European Journal of Neurology

Recently in the journal, epidemiological data are presented on visual snow syndrome (VSS) using a crowdsourcing online platform [1]. The authors applied criteria for VSS to a sample of people matched for age, sex, and ethnicity to United Kingdom census data. Further, they assessed frequent headache lasting longer than 4 hours, which was interpreted as migraine, and neurological symptoms during headache, interpreted as aura.

Progressive multifocal leukoencephalopathy (PML) is a dreaded and potentially lethal opportunistic viral complication of a variety of immune deficiency states. While the causative JC virus (JCV) is present in a majority of adults worldwide, the prevalence of this condition remains rare, making it challenging to study. The cases described by Grassl et al and Goreci et al in this issue of EJN ( refs) typify the dreaded subacute progression and death associated with this virus. These cases add to the case report literature exploring the potential of checkpoint inhibitors as therapy for PML...

A 71-year-old Caucasian man presented with dysarthria and fluctuating hypesthesia of the right upper limb in early 2019. Brain MRI demonstrated T2/FLAIR hyperintense lesions in the left parietal cortical grey matter and adjacent white matter compatible with embolic stroke of undetermined source. Eight weeks later symptoms had further progressed with loss of adequate communication, disturbance of fine motor skills, ataxia and neuropsychiatric symptoms. Widespread disease on brain MRI and the detection of JC polyomavirus (JCPyV) DNA from cerebrospinal fluid (CSF) confirmed the diagnosis of progressive multifocal leukoencephalopathy (PML)...

Progressive multifocal leukoencephalopathy (PML) is a rare but severe opportunistic CNS infection caused by JC polyomavirus in immunosuppressed patients. Immune checkpoint blockade stimulates immune function and has been suggested to reinvigorate viral clearance in PML patients by expanding JCV-specific T cells1 . Among the first 13 published PML patients treated with programmed cell death 1 inhibitors (PD1I) 8 showed a mild to marked treatment response2-7 . The underlying conditions ranged from chronic lymphocytic leukemia, AIDS, Non-Hodgkin lymphoma, idiopathic lymphopenia, variable immune deficiency, Hodgkin lymphoma, B-cell lymphoma to primary and combined immunodeficiency...

BACKGROUND: Migraine is a common and costly neurological disorder. The aim of this study was to quantify the costs of chronic (CM) and episodic migraine (EM) in Spain, evaluating the impact of psychiatric comorbidities and disability, and estimate the economic savings of reducing the number of migraine days by 50%. METHODS: This was an observational, cross-sectional analysis of data from migraine patients who participated in the Spanish Migraine Atlas. The participants were invited to complete a structured questionnaire including the following scales: Headache Needs Assessment (HANA), Hospital Anxiety and Depression Scale (HADs), and Migraine Disability Assessment Scale (MIDAS)...

BACKGROUND AND PURPOSE: The patient's perspective is becoming increasingly important for endpoints in studies about multiple sclerosis. However, relapse data generated from the patient's perspective in combination with an independent documentation by the physician are scarce. Our objective was to compare self-reported relapses by the patient to physician documented relapses within a routine clinical practice setting of quarterly visits. METHODS: Two-year data (n=1921 patients) were extracted from two prospective, non-interventional, multicenter cohort studies in Germany...

BACKGROUND: The existence of contraindications to intravenous thrombolysis (IVT) is considered a criterion for direct transfer of patients with suspected acute stroke to thrombectomy-capable centers (EVT-C) in the prehospital setting. Our aim was to assess the utility of this criterion in a setting where routing protocols are defined by the Madrid-DIRECT prehospital scale. METHODS: Post-hoc analysis of the M-DIRECT study. Reported contraindications to IVT were retrospectively collected from EMS reports and categorized into late window, anticoagulant treatment and other contraindications...

BACKGROUND: Post-stroke fatigue (PSF) is a common neuropsychiatric affective symptom occurring after stroke. Evidence indicates the activated inflammatory pathways are involved in modulating the stroke and fatigue. High-sensitivity C-reactive protein (hs-CRP) is one of the most sensitive indicator of inflammation. Our aim was to estimate the association between plasma hs-CRP and PSF after acute ischemic stroke (AIS). METHODS: 212 AIS patients were consecutively recruited within the first 14 days after stroke onset and followed up for 6 months...

The interaction between DOACs and antiepileptic drugs is an issue commonly encountered in neurological practice. Due to quite frequent occurrence of post-stroke epilepsy (PSE), neurologists may often prescribe both DOACs and antiepileptics in the same patient. The 2018 European Society of Cardiology (ESC) guidelines provided strong restrictions on the concomitant use of DOACs and some antiepileptics.

We read with interest the paper "SOD1 p.D12Y variant is associated with ALS/distal myopathy spectrum", recently published by Tasca et al., where four patients heterozygous for the p.D12Y variant in SOD1 were described. Two of them had a classical ALS phenotype. The other two (patient 1 and patient 4, previously reported in) had, according to the authors, a "concomitant distal myopathy". The authors rely mainly on histologic findings to suggest concomitant involvement of skeletal muscle. However, in our opinion, these changes, including the presence of rimmed vacuoles, cytoplasmic bodies and small foci of necrosis and phagocytosis are not specific of a myopathic process and can be found in slowly progressive motor neuron disorders, as a result of long-standing denervation and reinnervation...

INTRODUCTION: Limited data are available in literature for upper limb (UL) impairment in multiple sclerosis (MS). This study aims to report the distribution of values of Hand Grip Strength (HGS), of Box-and-Block Test (BBT) and of 9-Hole Peg Test (9HPT), correlated with demographic and clinical data in subjects with MS. MATERIALS AND METHODS: This study involved 5 Italian neurological centers. INCLUSION CRITERIA: age ≥18, MS diagnosis, stable disease phase, right hand dominance...

We thank Dr de Fuenmayor-Fernández de la Hoz and colleagues for their interest in our paper and their comments. Clearly distinguishing neurogenic from myogenic disease processes is not always straightforward and we fully share the view that, to some extent, myopathic alterations on muscle biopsy may come along with long-standing denervation, thus configuring a so-called "secondary myopathy". This could in fact be the major contributing factor to explain the imaging and pathology features of patient 4 in our paper...

BACKGROUND: Advance care planning allows people to plan for their future care needs and can include medical, psychological and social aspects. However, little is known on the use, experience of and attitudes towards advance care planning in patients with Parkinsonian disorders, their family carers and in health care professionals. METHODS: We conducted a systematic search of online databases in April 2019 using a narrative synthesis approach with thematic analysis and tabulation to synthesise the findings...

BACKGROUND AND PURPOSE: The frequency of infectious encephalitis and the distribution of causative pathogens in the tropical areas are poorly known and may be influenced by emerging and rare infections. The aim was to characterize a large series of acute infectious encephalitis and myelitis in immunocompetent patients from the Caribbean island of Guadeloupe identifying clinical, biological and radiological features according to pathogens. METHODS: Using a hospital database, we retrospectively collected detailed information on a comprehensive series of immunocompetent patients with acute infectious myelitis and encephalitis over the 2012-2018 period...

BACKGROUND: Both, optical coherence tomography (OCT) and MRI volumetric measures have been postulated as potential biomarkers of MS related disability. OBJECTIVE: To investigate the association between OCT and brain volume (BV) and spinal cord area (SCA) parameters in relapsing MS patients, and to assess their independent associations with disability. METHODS: Cross-sectional analysis of 90 MS patients that underwent OCT and MRI exams. Values of peripapillary retinal nerve fibre layer (pRNFL), ganglion cell-inner plexiform layer (GCIPL), and inner nuclear layer (INL) of eyes without previous ON were obtained...

BACKGROUND: Different amount of cumulative exposure to the toxic mutant form of the huntingtin protein might underlie distinctive pattern of striatal connectivity in premanifest Huntington's disease (pre-HD). Aim of this study is to investigate disease burden-dependent cortical- and subcortical-striatal loops in different pre-HD stages. METHODS: Sixteen pre-HD participants and 25 controls underwent magnetic resonance exam to investigate striatal structural and functional connectivity...

OBJECTIVE: Most recurrent cervical artery dissection (CeAD) events occur shortly after the acute first CeAD. This study compared characteristics of recurrent and first CeAD events and searched for associations between subsequent events of an individual person. METHODS: CeAD patients with a new CeAD event occuring during a three to six months follow-up were retrospectively selected in seven specialized stroke centers. Clinical and vascular characteristics of the initial and the recurrent CeADs were compared...

BACKGROUND AND PURPOSE: Clinical trials (CTs) aimed at vulnerable groups such as patients with mental disorders create ethical complexity. The Patient Information Sheet (PIS) should provide all the information about the CT that is relevant to the subject's decision to participate. After being informed, the subject will decide freely whether to take part in the CT and will read and sign the Informed Consent Form (ICF). The objective was to assess the quality of PIS/ICFs from a Hospital Neurology Service (NS)...

BACKGROUND: Soluble ST2 (sST2) is a promising biomarker in inflammation, atherosclerosis, and cardiovascular diseases. We investigated the association between serum sST2 and poor outcome in patients with transient ischaemic attack (TIA)/ischaemic stroke. METHODS: Patients within 24 h after onset and with measured serum sST2 were prospectively enrolled in this study. Poor outcome was a combination of a new stroke event (ischaemic or haemorrhagic) and all-cause death within 90 days and 1 year...

BACKGROUND: Clinical use of continuous electrocardiogram (cECG) for detecting atrial fibrillation (AF) after stroke is unclear. In a Danish nationwide cohort we described post-stroke time trends in outpatient cECG usage and atrial fibrillation incidences and characterized factors associated with cECG use. METHODS: Patients without AF discharged after their first ischemic stroke between 2010 and 2016 were identified from Danish nationwide registries. cECG included Holter or event recording within 120 days from discharge...