European Journal of Neurology

BACKGROUND: Patients with secondary progressive multiple sclerosis (SP MS) and clinical and/or radiological activity could be the more likely to benefit from disease modifying treatments. To evaluate the proportions each year after progression onset, we studied patients with SP MS onset between 2002 and 2012 from a population-based multiple sclerosis registry in North-Eastern France. METHODS: Progression onset was first identified by the neurologist's diagnosis (N cohort), then by using an automated data-driven definition (D cohort)...

BACKGROUND: Measurement of the cross-sectional area (CSA) of cervical nerve roots using ultrasound is useful in the evaluation of inflammatory polyneuropathies and measurement of CSA of the vagal nerve might give information about involvement of autonomic nervous system. We performed a systematic review and meta-analysis of published CSA reference values for cervical nerve roots and vagal nerve. METHODS: We included available to date nerve ultrasound studies on healthy adults and provide meta-analysis for CSA of the following nerves: cervical nerve roots C5, C6, and C7 as well as vagal nerve in carotid sheath at the carotid bifurcation level...

BACKGROUND: Facioscapulohumeral muscular dystrophy is a debilitating inherited muscle disease for which various therapeutic strategies are being investigated. Thus far, little attention has been given in FSHD to the development of scientifically sound outcome measures fulfilling regulatory authorities' requirements. The aim of this study is to design a patient-reported Rasch-built interval scale on activity and participation for FSHD. METHODS: A pre-phase FSHD-Rasch-built overall disability scale (pre-FSHD-RODS; consisting of n=159 activity/participation items), based on the WHO international classification of disease-related functional consequences was completed by 762 FSHD patients (Netherlands: n=171; UK: n=287; USA n=221; France: n=52; Australia n=32)...

BACKGROUND: Mounting evidence supports an association between Guillain-Barré syndrome spectrum (GBSs) and severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. However, GBSs in the setting of coronavirus disease-2019 (COVID-19) remains poorly characterized, while GBSs prevalence among COVID-19 patients has not been previously systematically evaluated using a meta-analytical approach. METHODS: We performed a systematic review and meta-analysis of observational cohort and case-series studies reporting on the occurrence, clinical characteristics and outcomes of patients with COVID-19-associated GBSs...

BACKGROUND: At patient-level, the prognostic value of several features that are known to be associated with an increased risk of converting from relapsing remitting (RR) to secondary phase (SP) multiple sclerosis (MS), remain limited. METHODS: Among 262 RRMS patients followed up for ten years, we assessed the probability of developing the SP course based on clinical and conventional and non-conventional magnetic resonance imaging (MRI) parameters at diagnosis and after two years...

Panda et al state that we concluded that there is a higher likelihood of observing abnormal neurological signs and more student-centered teaching in the ward as compared to the outpatient setting, and that is why the students posted in the ward performed better in MCQ and OSCE examinations. However, we would like to clarify that this is just a possible interpretation of our findings. Our results indicate that the teaching setting had a significant impact on the learning outcomes and we hypothesize that this may be due to a higher likelihood of observing abnormal neurological signs or to more student-centered teaching on the ward...

BACKGROUND: 5-Fluorouracil (5-FU) and its oral prodrug capecitabine have been rarely but consistently associated with acute central nervous system toxicity, including transient leukoencephalopathies involving the splenium of the corpus callosum. METHODS: We performed a retrospective research in the French Pharmacovigilance database (FPDB) (January 1985 - July 2020) for adult patients affected by solid cancers who developed acute toxic leukoencephalopathies with splenial lesions following treatment with 5-FU or capecitabine...

OBJECTIVE: IVIg consists of pooled donor immunoglobulins (IgG), possibly including anti-Borrelia burgdorferi (Bbsl) antibodies. Apparent IVIg-related Bbsl seroconversion could lead to incorrect diagnosis of Lyme borreliosis. This cohort study was designed to determine how often IVIg treatment leads to apparent Bbsl seroconversion and whether antibodies disappear post-treatment. METHODS: Sera from chronic inflammatory demyelinating polyneuropathy (CIDP) and myositis patient were analyzed, drawn pre-treatment and 6-12 weeks after start of IVIg...

Recently, we have read the original article written by Yaling Lu et al1 , which was published in European Journal of Neurology [1]. The authors enrolled 635 acute ischemic stroke patients with soluble suppression of tumorigenicity 2 (sST2) measurements from the China Antihypertensive Trial in Acute Ischemic Stroke, one multicenter randomized clinical trial in China, to explore the relationship between sST2 and post-stroke depression (PSD).

BACKGROUND AND PURPOSE: Root and cord irritation from cervical spinal degenerative disease may share clinical features with progressive multiple sclerosis (MS), so diagnostic overshadowing may occur. We hypothesized that cervical stenotic spinal degenerative disease is commoner in progressive MS, compared to controls. METHODS: A retrospective case-control study of 111 cases (56 with progressive MS and 55 controls) was conducted. Five types of cervical spinal degenerative disease (disc degeneration, posterior disc protrusion, endplate changes, canal stenosis and foraminal stenosis) were assessed objectively on magnetic resonance imaging using published scales...

BACKGROUND: Although functional recovery is most pronounced in the first 6 months after stroke, improvement is possible also in the late phase. We explored the value of plasma neurofilament light chain (NfL), a biomarker of axonal injury and secondary neurodegeneration, for the prediction of functional improvement in late phase after stroke. METHODS: We measured baseline plasma NfL levels in 115 participants of a trial on the efficacy of multimodal rehabilitation in late phase after stroke...

BACKGROUND: Measurement of the cross-sectional area (CSA) of peripheral nerves using ultrasound is useful in the evaluation of focal lesions like entrapment syndromes and inflammatory polyneuropathies. We performed a systematic review and meta-analysis of published CSA reference values for lower extremity nerves. METHODS: We included available to date nerve ultrasound studies on healthy adults and provide meta-analysis for CSA of the following nerves: fibular nerve at fibular head, popliteal fossa; tibial nerve at popliteal fossa, malleolus; sural nerve at the level of the two heads of gastrocnemius muscle...

We read with interest the article published by Martins et al[1]. They concluded that there is a higher likelihood of observing abnormal neurological signs and more student-centered teaching in the ward as compared to the outpatient setting and that is why the students posted preferably in the ward performed better in MCQ and OSCE examinations.

Bradykinesia is one of the cardinal motor symptoms of Parkinson's disease. However, clinical and experimental studies indicate that bradykinesia may also be observed in various neurological diseases not primarily characterized by parkinsonism. These conditions include hyperkinetic movement disorders, such as dystonia, chorea, and essential tremor. Bradykinesia may also be observed in patients with neurological conditions that are not seen as "movement disorders", including those characterized by the involvement of the cerebellum and corticospinal system, dementia, multiple sclerosis, or psychiatric disorders...

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is associated with a range of clinical phenotypes and shows progressive degeneration of upper and/or lower motor neurons, and pTDP-43 inclusions in motor and non-motor pathways. Parkinsonian features have been reported in up to 30% of ALS patients and Lewy bodies, normally associated with Lewy body disorders (LBD), have been reported in a small number of ALS cases, with unknown clinical relevance. This study investigates the prevalence of clinically relevant LBD in a prospectively studied ALS cohort to determine if concomitant pathology contributes to the clinical heterogeneity...

BACKGROUND: There has been increasing attention on the epidemiology of rare diseases (RDs) in recent years. Rare neurological diseases (RNDs) constitute a significant proportion of RDs; however, relevant research is still lacking. METHODS: A list of ICD-10 codes corresponding to RNDs was compiled using adaptations from the Orphanet Classification of Rare Diseases, and classified into rare epilepsy, movement-related, neurocutaneous, neuroimmune, neurometabolic and neurodegenerative, neuromuscular and other RNDs...

Thank you for your interest and comments about our study "Headache at onset of first-ever ischemic stroke: Clinical characteristics and predictors" which was recently published in European Journal of Neurology. We would like to mention that our study did contain the data about stressful events before ischemic stroke. We took in consideration anxious symptomatology as associated factor for headache.

BACKGROUND: Both baseline prognostic factors and short-term predictors of treatment response can influence the long-term risk of disability accumulation in patients with relapsing-remitting multiple sclerosis (RRMS). OBJECTIVE: To develop and validate scoring system combining baseline prognostic factors and one-year variables of treatment response into a single numeric score predicting the long-term risk of disability. METHODS: We analyzed two independent datasets of patients with RRMS who started Interferon Beta or Glatiramer Acetate, had an Expanded Disability Status Scale (EDSS) score <4...

INTRODUCTION: Creutzfeldt-Jakob disease (CJD) is a rare form of rapidly progressive neurodegenerative disorder. Seizures are uncommon in the early stage of CJD, increasing diagnostic difficulty. We report an autopsy-proven case of CJD presenting initially as an epilepsia partialis continua (EPC), in which the initial workup was all unremarkable. Retrospectively, the presence of nystagmus, which proven to be non-epileptic, pointed to a cerebellar lesion before a diagnosis of clinically probable CJD was made...

BACKGROUND AND PURPOSE: Motoric cognitive risk syndrome (MCR) is a predementia syndrome characterized by cognitive complaints and slow gait. MCR is associated with increased risk of cognitive decline and incident dementia. Predictors of transition to dementia in MCR patients are still obscure. METHODS: We examined clinical, biological and lifestyle parameters related to conversion to dementia using Cox models in 439 older adults with prevalent MCR (mean age 79.87 ±8...