European Journal of Neurology

OBJECTIVE: To determine the effects of Apolipoprotein E ε4 (APOE ε4) genotype and sex together on longitudinal change in adjusted hippocampal volume (hippocampal volume/intracranial volume ratio [HpVR]) across the Alzheimer's disease (AD) continuum. METHODS: At baseline, we included 372 individuals with normal cognition (NC), 738 individuals with mild cognitive impairment (MCI), and 271 patients with mild AD from the Alzheimer's Disease Neuroimaging Initiative (ADNI) database...

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BACKGROUND: As high proportion of people with clinically isolated syndrome (pwCIS) exhibit sympathetic adrenergic and sudomotor dysfunction, the aim of this study was to investigate the evolution of autonomic nervous system (ANS) abnormalities in pwCIS over a two-year follow-up. METHODS: This was a prospective cohort study in which 121 pwCIS were enrolled and followed for two years. After two-year follow-up, data were available for 84 pwCIS. ANS symptoms were evaluated with the Composite Autonomic System Score-31 (COMPASS-31) and results of the ANS tests were expressed using the the Composite Autonomic Scoring Scale (CASS) at baseline and month 24 visits...

INTRODUCTION: Essential Tremor (ET) and Parkinson's disease (PD) sometimes overlap in their clinical expression with ET preceding PD onset, leading often to misdiagnosis. Transcranial Sonography (TCS) has been shown as valid and non-invasive diagnostic tool to identify early idiopathic PD and to differentiate it from ET. The purpose of this study was to investigate the relevance of hyperechogenicity of Substantia Nigra (SN+) in ET patients. METHODS: 138 patients (79 PD, 59 ET) and 50 matched controls underwent TCS examination at baseline...

BACKGROUND AND PURPOSE: Previous animal studies have suggested disrupted intestinal microbiome in amyotrophic lateral sclerosis (ALS). Due to the known effect of antibiotics on gut microflora, the potential role of antibiotics use on the risk of ALS deserves an investigation. METHODS: A nested case-control study was conducted using several Swedish national registers. We included 2,484 ALS patients diagnosed between July 1, 2006 and December 31, 2013 as cases and randomly selected five controls per case who were individually matched to the case by sex, birth year, and area of residence from the general Swedish population...

BACKGROUND AND PURPOSE: CLEC-2 is a C-type lectin-like receptor with the prominent involvement in platelet activation, which was increased in coronary heart disease (CHD) and acute ischemic stroke (AIS) and was associated with stroke progression and stroke prognosis. Here, we aimed to examine the prognostic value of CLEC-2 in death and vascular events recurrence in acute ischemic stroke patients. METHODS: 352 patients with AIS were prospectively studied. All patients were followed up for 1 year...

BACKGROUND: Inflammation and oxidative stress (OS) have been clearly linked to neurodegeneration. However, studies investigating the associations between peripheral markers of inflammation and cognitive decline have produced mixed results. This is possibly due to the fact that markers are typically tested individually despite the fact that biologically they function interactively. Thus, the aim of this study was to investigate the association between a combination of OS/inflammation markers and outcomes including MCI diagnosis, cognitive decline, and hippocampal atrophy...

BACKGROUND: To determine the electroclinical findings in benign childhood focal epilepsy with vertex spikes (BEVS) with epileptic negative myoclonus (ENM) restricted to lower limbs. METHODS: We reviewed electroencephalogram (EEG) database of Peking University First Hospital and medical records of patients with BEVS and ENM restricted to lower limbs. RESULTS: Twenty-seven patients with BEVS had ENM restricted to lower limbs. Twelve started as ENM restricted to lower limbs...

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BACKGROUND: Ischemic stroke frequently has a cardioembolic (CE) source. We evaluated clinical and echocardiographic parameters associated with CE stroke. METHODS: We retrospectively analysed 93 consecutive ischemic stroke patients who underwent a transthoracic echocardiogram; strokes were classified by TOAST (Trial of Org 10172 in Acute Stroke Treatment) criteria. Echocardiographic parameters related to cardioembolic stroke, including left atrial volumes and function, were compared to 73 healthy controls...

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BACKGROUND: The clinical characteristics of cluster headache (CH) are based mainly on retrospective attack descriptions of "usual" attacks, but whether these reports are reliable is uncertain. We aimed to compare retrospective and prospective attack descriptions and describe the within- and between patient variability of attacks. METHODS: Fifty-seven CH patients underwent a semi-structured interview obtaining a retrospective account of usual CH attacks...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

BACKGROUND AND PURPOSE: Low density lipoprotein receptor-associated protein 4 (LRP4) autoantibodies have recently been detected in myasthenia gravis (MG), but little is known about the clinical characteristics associated with this serological type. In this study, the clinical features of Chinese patients with anti-LRP4 antibody-positive MG were characterized. METHODS: A total of 2172 MG serum samples were collected from patients in various parts of China. ELISA was used to detect AChR-Ab and Titin-Ab, and cell-based assays were used to detect MuSK-Ab and LRP4-Ab...

BACKGROUND: A composite instrument able to rapidly and reliably assess the most relevant motor and non-motor afflictions suffered by Parkinson's disease (PD) patients in a real world clinic setting is an unmet need. The recently-validated PD Composite Scale (PDCS) was designed to fulfill this gap as a quick, comprehensive PD assessment. OBJECTIVE: Extensive evaluation of the PDCS's clinimetric properties using a large international sample. METHODS: Cross-sectional study in which the PDCS, the Movement Disorder Society-Unified Parkinson's Disease Rating Scale, and the Clinical Impression of Severity Index for PD were applied...

OBJECTIVE: To optimize subcutaneous therapy with immunoglobulins we compared large volume infusion of IgG facilitated by pretreatment with hyaluronidase (fSCIG) to conventional infusion of multiple small dosages (cSCIG) in 20 patients with multifocal motor neuropathy (MMN). METHODS: A randomized, non-inferiority and observer-blinded cross-over design was applied with a treatment period of 24 weeks at each therapy. RESULTS: In 18 patients fSCIG was feasible, 2 patients leaving the study due to side-effects...

BACKGROUND: Patients with acute pontine infarcts generally have good short-term motor outcomes; however, the mechanisms underlying this recovery of function remain unclear. METHODS: We recruited 20 well-recovered patients with acute pontine infarcts and 20 well-recovered patients with acute striato-capsular infarcts. Fugl-Meyer assessment and resting-state functional magnetic resonance imaging were performed 1, 4, and 12 weeks after onset. Patients were further assigned to better and worse recovery subgroups according to the degree of motor recovery at the 12th week after stroke...

OBJECTIVES: Neurosarcoidosis is a rare inflammatory disorder of unknown cause. The aim of this study was to evaluate the value of T/B lymphocyte population counts and the concentrations of the cytokines IL-6 and 10 in the cerebrospinal fluid (CSF) of neurosarcoidosis patients. METHODS: We conducted a retrospective study of CSF biomarkers in patients with neurosarcoidosis who underwent CSF analysis between 2012 and 2017 as well as various control populations. RESULTS: We analyzed 43 patients with neurosarcoidosis, 14 with multiple sclerosis (MS), and 48 with other inflammatory disorders...