Acta Neurologica Scandinavica

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Sudden unexpected death in epilepsy (SUDEP) is the major cause of premature death in epilepsy patients, particularly those with refractory epilepsy. Sudden unexpected death in epilepsy is thought to be related to peri-ictal cardiac dysfunction, respiratory depression, and autonomic dysfunction, albeit the exact etiology is unknown. Sudden unexpected death in epilepsy prevention remains a huge challenge. The sole presence and frequency of generalized tonic-clonic seizures (GTCS) are the most important risk factors for SUDEP, and nocturnal monitoring may lower the risk with the use of remote listening devices...

18 F-fluoro-deoxyglucose position emission tomography (18 F-FDG-PET) has been proven as a sensitive and reliable tool for diagnosis of autoimmune encephalitis (AE). More attention was paid to this kind of imaging because of the shortage of MRI, EEG, and CSF findings. FDG-PET has been assessed in a few small studies and case reports showing apparent abnormalities in cases where MRI does not. Here, we summarized the patterns (specific or not) in AE with different antibodies detected and the clinical outlook for the wide application of FDG-PET considering some limitations...

With sudden and unpredictable nature, seizures lead to great risk of the secondary damage, status epilepticus, and sudden unexpected death in epilepsy. Thus, it is essential to use a wearable device to detect seizure and inform patients' caregivers for assistant to prevent or relieve adverse consequence. In this review, we gave an account of the current state of the field of seizure detection based on wearable devices from three parts: devices, physiological activities, and algorithms. Firstly, seizure monitoring devices available in the market primarily involve wristband-type devices, patch-type devices, and armband-type devices, which are able to detect motor seizures, focal autonomic seizures, or absence seizures...

With the development and application of next-generation sequencing technology, the aetiological diagnosis of genetic epilepsy is rapidly becoming easier and less expensive. Additionally, there is a growing body of research into precision therapy based on genetic diagnosis. The numerous genes in the potassium ion channel family constitute the largest family of ion channels: this family is divided into different subtypes. Potassium ion channels play a crucial role in the electrical activity of neurons and are directly involved in the mechanism of epileptic seizures...

Rasmussen's encephalitis (RE) is a rare condition of unknown etiology that causes a severe chronically neurological disorder with mostly affecting children. The main clinical feature of RE includes frequent seizures with drug-resistant, unilateral hemispheric atrophy, and progressive neurological deficits. In this review, we summarized five pathogenesis on the basis of the current research including virus infection, antibody-mediated degeneration, cell-mediated immunity, microglia-induced degeneration, and genetic mutations...

Epilepsy, one of the most common neurological diseases in China, is notorious for its spontaneous, unprovoked and recurrent seizures. The etiology of epilepsy varies among individual patients, including congenital gene mutation, traumatic injury, infections, etc. This heterogeneity partly hampered the accurate diagnosis and choice of appropriate treatments. Encouragingly, great achievements have been achieved in computational science, making it become a key player in medical fields gradually and bringing new hope for rapid and accurate diagnosis as well as targeted therapies in epilepsy...

Sleep disorder is common in epilepsy. With a recent rapid development in sleep medicine, it has been increasingly recognized that anti-seizure therapies, either anti-seizure medications (ASMs) or non-pharmaceutical approaches, can take direct or indirect influence on sleep in patients with epilepsy. Here, we systematically review the effect of anti-seizure treatments on sleep. ASMs targeting at different sites exerted various effects on both sleep structure and sleep quality. Non-pharmaceutical treatments including resective surgery, ketogenic diet, and transcranial magnetic stimulation appear to have a positive effect on sleep, while vagus nerve stimulation, deep brain stimulation, and brain-responsive neurostimulation are likely to interrupt sleep and exacerbate sleep-disordered breathing...

OBJECTIVE: To explore the relationship between walking measurements (i.e., walking speed, walking performance and walking confidence), and participation in ambulatory people with chronic stroke. MATERIALS AND METHODS: Participation was measured using the mobility domain of Brazilian version of the Stroke Impact Scale 3.0. Walking measures were walking speed, assessed by 10-m Walk Test, walking performance, assessed by ABILOCO, walking confidence, assessed by mGES...

OBJECTIVE: Anti-neurofascin 155 (NF155) antibody has been discovered in chronic demyelinating conditions. However, the positive rate and clinical description were insufficient in acute demyelinating conditions, such as Guillain-Barré syndrome (GBS). This study aimed to explore the positive rate of anti-NF155 antibody in GBS patients and determine whether there were unique clinical characteristics in these patients. MATERIALS & METHODS: Serum anti-NF155 antibody was detected from 94 GBS patients and 50 sex- and age-matched healthy controls using cell-based assay and tissue-based assay with immunostaining of mouse teased sciatic nerve fibers...

OBJECTIVES: To describe 18 F-fluorodeoxyglucose positron emission tomography/magnetic resonance imaging (18 F-FDG PET/MRI) along with semiology and electroencephalography (EEG) in patients with gray matter heterotopia (GMH); to evaluate the concordance between 18 F-FDG PET/MRI and clinical epileptogenic zone (EZ). MATERIALS & METHODS: GMH (subcortical heterotopia [SCH] and periventricular nodular heterotopia [PNH]) patients with epilepsy who underwent 18 F-FDG PET/MRI were retrospectively enrolled...

OBJECTIVES: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a cerebral small vessel disease caused by pathogenic variants in the NOTCH3 gene. In Finland, the majority of CADASIL patients carry the pathogenic founder variant c.397C>T, (p.Arg133Cys), but the spectrum of other NOTCH3 variants has not been investigated previously. The aim of the study was to investigate the spectrum and prevalence of NOTCH3 variants Finnish CADASIL patients and to examine the clinical features associated with them...

BACKGROUND: Robust collateral circulation is strongly associated with good outcomes in acute ischemic stroke (AIS). AIMS: To determine whether collateral circulation detected by arterial spin labeling (ASL) magnetic resonance imaging could predict good clinical outcome in AIS patients with 90 days follow-up. MATERIALS AND METHODS: Total 58 AIS patients with anterior circulation stroke were recruited. Collateral circulation was defined as arterial transit artifact in ASL images...

Ocrelizumab and siponimod are the two on-label drugs used for progressive forms of multiple sclerosis (PMS). However, many patients with PMS do not have access to these high-efficacy disease-modifying drugs (DMDs). Off-label prescription of other high-efficacy DMDs (fingolimod, rituximab and natalizumab) may be a strategy to improve access to immunotherapy for these patients. We aim to compare on-label and off-label high-efficacy drugs for their effect on disability progression in PMS. In December 2021, we searched MEDLINE (PubMed), Embase, Cochrane Central and Scopus databases for randomized clinical trials involving patients with PMS...

OBJECTIVES: Clobazam is a well-known benzodiazepine used as an anti-anxiety drug as well as an anti-epileptic, particularly for patients who are not responding to first-line treatments. Recent case reports have indicated the association of clobazam with drug reaction with eosinophilia systemic symptoms syndrome (DRESS Syndrome). However, DRESS syndrome is not known to be associated with clobazam. Thus, the main objective of the current study was to identify the potential signal of clobazam-associated DRESS Syndrome...

Vestibular neuritis is a common neuro-otological entity. Therapeutically, corticosteroids are advised, although the evidence is limited. The objective of this review is to update meta-analyses of clinical trials that address the question of whether patients with vestibular neuritis treated with corticosteroids show better recovery than control patients. The electronic databases Medline, Scopus and Cochrane were searched for clinical trials for the years 1970-2020 without language restriction. Data were extracted, and outcome parameters were subjected to conventional and cumulative meta-analysis using a commercially available software program (www...

OBJECTIVES: Endovascular treatment (EVT) is the gold standard treatment for emergent large vessel occlusion (LVO). The benefit of EVT for emergent LVO in elderly patients (>80 years old) is still debated as they have been under-represented in randomized controlled trials. Elderly patients with an emergent LVO are a growing population warranting further study. MATERIALS & METHODS: We included 225 consecutive patients treated with EVT for LVO either in the anterior or posterior circulation...

OBJECTIVES: To investigate control of epileptic seizures during pairs of successive pregnancies in antiseizure medication (ASM)-treated women with epilepsy. MATERIALS AND METHODS: Analysis of seizure freedom rates during 436 pairs of successive pregnancies in Australian women with epilepsy, in nearly all instances long-standing epilepsy. RESULT: There was a higher rate of seizure-free second pregnancies compared with first paired pregnancies (63...

BACKGROUND: We aimed to describe baseline characteristics of patients with oral anticoagulant-related intracerebral hemorrhage (OAC-ICH) in Sweden and to identify predictive variables associated with receiving hemostatic treatment in the event of OAC-ICH. METHODS: We performed an observational study based on data from Riksstroke and the Swedish Causes of Death Register to define baseline characteristics of patients with OAC-ICH who received reversal treatment compared with patients who did not receive reversal treatment during 2017-2019...

BACKGROUND: Non-REM sleep symptoms remain poorly understood in alpha-synucleinopathies. AIMS: The aims of the study were to compare sleep stability and transitions, arousals, and sleep cycle structure between isolated rapid eye movement (REM) sleep behavior disorder (iRBD), Parkinson's disease (PD), and dementia with Lewy Bodies (DLB). MATERIALS AND METHODS: Sleep transition and stability measures were assessed in one-night video-polysomnography records...