European Journal of Neurology

BACKGROUND: Anemia is associated with poor clinical outcome after ischemic and hemorrhagic stroke. We examined the association between anemia and outcome in patients with cerebral venous thrombosis (CVT). METHODS: Consecutive adult patients with CVT were included from seven centers. Anemia at admission was scored according to World Health Organization definitions. Poor clinical outcome was defined as a modified Rankin Scale (mRS) score 3-6 at last follow-up. A multiple imputation procedure was applied for handling missing data in the multivariable analysis...

BACKGROUND: Subthalamic Deep Brain Stimulation (STN DBS) is an effective therapy against medication-refractory motor complications in patients with Parkinson's Disease (PD). However, it remains difficult to predict which baseline patient characteristics are associated with Quality of Life (QoL) after surgery. OBJECTIVE: To identify preoperative factors associated with QoL after STN DBS by systematically reviewing publications of sufficient methodological quality...

From all ischemic stroke subtypes, those attributed to atrial fibrillation are known to present with increased neurological severity resulting in death or major long-term disability [1]. Non-vitamin K oral anticoagulants (NOACs) are known to be both safe and effective for stroke prevention in patients with history of atrial fibrillation (AF), with evidence from randomized controlled clinical trials (RCTs) suggesting a significant reduction for the risk of both cerebral/systemic embolism and intracranial hemorrhage (ICH) compared to warfarin...

BACKGROUND: To investigate the prevalence and cardiovascular risk factors (CRFs) of asymptomatic intracranial atherosclerotic stenosis (aICAS) among middle-aged and older adults living in rural communities in China. METHODS: This population-based study included 2019 subjects (age ≥40 years, 52.3% women) who were free of stroke and living in rural communities in China. In October 2017-May 2018, data on demographics, CRFs, and health conditions were collected through face-to-face interviews, physical examination, and laboratory test...

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BACKGROUND: Drug resistant idiopathic generalized epilepsy (IGE) remains challenging despite a favorable overall prognosis of IGE and little is known about basic epidemiology and long-term outcome of drug resistant IGE. We aimed at describing the incidence, prevalence, and outcome of IGE in an unbiased, population-based cohort. METHODS: In 2014-2018, all patients (≥17 years) with IGE inhabiting the island of Funen (496,000 inhabitants) were recruited. We assessed socioeconomic and clinical information available for 406 individuals...

In a recent original article [1] and the accompanying editorial [2], the term pragmatic was used to define a trial conducted to determine the efficacy of minocycline (daily doses of 400 mg or 200 mg) vs placebo in patients with mild Alzheimer's disease (MADE trial). Whereas the authors [1] mentioned 'pragmatic' only once, the editorialist mentioned it several times, and included it in the title [2].

BACKGROUND: Previous studies reported conflicting results regarding possible anticipation in familial E200K Creutzfeldt-Jakob disease (fCJD). OBJECTIVES: To use a large database to assess age of disease onset (AODO) in CJD. METHODS: The study population included 477 CJD patients (266 with fCJD,145 with sporadic CJD (sCJD) and 66 patients of Libyan origin but negative family history) from the Israeli registry of CJD conducted since 1954. In all patients AODO in relatives and family trees were documented...

BACKGROUND AND PURPOSE: Transorbital sonography (TOS) emerged as promising imaging method for the diagnosis and follow-up of acute optic neuritis (ON). Available studies report increase in the optic nerve diameter (OND) and optic nerve sheath diameter (ONSD) in case of first episode of ON in the affected eye compared to either the contralateral unaffected eye or controls. However, the utility of TOS in case of recurrent episodes of ON has never been assessed. METHODS: In our prospective cohort study we assessed the diagnostic utility of TOS in patients with demyelinating diseases of the central nervous system, and examined further the association between TOS, optical coherence tomography (OCT) and visual evoked potentials (VEPs)...

BACKGROUND: Autoimmune encephalitides (AE) include a spectrum of neurological disorders, whose diagnosis revolves around the detection of neuronal antibodies (Abs). Consensus-based diagnostic criteria (AE-DC) allow clinic-serological subgrouping of AE, with unclear prognostic implications. We studied AE-DC impact on patients' management, focusing on the subgroup of Ab-negative-AE. METHODS: Retrospective multicenter study on patients fulfilling AE-DC. All patients underwent Ab testing with commercial cell-based-assays (CBAs) and, when available, in-house assays (immunohistochemistry, live/fixed CBAs, neuronal cultures), that contributed to define final categories...

BACKGROUND: To examine the consequences of antiepileptic drug (AED) exposure during pregnancy on language abilities in children of mothers with epilepsy aged 5 and 8 years. METHODS: The study population included children of mothers with and without epilepsy enrolled in the Norwegian Mother and Child Cohort study 1999-2008. Mothers prospectively provided information on epilepsy diagnosis, AED use during pregnancy, and the child's language abilities at age 5 and 8 years, in questionnaires with validated language screening tools...

BACKGROUND AND PURPOSE: Little is known about the long-term association between transient ischaemic attack (TIA) and stroke. Therefore, the goal of this study was to analyze the long-term risk of stroke and associated predictors in a large cohort of TIA patients followed in general practices in Germany. METHODS: This study included patients with an initial TIA diagnosis and subsequently followed up in one of 1262 general practices in Germany between January 2007 and December 2016 (N = 19 824 patients)...

BACKGROUND: Previous investigations show that bilinguals exhibit the first symptoms of dementia 4-5 years later than monolinguals. Therefore, bilingualism has been proposed as a cognitive reserve mechanism. Recent studies have advanced towards an understanding of the brain mechanisms underlying bilingualism's protection against dementia, but none of them deals with white matter (WM) diffusion. METHODS: In this study, we investigated this topic by measuring WM integrity in a sample of 35 bilinguals and 53 passive bilinguals with Mild Cognitive Impairment (MCI)...

BACKGROUND: In addition to lowering stroke risk, warfarin use is also associated with reduced stroke severity in patients with atrial fibrillation and acute ischaemic stroke. We sought to determine whether the effect of non-vitamin K oral anticoagulants (NOACs), compared to warfarin, differed by stroke severity. METHODS: We identified phase III randomised controlled trials with participants who were randomised to receive NOACs or warfarin for stroke prevention in the setting of non-valvular atrial fibrillation...

BACKGROUND: The pathophysiology of Parkinson's disease (PD) remains unclear. Voxel-based morphometry (VBM) detects local structural differences in brain tissue such as grey matter volume (GMV) between groups, which is helpful in understanding the pathophysiology of PD. Published VBM studies of GMV changes in PD have shown inconsistent results. Therefore, we conducted a voxel-wise meta-analysis of VBM studies to detect consistent GMV changes in PD. METHODS: We searched the published literature comparing whole-brain GMV between PD patients and healthy controls (HCs) using VBM...

BACKGROUND AND PURPOSE: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a heterogeneous autoimmune disorder critically lacking diagnostic biomarkers. Autoantibodies to nodal and paranodal components have recently been described in a small subset of patients. Here, the diagnostic value of immune reactivity toward the myelin compartment was investigated. METHODS: Ninety-four French CIDP patients were retrospectively studied. The reactivity toward the peripheral nerve was investigated...

OBJECTIVE: To assess the value of insoluble PABPN1 muscle fibres nuclei accumulation in the diagnosis of atypical cases of Oculopharyngeal muscular dystrophy (OPMD). METHODS: We analysed muscle biopsies from a selected cohort of 423 adult patients by immunofluorescence from several Italian neuromuscular centres: 30 muscle biopsies of genetically proven OPMD, 30 biopsies from patients not affected by neuromuscular disorders, 220 from genetically undiagnosed patients presenting ptosis or swallowing disturbances, progressive lower proximal weakness and/or isolated rimmed vacuoles at muscle biopsy and 143 muscle biopsies of patients affected from other neuromuscular diseases...

BACKGROUND AND PURPOSE: Amyotrophic lateral sclerosis (ALS) is increasingly recognized as a multisystem disorder with 30%-50% of patients exhibiting cognitive impairment. The pathophysiological mechanisms of cognitive dysfunction are probably multifactorial although hypoventilation secondary to respiratory dysfunction may contribute to cognitive decline. The current study aimed to identify the relationship between respiratory function in ALS patients and the presence and degree of cognitive impairment...

BACKGROUND: Genetic Generalized Epilepsies (GGEs) encompass a group of syndromes of mainly genetic causes, characterized by the involvement of both hemispheres. MicroRNAs (miRNAs) are small non-coding RNAs with a critical role in the regulation of neuronal biological processes through gene expression modulation. Dysregulated miRNA expression has been shown in epilepsy. Due to their stability in biological fluids like serum, microRNAs have assumed a prominent role in biomarker research...

BACKGROUND AND PURPOSE: Increasing evidence has demonstrated that aquaporin-4 (AQP4) immunoglobulin G causes damage to the kidney in neuromyelitis optica spectrum disorder (NMOSD). However, changes in urinalysis in NMOSD have not been investigated thus far. Our objective was to evaluate the changes in urinalysis in NMOSD patients. METHODS: Case data were collected from 44 patients with AQP4 antibody-positive NMOSD, 53 patients with multiple sclerosis (MS) and 79 age- and sex-matched healthy controls...