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-ic...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. In addition, studies in animal models of SUDEP have discovered that multiple neurotransmitters, including serotonin (5-HT) and adenosine, may be involved in the pathophysiological mechanisms of SUDEP and that these neurotransmitters could be the targets of future pharmacological intervention for SUDEP. The latest research findings on the epidemiology, clinical risk factors, and probable causes of SUDEP are presented in this review.
Most seizures in critical ill patients are non-convulsive, and some patients may develop non-convulsive status epilepticus (NCSE), a state of continuous or repetitive seizures without convulsions. With the growing use of...Most seizures in critical ill patients are non-convulsive, and some patients may develop non-convulsive status epilepticus (NCSE), a state of continuous or repetitive seizures without convulsions. With the growing use of continuous electroencephalogram (EEG) monitoring in neuro-intensive care units, non-convulsive seizure (NCS) and NCSE are increasingly diagnosed in patients with impaired consciousness, and progress has been made in identifying various EEG characteristics of NCS/NCSE. Epidemiological studies have contributed to a better understanding of etiologies and risk factors for NCS and NCSE. However, sufficient clinical trials about the treatment of NCS and NCSE are still lacking. The appropriate level of aggressiveness in the treatment of NCSE is still debated, particularly with regard to the use of anesthetics in patients with refractory NCSE. In this review, we summarize the EEG, clinical, epidemiological, diagnostic and therapeutic knowledge of NCS and NCSE in the neuro-intensive care setting in detail.
F-fluoro-deoxyglucose position emission tomography ( 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 t...F-fluoro-deoxyglucose position emission tomography ( 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. Specific patterns based on antibody subtypes and clinical symptoms were critical for identifying suspicious AE, the most common of which was the anteroposterior gradient in anti- N -methyl- d -aspartate receptor (NMDAR) encephalitis and the medial temporal lobe hypermetabolism in limbic encephalitis. And the dynamic changes of metabolic presentations in different phases provided us the potential to inspect the evolution of AE and predict the functional outcomes. Except for the visual assessment, quantitative analysis was recently reported in some voxel-based studies of regions of interest, which suggested some clues of the future evaluation of metabolic abnormalities. Large prospective studies need to be conducted controlling the time from symptom onset to examination with the same standard of FDG-PET scanning.
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...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. Secondly, seizure-related physiological activities involve the discharge of brain neurons presented, autonomous nervous activities, and motor. Plenty of studies focus on features from one signal, while it is a lack of evidences about the change of signal coupling along with seizures. Thirdly, the seizure detection algorithms developed from simple threshold method to complicated machine learning and deep learning, aiming at distinguish seizures from normal events. After understanding of some preliminary studies, we will propose our own thought for future development in this field.
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: Pa...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. Pearson correlation coefficients were used to explore the relationships between the walking measures and social participation, and step-wise multiple linear regression analysis was used to identify which walking measures would explain participation after stroke. RESULTS: Ninety-five chronic stroke individuals (38 men), with a mean age of 67 (SD 13) years were assessed. Significant positive correlations, of high magnitude, were found between participation and all walking measures (r ≥ .53; p < .001). Regarding the regression analysis, walking confidence alone explained 44% (F = 72.4; p < .001) of the variance in participation. When perceived locomotion ability was included in the model, the explained variance increased to 48% (F = 42.8; p < .001). CONCLUSION: All walking measures were correlated with social participation after stroke, but only perceived locomotion ability and walking confidence explained the variance in participation. Clinicians should be encouraged to evaluate real-life performance and personal factors that may limit community participation after stroke.
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 G...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. Clinical characteristics, laboratory data, and electrophysiology examinations were retrospectively collected. RESULTS: Seven of 94 (7.45%) GBS patients were positive for anti-NF155 antibody, and the main IgG subclass was IgG1. Compared with anti-NF155 antibody-negative GBS patients, anti-NF155 antibody-positive GBS patients had a higher GBS disability score at nadirs (p = .010), higher modified Erasmus GBS outcome score (p = .022), higher rate of abnormal compound motor action potential (CMAP) amplitude (p = .002), higher frequency of prolonged F-wave latency (p < .001), lower frequency of abnormal sensory conduction velocity (p < .001) and sensory nerve action potential amplitude (p < .001), more axonal type (p = .040), and poorer therapeutic effect (p = .017). CONCLUSIONS: Anti-NF155 antibody exists in a small portion of GBS patients. Anti-NF155 antibody-positive GBS patients possibly have a more severe clinical course, less sensory nerves involved, higher proportion of axonal type, poorer therapeutic effect, and worse prognosis, but the pathogenicity of the anti-NF155 antibody in GBS needs further study.
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 resear...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. In China, scientific research on genetic diagnosis and studies of precision therapy for genetic epilepsy are progressing rapidly. Many cases of epilepsy caused by mutation of potassium channel genes have been identified, and several potassium channel gene targets and drug candidates have been discovered. The purpose of this review is to briefly summarize the progress of research on the precise diagnosis and treatment of potassium ion channel-related genetic epilepsy, especially the research conducted in China. Here in, we review several large cohort studies on the genetic diagnosis of epilepsy in China in recent years, summarized the proportion of potassium channel genes. We focus on the progress of precison therapy on some hot epilepsy related potassium channel genes: KCNA1, KCNA2, KCNB1, KCNC1, KCND2, KCNQ2, KCNQ3, KCNMA1, and KCNT1.
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...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. So far, no exact virus in RE brain tissue or definite antigen in humoral immune system was confirmed as the determined etiology. The importance of cytotoxic CD8 T lymphocytes and activated microglial and the role of their immune mechanism in RE development are gradually emerging with the deep study. Genetic researches support the notion that the pathogenesis of RE is probably associated with single nucleotide polymorphisms on immune-related genes, which is driven by affecting inherent antiretroviral innate immunity. Recent advances in treatment suggest immunotherapy could partially slows down the progression of RE according to the histopathology and clinical presentation, which aimed at the initial damage to the brain by T cells and microglia in the early stage. However, the cerebral hemispherectomy is an effective means to controlling the intractable seizure, which is accompanied by neurological complications inevitably. So, the optimal timing for surgical intervention is still a challenge for RE patient. On the contrary, exploration on other aspects of pathogenesis such as dysfunction of adenosine system may offer a new therapeutic option for the treatment of RE in future.
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 m...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. Here, we historically review the advances of computerized applications in epilepsy-especially those tremendous findings achieved in China-for different purposes including seizure prediction, localization of epileptogenic zone, post-surgical prognosis, etc. Special attentions are paid to the great progress based on artificial intelligence (AI), which is more "sensitive", "smart" and "in-depth" than human capacities. At last, we give a comprehensive discussion about the disadvantages and limitations of current computerized applications for epilepsy and propose some future directions as further stepping stones to embrace "the era of AI" in epilepsy.
Acta Neurol Scand
· 2022 Oct · PMID 36156207
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Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease among adults. With diagnosis reached relatively late into the disease process, extensive motor cell loss narrows the window for therapeutic oppo...Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease among adults. With diagnosis reached relatively late into the disease process, extensive motor cell loss narrows the window for therapeutic opportunities. Clinical heterogeneity in ALS and the lack of disease-specific biomarkers have so far led to large-sized clinical trials with long follow-up needed to define clinical outcomes. In advanced ALS patients, there is presently limited scope to use imaging or invasive cerebrospinal fluid (CSF) collection as a source of disease biomarkers. The development of more patient-friendly and accessible blood biomarker assays is hampered by analytical hurdles like the matrix effect of blood components. However, blood also provides the opportunity to identify disease-specific adaptive changes of the stoichiometry and conformation of target proteins and the endogenous immunological response to low-abundance brain peptides, such as neurofilaments (Nf). Among those biomarkers under investigation in ALS, the change in concentration before or after diagnosis of Nf has been shown to aid prognostication and to allow the a priori stratification of ALS patients into smaller sized and clinically more homogeneous cohorts, supporting more affordable clinical trials. Here, we discuss the technical hurdles affecting reproducible and sensitive biomarker measurement in blood. We also summarize the state of the art of non-CSF biomarkers in the study of prognosis, disease progression, and treatment response. We will then address the potential as disease-specific biomarkers of the newly discovered cryptic peptides which are formed down-stream of TDP-43 loss of function, the hallmark of ALS pathobiology.
Acta Neurol Scand
· 2022 Oct · PMID 36156206
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Movement disorders have been carefully clinically defined, based on clinico-pathological series; however there is often diagnostic and prognostic uncertainty, especially in early stage disease. Blood-based biomarkers for...Movement disorders have been carefully clinically defined, based on clinico-pathological series; however there is often diagnostic and prognostic uncertainty, especially in early stage disease. Blood-based biomarkers for Alzheimer's disease (AD), particularly p-tau181 and p-tau217, may be useful in the movement disorder clinic, especially in identifying corticobasal syndrome due to AD pathology and in identifying Parkinson's disease (PD) patients at high risk for the future development of dementia. Serum or plasma neurofilament light (NfL) may be useful in separating Parkinson's plus syndromes (progressive supranuclear palsy-PSP, multiple system atrophy - MSA, and corticobasal syndrome-CBS) from PD. NfL is also a prognostic biomarker, in that the level of baseline or cross-sectional plasma/serum NfL is associated with a worse prognosis in PD and PSP. The development of protein aggregation assays in cerebrospinal fluid and multiplex assays which can measure 100 s-1000s of proteins in blood will provide new tools and insights for movement disorders for clinicians and researchers. The challenge is in efficiently integrating these tools into clinical practice and multi-modal approaches, where biomarkers are combined with clinical, genetic, and imaging data may guide the future use of these technologies.
Deep brain stimulation of the subthalamic nucleus (STN-DBS) is a promising palliative option for patients with refractory epilepsy. However, crucial questions remain unanswered: Which patients are the optimal candidates?...Deep brain stimulation of the subthalamic nucleus (STN-DBS) is a promising palliative option for patients with refractory epilepsy. However, crucial questions remain unanswered: Which patients are the optimal candidates? How, where, and when to stimulate the STN? And what is the mechanism of STN-DBS action on epilepsy? Thus, we reviewed the clinical evidence on the antiepileptic effects of STN-DBS and its possible mechanisms on drug-resistant epilepsy, its safety, and the factors influencing stimulation outcomes. This information may guide clinical decision-making. In addition, based on the current knowledge on the effect of STN-DBS on epilepsy, we suggest research that needs to be carried out in the future.
Acta Neurol Scand
· 2022 Nov · PMID 36121184
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The aim of this systematic review was to assess the effectiveness of Internet-based psychological interventions in the treatment of physical, socio-affective and cognitive symptoms and quality of life (QoL) in people wit...The aim of this systematic review was to assess the effectiveness of Internet-based psychological interventions in the treatment of physical, socio-affective and cognitive symptoms and quality of life (QoL) in people with multiple sclerosis (pwMS) to provide currently available evidence. Systematic searches for eligible studies were carried out in four databases (August 2021) using key words. Studies were screened, data extracted, quality appraised and analysed by three independent reviewers, using predefined criteria and following the PRISMA rules. Study quality was assessed using Standard Quality Assessment Criteria for Evaluating Primary Research Papers from a Variety of Fields QUALSYST tool. Physical, socio-affective and cognitive symptoms and QoL were the primary outcomes. Thirteen studies were included. Two principal approaches were reported: Cognitive behavioural therapy (CBT) and mindfulness-based interventions (MBI). Interventions varied from tailored versions to videoconference by a clinician, duration mean 8 weeks, delivered via individually and groups, all online. The review found that iCBT interventions were effective for improve depression, anxiety, fatigue and QoL, and slightly in cognitive functioning in pwMS, whereas MBI interventions reported benefits in depression, anxiety, stress and QoL, and less evidence in fatigue. Generally, study quality was acceptable in most studies; eleven of the studies scored a low risk of bias on all items in the Qualsyst Tool, whereas only two studies were considered unacceptable. Psychological online interventions may improve physical, socio-affective and cognitive symptoms as well as QoL in pwMS, overcoming the face-to-face barriers (i.e. disability). Contact with the therapist and groups sessions have been identified as enablers of the online interventions. Nevertheless, the limited number of studies and the heterogeneity of health outcomes reported made difficult to afford robust conclusions on psychological intervention effects in pwMS.
BACKGROUND: The purpose of this study is to investigate the reliability and validity as well as the clinical utility of the Silhouettes Fatigue Scale (SFS), a single-item visual scale to assess fatigue, in adult patients...BACKGROUND: The purpose of this study is to investigate the reliability and validity as well as the clinical utility of the Silhouettes Fatigue Scale (SFS), a single-item visual scale to assess fatigue, in adult patients with multiple sclerosis (MS). METHODS: The study included 61 MS patients and 73 matched healthy controls. Demographic data and disease-related variables of all participants were recorded. Then, the SFS, Visual Analogue Scale (VAS)-fatigue, Fatigue Impact Scale (FIS), Fatigue Severity Scale (FSS), and Beck Depression Inventory (BDI) were applied. SFS, VAS-fatigue, and FSS were repeated after one week. Reliability was evaluated with the intra-class correlation coefficient (ICC) and Bland-Altman analysis. Validity was tested by comparison of healthy controls and patients with MS and correlations with other scales. Accuracy and clinical utility were also evaluated. RESULTS: SFS scores were 4.49 ± 3.11 (mean ± SD) in MS patients and 1.40 ± 1.44 in healthy controls (p < .001). The ICC for SFS was 0.946. The mean difference between test-retest measurements of SFS was -0.04651 (-95% CI, -0.4815-0.38848), and there was no systemic bias. SFS scores were not correlated with the expanded disability status scale, whereas they were poorly correlated with BDI. Correlations ranging from poor to good were calculated between the SFS and other fatigue-related scales. The optimum cut-off score of the SFS scale was four, with a sensitivity of 0.72 and a specificity of 0.84. CONCLUSION: This study demonstrated that the SFS is a reliable, responsive, and valid scale with acceptable sensitivity and specificity to assess and quantify clinically significant fatigue in MS patients. These findings as well as the brief and understandable nature of the SFS were encouraging that this scale has good clinical utility.
OBJECTIVE: We previously investigated the preclinical state of idiopathic normal pressure hydrocephalus (iNPH): asymptomatic ventriculomegaly with features of iNPH on magnetic resonance imaging (AVIM) found in community...OBJECTIVE: We previously investigated the preclinical state of idiopathic normal pressure hydrocephalus (iNPH): asymptomatic ventriculomegaly with features of iNPH on magnetic resonance imaging (AVIM) found in community inhabitants. The aim of the study was to determine how iNPH develops longitudinally. MATERIALS AND METHODS: A previous longitudinal prospective community-based cohort study was initiated in 2000. The 271 70 year-old participants were followed up in 2016 at the age of 86 years. At this time, 104 participants could be reached for clinical examinations and brain magnetic resonance imaging (MRI). iNPH in this study was diagnosed if the participant had more than one symptom in the clinical triad and disproportionately enlarged subarachnoid space hydrocephalus (DESH) on MRI, fulfilling at least an Evans index >0.3 (ventricular enlargement, VE) and a narrowing of the subarachnoid space at the high convexity (tight high convexity, THC). Asymptomatic VE (AVE) plus THC were considered AVIM. RESULTS: Longitudinally throughout 16 years, 11 patients with iNPH were found. The hospital consultation rate was only 9%. Five of the eight patients with AVIM (62.5%) and six of 30 with AVE (20.0%) developed iNPH. Cross-sectionally, eight patients had iNPH (8/104, 7.7% prevalence at the age of 86) in 2016. Disease development was classified into THC-preceding and VE-preceding iNPH. One VE-preceding iNPH case was considered a comorbidity of Alzheimer's dementia. CONCLUSION: Idiopathic normal pressure hydrocephalus had a high prevalence among octogenarians in the evaluated community. iNPH developed not only via AVIM but also via AVE, the latter was also frequent in the elderly.
OBJECTIVES: To describe F-fluorodeoxyglucose positron emission tomography/magnetic resonance imaging ( F-FDG PET/MRI) along with semiology and electroencephalography (EEG) in patients with gray matter heterotopia (GMH);...OBJECTIVES: To describe F-fluorodeoxyglucose positron emission tomography/magnetic resonance imaging ( F-FDG PET/MRI) along with semiology and electroencephalography (EEG) in patients with gray matter heterotopia (GMH); to evaluate the concordance between 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 F-FDG PET/MRI were retrospectively enrolled. Two radiologists evaluated brain MRI, while two nuclear medicine specialists assessed the F-FDG PET. The SUVmax values of visually hypometabolic cortical areas were compared to the contralateral cortex using a SUVmax threshold value of 10%; the SUVmax values of GMH lesions were compared with that of the right precentral gyrus. The cortex or GMH with hypometabolism on F-FDG PET/MRI was considered representative of the EZ. The clinical EZ was identified using EEG and semiology. RESULTS: Thirty patients (19 PNH; 11 SCH) with a mean age of 28.46 ± 9.52 years were enrolled. The heterotopic nodules were ametabolic in 3 patients (10%), hypometabolic in 16 (33.33%), isometabolic in 13 (26.66%), and hypermetabolic in 4 (10%). F-FDG PET/MRI demonstrated hypometabolism in the cortex and GMH in 22/30 (73.33%) and 16/30 (53.33%). We could identify a clinical EZ in 18 patients, and 15 out of 18 (83.33%) had concordant F-FDG PET/MRI findings. CONCLUSION: Heterotopic nodules in GMH patients show different metabolic patterns on F-FDG PET/MRI, with nearly three-quarters of the patients having cortical hypometabolism. F-FDG PET/ MRI findings are mostly concordant with the clinical EZ.