European Journal of Neurology

BACKGROUND: This study was conducted to evaluate whether post-thymectomy rapid remission of ocular myasthenia gravis (oMG) is a prognostic indicator of good long-term neurological outcome. METHODS: We enrolled 84 oMG patients who underwent thymectomy at our institute. The incidence of 5-year complete stable remission (CSR) was compared between patients with rapid remission of MG status (<1 month after surgery) and those with non-rapid remission. RESULTS: Kaplan-Meier survival analysis indicated that the incidence of CSR was higher in oMG patients with rapid remission than in those without rapid remission (P<0...

BACKGROUND: The aim of this study is to assess the prevalence of cancer and its characteristics in patients with ischemic stroke and to compare this with cancer prevalence in the general population. METHODS: This is a multicenter cohort study with 2,736 patients presenting with ischemic stroke or TIA. The prevalence of cancer has been assessed by interview and verified by reviewing all medical records. In stroke patients with a history of cancer, we studied subtype of cancer and its treatment characteristics...

BACKGROUND: Parkinson's disease (PD), Dementia with Lewy bodies (DLB) and Alzheimer's disease (AD) are three of the most common neurodegenerative disorders. Up to 20% of these patients have the wrong diagnosis, due to overlapping symptoms and shared pathologies. A cerebrospinal fluid (CSF) biomarker panel for AD is making its way into the clinic, but an equivalent panel for PD and DLB, and for improved differential diagnoses is still lacking. OBJECTIVE: Using well defined, community based cohorts and validated analytical methods, we determined the diagnostic value of CSF total-α-syn (t-α-syn) alone and in combination with total tau (t-tau) in newly-diagnosed patients with PD, DLB and AD...

BACKGROUND AND PURPOSE: Cost of medication overuse headache (MOH) is underestimated. We aimed to address cost-effectiveness of a structured treatment protocol and to present annual cost estimates. METHODS: Patients were enrolled on occasion of structured treatment protocol, administered a research protocol addressing direct and indirect cost, and followed-up for three months. RESULTS: Out of 176 enrolled patients, 138 completed the study. Three-month cost per patient fell from 2,989€ to 1,160€: the difference was 696€ per month for patients treated in ward and 466€ for those treated in day-hospital, thus it takes two to three months to compensate for protocol's cost...

BACKGROUND AND PURPOSE: Data suggest a relationship between sexual dysfunction, mainly erectile dysfunction in men, and worse disease progression in Parkinson's disease (PD). There is scant evidence on the correlates of sexual activity in PD patients. By involving a subgroup of 355 patients from the PRIAMO (Parkinson Disease Non Motor Symptoms) study, the present 24-month longitudinal prospective analysis aims to demonstrate that the presence of active sexual life is associated with disease progression in early PD...

BACKGROUND: Although non-alcoholic fatty liver disease (NAFLD) shares common cardiovascular risk factors with cerebral white matter hyperintensity (WMH), a few studies reported the association between NAFLD and WMH. We investigated the association between presence of NAFLD with its severity and the volume of WMH. METHODS: This cross-sectional study was conducted for the 2,460 subjects who voluntarily participated in health screening check-ups including brain MRI and liver ultrasonography at Health Promotion Center at Seoul National University Hospital from 2009 to 2013...

BACKGROUND: Elevated blood pressure (BP) is prevalent, modifiable and has been hypothesized to lead to increased risk of dementia. DATA: 2,593,629 people from the United Kingdom Clinical Practice Research Database, aged ≥40 years or older with a BP measurement between 1992 to 2011 and no prior record of dementia. METHODS: We used Poisson regression models to study the association between BP and physician-diagnosed dementia. BP is believed to fall during the prodromal phase of dementia development, so we investigated associations by categories of time since BP measurement (<5 years, 5-10 years, >10 years), and by subtypes of dementia...

BACKGROUND: We conducted a nationwide survey to understand the epidemiology of cerebral amyloid angiopathy-related intracerebral hemorrhage (CAA-related ICH) and cerebral amyloid angiopathy-related inflammation/vasculitis (CAA-ri) in Japan. METHODS: To estimate the total number and clinical features of patients with CAA-related ICH and CAA-ri between January 2012 and December 2014 and to analyze their clinical features, we sent questionnaires to randomly selected hospitals in Japan...

BACKGROUND: Relationship of estimated glomerular filtration rate (eGFR) with complications after stroke has not been fully characterized for entire clinical spectrum of eGFR and for the fluctuation in eGFR during hospital stay. METHODS: Data from the Norfolk and Norwich Stroke Registry recorded between January 2003 and April 2015 was analysed. eGFR was categorized into six clinically relevant categories as per Kidney Disease Improving Global Outcomes guidelines...

BACKGROUND: To analyze the characteristics and progression of cognitive dysfunction in non-demented idiopathic rapid eye movement sleep behavior disorder (iRBD) patients with baseline olfactory function. METHODS: From a prospective polysomnography-confirmed iRBD cohort, 25 patients (16 patients in 2-year follow-up) and 13 normal controls were included. Initial and 2-year follow-up cognitive functions were analyzed with olfactory function and 18 F-FP-CIT uptake in deep nuclei at the beginning...

BACKGROUND: To analyze the clinical characteristics of a long-term follow-up of patients with chronic and high frequency episodic migraine in treatment with OnabotulinumtoxinA. METHODS: We included patients diagnosed with high-frequency episodic migraine (HFEM) or chronic migraine (CM) according to ICHD-3beta. A comparative analysis was carried out at each study timepoint identifying outcome measures according to initial diagnosis and treatment duration. RESULTS: 578 patients were recruited and after 24 months outcome data was collected from 100 patients: 84...

BACKGROUND: Correct diagnosis of idiopathic inflammatory myopathies (IIM) may prevent harm from both lack of treatment in IIM patients and unnecessary treatment in non-IIM patients. However, it is unknown whether additional histopathological fascia examination may contribute to diagnosing IIM. METHODS: We reviewed 32 Magnetic Resonance Imaging (MRI)-guided en-bloc biopsies from patients diagnosed with IIM (except inclusion body myositis) from 2010-2017: dermatomyositis (DM; n=6), non-specific/overlap myositis (NM/OM; n=11), immune-mediated necrotizing myopathy (IMNM; n=12) and anti-synthetase syndrome (ASS; n=3)...

Congenital disorders of glycosylation (CDG) constitutes a rare group of inborn error of metabolism with myriad clinical presentation due to multisystem involvement. They are caused by mutations in the genes encoding for enzymes involved in the biosynthesis or remodelling of the oligosaccharide moieties of glycoprotein and glycolipid glycans. This article is protected by copyright. All rights reserved.

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BACKGROUND: MS patients frequently report cognitive difficulties which impact daily functioning. OBJECTIVE: Investigate the relationship between patient-reported cognitive impairment and depression, demographic and MS-related variables, and clarify its impact on self-reported health measures and employment. METHOD: A large 2-center survey included the Multiple Sclerosis Neuropsychological Screening Questionnaire (MSNQ), 2-question screening tool for depression, vitality, Health-Related Quality of Life (HR-QoL), Health-Promoting Lifestyle Profile II (HPLP-II) and questions assessing social network satisfaction and employment status...

BACKGROUND AND OBJECTIVE: In Parkinson's disease (PD), the course of the disorder is highly variable between patients. Well-designed, prospective studies for identifying PD progression biomarkers are necessary. Our aim is to show the results of baseline evaluations of an ongoing global PD Project, COPPADIS-2015 (COhort of Patient's with PArkinson's DIsease in Spain, 2015). METHODS: Observational, descriptive, nationwide study (Spain). The recruitment period ended in October 2017...

Previously, we described a significantly higher risk of Amyotrophic Lateral Sclerosis (ALS) among the population living on the eastern flank of the Mt. Etna with respect to the western flank (RR 2.75; 95% CI 1.64-4.89).[1] Since winds usually blow Etna ashes from westerly to north-westerly [2] , the eastern flank is the most exposed area and volcanogenic metals were proposed as a possible explanation. This article is protected by copyright. All rights reserved.

PURPOSE: To identify whether post-stroke hyperglycaemia (PSH) influences the levels of circulating biomarkers of brain damage and repair, and to explore whether these biomarkers mediate the effect of PSH on the ischemic stroke (IS) outcome. METHODS: Secondary analysis of the Glycaemia in Acute Stroke II study. Biomarkers of inflammation, prothrombotic activity, endothelial dysfunction, blood-brain barrier rupture, cell death and brain repair processes were analysed at 24-48 hours (baseline) and 72-96 hours (follow-up) after IS...

BACKGROUND: To investigate whether the neuromelanin-positive substantia nigra pars compacta area (NM-SNc) on neuromelanin magnetic resonance imaging (NM-MRI), and the specific binding ratio (SBR) on 123 I-N-v-fluoropropyl-2b-carbomethoxy3b-(4-iodophenyl)nortropane single photon emission computed tomography (DaT-SPECT) can be correlated with motor fluctuations (MF) in advanced Parkinson's disease (PD). METHODS: We enrolled 35 PD patients (60 ± 13 years) and 23 healthy individuals as controls (59 ± 19 years)...

BACKGROUND: High levels of autoantibodies against glutamic acid decarboxylase (GAD-ab) are associated with stiff-person syndrome (SPS). However, the full clinical spectrum associated with GAD-ab in Asians is unclear. We reviewed the clinical and immunological features of patients positive for GAD-ab in a large Taiwanese series. METHODS: Retrospective case series and immunological investigations were conducted between July 2007 and July 2017 at a tertiary referral centre in Taiwan...