...

BACKGROUND: The prevalence and duration of NMS in prodromal PD has not been extensively studied. The aim of this study was to determine the prevalence and duration of prodromal NMS (pNMS) in a cohort of recently diagnosed PD patients. METHODS: We evaluated the prevalence and duration of prodromal NMS in early PD patients (n= 154). NMS were screened for using the Nonmotor Symptom Questionnaire (NMS Quest). We subtracted the duration of the presence of each individual NMS reported from the duration of earliest motor symptom...

BACKGROUND: Elevation of cardiac troponin (cTn), a sensitive biomarker of myocardial injury, is observed frequently in severe acute neurological disorders. Case reports suggest that cardiac dysfunction may also occur in patients with transient global amnesia (TGA). Until now, no study has systematically assessed this phenomenon. METHODS: We performed a case-control study using data of consecutive patients presenting with TGA from 2010 to 2015. Multiple logistic regression analysis accounting for age, sex, and cardiovascular risk factors was performed to compare the likelihood of myocardial injury (defined as elevation of cTn>99th percentile, ≥14ng/L; high-sensitivity cTnT assay) in TGA with three reference groups: migraine with aura, vestibular neuritis, transient ischemic attack (TIA)...

BACKGROUND: Because of their potential to alter the integrity of collagen and other components of the extracellular matrix fluoroquinolone antibiotics might be involved in the pathogenesis of spontaneous cervical artery dissection (sCeAD). METHODS: In the setting of a single-center case-control study, we assessed whether fluoroquinolone use in the 30-day period before the index event was associated with sCeAD (cases) in comparison with a group of age- and sex-matched patients who suffered a first-ever acute cerebral infarction of a cause other than CeAD (non-CeAD IS, controls)...

OBJECTIVE: Presurgical evaluation of pediatric patients with drug-resistant focal epilepsy and negative (nonlesional) MRI is particularly challenging. Focal cortical dysplasia (FCD), a frequent pathological substrate in such setting, may be subtle on MRI and evade detection. The aim of this study was to use voxel-based MRI postprocessing to improve detection of subtle FCD in pediatric surgical candidates. METHODS: We included a consecutive cohort of pediatric patients undergoing presurgical evaluation with a negative MRI by visual analysis...

BACKGROUND: Significant clinical recovery has been commonly observed in ischemic stroke patients with irreversible brain structural damage. However, brain mechanisms that help to maintain clinical function remain unclear. METHODS: Sixty-two patients with acute ischemic stroke underwent longitudinal clinical assessments and MRI scanning. We evaluated clinical recovery trajectory using Hierarchical Linear Model and analysed intrinsic connectivity with a seed-based approach to examine its changing pattern based on the regional volume changes calculated using voxel-wise analysis...

BACKGROUND: We aimed to evaluate the efficacy of the catechol-O-methyltransferase (COMT) inhibitor, opicapone (25 and 50mg), as adjunct therapy to levodopa in a pooled population of Parkinson's disease patients who participated in the pivotal double-blind trials of opicapone and their 1-year open-label extensions. METHODS: Data (placebo, opicapone 25mg and opicapone 50mg) from the BIPARK 1 and 2 double-blind and open-label studies were combined. The studies had similar designs, eligibility criteria and assessment methods...

BACKGROUND: Subsyndromal delirium (SSD) refers to patients with delirious symptoms who do not meet criteria for delirium. We aimed to determine the prognostic significance of SSD in stroke patients. METHODS: We included 564 patients with ischemic stroke (median age: 71, 50.5% female). We used Confusion Assessment Method to assess symptoms of delirium and DSM-5 criteria to diagnose delirium. We defined SSD as one or more core features of delirium without fulfilling diagnostic criteria...

BACKGROUND: Post-ischemic immune cell invasion into the brain is well characterized in animal stroke models and contributes to neuronal damage. Therefore, it represents a promising therapeutic target. Cerebrospinal fluid (CSF) is easily accessible and may reflect cellular events within the parenchyma. However, comprehensive studies on CSF immune cells in stroke patients are lacking. METHODS: In a retrospective cohort study, we performed extensive immune-cell profiling in CSF and peripheral blood of patients with acute ischemic stroke and healthy controls...