European Journal of Neurology

BACKGROUND: To evaluate the quality of smartphone videos (SVs) in adult epilepsy outpatients. METHODS: A prospective, multicenter cohort study evaluated SV quality in patients with paroxysmal neurologic events from 8/15/2015 through 8/31/2018. Epileptic seizures (ES), psychogenic nonepileptic attacks (PNEA), and physiologic nonepileptic events were confirmed by video-EEG monitoring. Experts and senior neurology residents blindly viewed cloud-based SVs without clinical information...

We here present the case of a patient with a sleep disturbance attributed to the exploding head syndrome, recently redefined as 'episodic cranial sensory shock'. The patient, who suffered with concomitant migraine, was treated for headache prevention with daily single-pulse transcranial magnetic stimulation. Following treatment, he reported a significant reduction in the episodes of exploding head syndrome, albeit not of his migraine. Neurologists could consider sTMS in the management of patients troubled by episodic cranial sensory shock, as it is a safe and non-invasive treatment that might provide benefit for this benign but occasionally bothersome parasomnia...

OBJECTIVE: To investigate the perceptions, the feelings and the unmet needs of Parkinson's Disease (PD) patients who experienced a two-month lockdown in a "red zone" in the northern part of Italy during the COVID-19 outbreak. METHODS: A descriptive design utilising cross-section online survey with open-ended questions enquiring the person's feelings concerning the risk of contracting Coronavirus, how their physical activity has changed, the needs related to Parkinson's disease which have not been meet during the pandemic period, their personal needs dictated by their condition, which had not been met in this pandemic period as compared to previous periods...

BACKGROUND: Cerebral amyloid angiopathy (CAA) is a frequent cause of both intracerebral hemorrhage (ICH) and cognitive impairment in the elderly. Diagnosis rely on the Boston criteria, which use MRI markers including ≥2 exclusively lobar cerebral microbleeds (lCMBs). Although amyloid PET may provide molecular diagnosis, its specificity relative to Alzheimer's disease (AD) is limited due to the prevalence of positive amyloid PET in cognitively normal elderly. Using early-phase 11 C-PiB as surrogate for tissue perfusion, a significantly lower occipital/posterior cingulate (O/PC) tracer uptake ratio in probable CAA relative to AD was recently reported, consistent with histopathological lesion distribution...

Coronavirus disease 2019 (COVID-19), a multi-organ disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), continues to challenge health and care systems around the globe. The pandemic has disrupted acute neurology services and routine patient care and has impacted the clinical course in patients with chronic neurologic disease. COVID-19 appears to have exposed inequalities of societies and healthcare systems and had disproportionate impact on already vulnerable communities. The next challenge will be to set up initiatives to stop disparities in all aspects related to COVID-19...

OBJECTIVE: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a clinical and electrophysiological heterogeneous immune-mediated polyneuropathy. Intravenous immunoglobulin (IVIg), corticosteroids and plasma exchange are proven effective treatments for CIDP. The clinical response to IVIg is variable between patients and currently unexplained. Finding biomarkers related to treatment response can help to understand the diversity of CIDP and personalise treatment choice. METHODS: We investigated whether genetic variation between patients may explain some of these differences in treatment response...

With great interest we read the article "Progression of fatigue in Parkinson's disease - a 9-year follow-up" by Ongre et al.(1). The authors compared the course of fatigue in subjects with Parkinson's disease (PD) and a healthy control group identifying higher fatigue rates in PD patients, along with several factors associated with fatigue.

I read with great interest the article by Ohara et al. [1] in which they found that progressive supranuclear palsy (PSP) patients develop idiopathic normal-pressure hydrocephalus (iNPH)-like MRI features, more than other neurodegenerative diseases. I appreciate the authors for conducting such an original study; however, I would like to make some comments for a better understanding of the report and present some crucial perspectives.

We read with interest the original article by De Giuli et al. on the cardiac sources of embolism associated with a history of migraine in stroke patients (1). While patent foramen ovale was significantly more frequent in patients with migraine with aura (MA), no association was detected between atrial fibrillation (AF) and any type of migraine.

BACKGROUND: Central retinal artery occlusion (CRAO) is a neuro-opthalmological emergency necessitating adequate and comprehensive diagnosis. Its optimal management and treatment, however, are still under debate. METHODS: In order to identify areas of improvement, we retrospectively analysed medical records of patients with CRAO treated on our stroke unit between January 2016 and August 2020. RESULTS: During the observational period, 101 patients with CRAO were admitted...

OBJECTIVE: Observational study to evaluate long-term effects of deep brain stimulation (DBS) of the globus pallidus internus (GPi) and the ventral intermediate thalamic nucleus (VIM) on patients with medically refractory myoclonus dystonia (MD). BACKGROUND: More recently, pallidal as well as thalamic DBS have been applied successfully in MD but long-term data are sparse. METHODS: We retrospectively analyzed a cohort of 7 MD patients with either separate (n=1, VIM) or combined GPi- and VIM-DBS (n=6)...

In this issue of the Journal, Frasquet et al. report on the genetic spectrum of a large cohort of patients with distal hereditary motor neuropathy (dHMN) from two large centres in Spain1 . All patients underwent detailed clinical assessment by experts in neuromuscular disease and are likely to have an accurate diagnosis. As the centre in Valencia provides all neuromuscular services for a defined geographical area, these two factors have allowed the authors to calculate a population prevalence for dHMN.

BACKGROUND: Parkinson´s disease (PD) has a large phenotypic variability, which may, at least partly, be genetically driven including alterations of gene products. Candidates might not only be proteins associated with disease risk but also pathways that play a role in aging. OBJECTIVE: To evaluate phenotype modifying effects of genetic variants in Klotho, a longevity gene. METHODS: We analysed two longitudinal cohorts. One local cohort comprising 459 PD patients who underwent genotyping for the KL-VS haplotype in Klotho including a subgroup of 125 PD patients and 50 healthy controls who underwent biochemical CSF analyses of Klotho and fibroblast growth factor 23 as well as Vitamin-D-metabolites...

BACKGROUND: It is unclear which cognitive outcome measure is the most useful for clinical trials in multiple sclerosis. To investigate the usefulness of the symbol digit modalities test (SDMT) as a clinical outcome measure in secondary progressive multiple sclerosis (SPMS), we describe the frequency of worsening and improvement events in a large randomized controlled trial (RCT) dataset. METHODS: Using original trial data from the ASCEND trial (n=889), a recent large RCT in SPMS, we describe worsening and similarly defined improvement with and without three month confirmation on the SDMT in the whole trial cohort and unconfirmed worsening and improvement on the Paced Auditory Serial Addition Test (PASAT) in a smaller subset (n=107)...

BACKGROUND: The transition from episodic migraine to chronic migraine, migraine chronification, is usually a gradual process, which involves multiple risk factors. To date, studies of the genetic risk factors for chronic migraine have focused primarily on candidate gene approaches using healthy individuals as controls. AIMS/METHODS: In this study, we use a large cohort of migraine families and unrelated migraine patients (n>2200) with supporting genotype and whole genome sequencing data...

BACKGROUND AND PURPOSE: The potentially increased risk of extrathymic cancers in MG remains uncertain. We present the occurrence of extrathymic cancer diagnoses in different MG subgroups. METHODS: A nationwide Swedish register-based cohort study included patients who had their first MG diagnosis or first prescription of acetylcholine esterase inhibitors between the years 2006 and 2018. Timing and subtypes of cancer diagnosis in relation to MG as well as corticosteroid sparing immunosuppressants (CSIS) were identified from national patient-, cancer- and drug registers...

BACKGROUND: The clinico-radiological paradox in MS is well recognised, relevant and yet poorly understood. We tested the suitability of an in vivo model for the clinico-radiological paradox, using internuclear ophthalmoplegia (INO) and the medial longitudinal fasciculus (MLF). METHOD: In this cross-sectional study lesions of the MLF were rated by an experienced MS neuroradiologist blinded to all other information. Presence of an INO was objectively determined by a validated infrared oculography protocol (DEMoNS)...

BACKGROUND: Strains of Streptococcus mutans expressing the cell surface collagen-binding protein, Cnm, encoded by cnm (cnm-positive S. mutans), are associated with hypertensive intracerebral hemorrhage (ICH) and the occurrence of cerebral microbleeds (CMBs). Small diffusion-weighted imaging (DWI) hyperintensities in patients with acute ICH are also associated with CMBs. However, the association between cnm-positive S. mutans and DWI hyperintensities is unclear. Hence, this study aimed to investigate the association between cnm-positive S...

BACKGROUND: Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection predisposes patients to arterial and venous thrombosis. This study aimed to systematically review the available evidence in the literature for cerebral venous thrombosis (CVT) in association with coronavirus disease-2019 (COVID-19). METHODS: We searched MEDLINE, EMBASE and Cochrane CENTRAL databases to identify cases of COVID-19-associated CVT. The search period spanned January 1st to December 1st 2020 and the review protocol (PROSPERO-CRD42020214327) followed PRISMA guidelines...

BACKGROUND: Functional neurological disorders (FND) represent a significant proportion of presentations to outpatient adult neurology services. There is little information relating to patients presenting to acute inpatient care. METHODS: We identified patients presenting as acute admissions with FND to Christchurch hospital, Christchurch, New Zealand, from 2016 to 2018. We analysed relevant demographic and clinical data from electronic records and measured incidence of presentation to secondary care and healthcare utilisation...