European Journal of Neurology

OBJECTIVE: Differentiating the primary progressive aphasia (PPA) variants in clinical settings remains complex and challenging, especially for the logopenic (lv-PPA) and non-fluent variants (nfv-PPA). Recent studies suggest that visuospatial memory is more compromised in lv-PPA than in nfv-PPA and is relatively spared in the semantic variant (sv-PPA). Accordingly, assessment of visuospatial memory performance may assist in the differential diagnosis of PPA variants. Here, we investigated the utility of a novel computerised visuospatial working memory test-the Box Task-to differentiate the three PPA variants and typical Alzheimer's disease (AD)...

BACKGROUND AND PURPOSE: The anterior and posterior hippocampal networks represent verbal and spatial memory respectively, and may play different roles in the pathological mechanism of amnestic mild cognitive impairment (aMCI) and non-amnestic MCI (naMCI), which hasn't been explored. METHODS: A total of 990 older adults with 791 normal control (NC, 65 ± 6 years, 502 women), 140 aMCI (66 ± 7 years, 84 women) and 59 naMCI (66 ± 7 years, 38 women) were included. We used a multivariate method, partial least squares, to assessed the structural covariance network of anterior hippocampus (aHC) and posterior hippocampus (pHC), and their relationships with verbal memory and spatial memory in the three groups...

BACKGROUND: A large proportion of headache sufferers do not routinely seek medical care. App-based technologies permit the collection of real-world data over time and between countries that can help assess true burden of headache. This study used a mobile phone application to collect information on real-world burden of self-diagnosed headache and to describe its impact on daily life in headache sufferers who do not routinely seek medical advice. METHODS: This retrospective, non-interventional, cross-sectional study analysed self-reported data from users of the 'Migraine Buddy' app...

BACKGROUND: Diabetes mellitus (DM) is considered a risk factor for Alzheimer's disease (AD), sharing some pathological pathways such as activation of amyloid cascade and tau phosphorylation. In the present work, we investigated to what extent DM could impact on neurodegeneration within the AD continuum, using β amyloid (A: Aβ1-42 ) and phosphorylated tau (T: p-Tau) biomarkers to discriminate patients into Alzheimer's pathologic change (A+/T-) and AD (A+/T+), according to the National Institute on Aging and Alzheimer's Association...

In medicine research, two universes exist. One is the universe of clinical studies in which clinicians and patients reside. In this universe, academic and non-academic driven clinical studies are performed in which prospective or retrospective collected clinical and paraclinical patient data are analyzed. Because standardization of patients is an illusion, these studies often require a huge number of included study subjects to demonstrate clinical relevant findings. In the case of prevalent pathology and large outcome effects, clinical research is highly rewarding and relevant...

BACKGROUND: Several clinical and demographic factors relate to anatomic spread of adult-onset isolated dystonia, but a predictive model is still lacking. OBJECTIVES: 1) To develop and validate a predictive model of anatomic spread of adult-onset isolated dystonia; 2) to evaluate whether presence of tremor associated with dystonia influences model predictions of spread. METHODS: Adult-onset isolated dystonia participants with focal onset from the Dystonia Coalition Natural History Project database were included...

OBJECTIVE: The severity of early brain edema after aneurysm rupture was reported to be strongly associated with the risk of poor outcome after aneurysmal subarachnoid hemorrhage (SAH). Using the recently developed SEBES (Subarachnoid Hemorrhage Early Brain Edema Score), we analyzed the predictors of early brain edema and its impact on the complications related to intracranial pressure (ICP) increase after SAH and poor outcome. METHODS: All consecutive SAH cases treated between January 2003 and June 2016 with assessable SEBES were included (n=745)...

BACKGROUND: Comorbidities are common in multiple sclerosis (MS), and have been associated with worse outcomes and increased healthcare resource usage. We studied the frequency of comorbidities and adverse health behaviors (AHB) in MS patients in the Mediterranean region of Catalonia. METHODS: Population-based, case-control study using primary healthcare information covering 80% of Catalonia's population. Cases were matched by age/sex with randomly-chosen controls (ratio 1:5)...

We read with interest the recent report by Wurthmann and colleagues of increased motion sensitivity as well as enhanced susceptibility to motion sickness in vestibular migraine (1). Our previous experimental data supports the findings of enhanced sickness susceptibility (2) but, in apparent contradiction, we observed that motion thresholds were raised (3).

BACKGROUND: Clinicians have reported an increase in functional tic-like behaviours in children and youth during the COVID-19 pandemic. We describe adults developing rapid onset of functional tic-like behaviours between May 2020 and June 2021. METHODS: Data were analysed from the Adult Tic Disorders Registry, a single-site,12-month prospective cohort study which began enrolment in January 2021. We compared clinical features of participants with Tourette Syndrome or Persistent Motor/Vocal Tic Disorder to participants with Rapid Onset Tic-Like Behaviours...

BACKGROUND: Cerebral venous sinus thrombosis (CVST) has been described after vaccination against SARS-CoV-2. We report clinical characteristics of 213 post-vaccination CVST cases notified to the European Medicines Agency (EMA). METHODS: Data on Adverse Drug Reactions after SARS-CoV-2 vaccination notified until 8 April 2021 under the Medical Dictionary for Regulatory Activities Term 'Central nervous system vascular disorders' were obtained from the EudraVigilance database...

BACKGROUND: We investigated plasma neurofilament light chain concentrations (pNfL) as a biomarker for neuroaxonal damage and disease activity using data from phase 3 trials of ozanimod in relapsing multiple sclerosis (RMS). METHODS: pNfL were measured before and after ozanimod 0.46 mg or 0.92 mg daily or interferon β-1a 30 µg weekly in the randomized, double-blind, SUNBEAM and RADIANCE trials. In these post hoc analyses, we investigated relationships between pNfL (at baseline and median percentage change from baseline to month 12 [SUNBEAM] or 24 [RADIANCE]) and clinical and magnetic resonance imaging outcomes...

BACKGROUND: Although the COVID-19 vaccines are currently recommended for people with multiple sclerosis (MS), the fact that they were not specifically tested in people with MS raises uncertainty regarding the safety of the vaccines in this population. The purpose of this study is to report real-life safety data of the BNT162b2 COVID-19 vaccine in a cohort of MS patients. METHODS: An anonymous survey was distributed to 425 MS patients. Participants were asked general demographic and disease-related questions and specific questions regarding the safety profile of the COVID-19 vaccine...

BACKGROUND: Non-myelopathic degenerative cervical spinal cord compression (NMDC) frequently occurs throughout aging and may progress to potentially irreversible degenerative cervical myelopathy (DCM). Whereas standard clinical MRI and electrophysiological measures assess compression severity and neurological dysfunction, respectively, underlying microstructural deficits still have to be established in NMDC and DCM patients. The study aims to establish tract-specific diffusion MRI markers of electrophysiological deficits to predict the progression of asymptomatic NMDC to symptomatic DCM...

BACKGROUND: Consensus criteria for autoimmune limbic encephalitis (ALE) allow for a diagnosis even without neural antibodies (Abs), but it remains unclear which clinical features should prompt neural Ab screening in temporal lobe epilepsy patients. METHODS: We identified 47 patients from a tertiary epilepsy center with mediotemporal lobe seizures and additional features suggestive of limbic involvement, including either memory deficits, psychiatric symptoms, mediotemporal MRI hyperintensities or inflammatory CSF...

BACKGROUND: Cognitive impairment frequently affects multiple sclerosis (MS) patients. However, its neuroanatomical correlates still need to be fully explored. We investigated the contribution of structural and functional magnetic resonance imaging (MRI) abnormalities in explaining cognitive impairment in MS. METHODS: Brain dual-echo, diffusion tensor, 3D T1-weighted and resting-state (RS) MRI sequences were acquired from 276 MS patients and 102 healthy controls...

BACKGROUND: Although body weight variability has been associated with mortality, cardiovascular disease, and dementia, the relationship between body weight variability and Parkinson's disease (PD) has been rarely studied. We aimed to investigate the longitudinal association between body weight variability and PD incidence. METHODS: A nationwide population-based, cohort study was conducted using the database from the Health Insurance Review and Assessment Service of the whole Korean population...

OBJECTIVE: To provide an overview of genotype and phenotype distribution in a cohort of patients with Charcot-Marie-Tooth disease (CMT) and related disorders from Central South China. METHODS: We enrolled 435 patients and collected detailed clinical data. Multiplex ligation-dependent probe amplification for PMP22 duplication/deletion and CMT multi-gene panels sequencing were performed. Whole-exome sequencing was further applied in the remaining patients who failed to achieve molecular diagnosis...

BACKGROUND: Left atrial cardiac disease is a suspected cause of embolic stroke of undetermined source (ESUS). AIMS: We tested the hypothesis that left atrial fibrosis quantified using late-gadolinium-enhancement MRI (LGE-MRI) predicts recurrent stroke or atrial fibrillation (AF) in patients with ESUS. METHODS: We compared atrial fibrosis in healthy controls and patients with lacunar stroke, ESUS, and known AF with or without prior stroke. We followed patients with ESUS prospectively for the primary outcome of recurrent ischemic stroke, incident AF, or both...

Neurological disorders are the third most common cause of disability and premature death in Europe and their prevalence and burden will likely increase with the progressive ageing of the European population (1). Almost one in three people worldwide has a neurological disorder at some point in their lifetime (2) with the most common neurological causes of disability being stroke, migraine, and Alzheimer's disease and other dementias (2).