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Frontiers in Immunology 2023Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) has gained recognition in recent years as an immune-mediated inflammatory demyelinating disease...
BACKGROUND AND PURPOSE
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) has gained recognition in recent years as an immune-mediated inflammatory demyelinating disease of the central nervous system. The clinical features and prognosis of MOGAD adult cerebral cortical encephalitis (adult CCE) have not been fully elucidated. This study aims to further characterize the clinical symptoms, magnetic resonance imaging (MRI) findings, and prognosis of CCE with anti-MOG antibody.
METHODS
We present two adult cases of CCE with anti-MOG antibody and summarize the clinical symptoms, magnetic resonance imaging (MRI) findings, and prognosis of this phenotype as per a completed systematic review of the literature.
RESULTS
We found a total of 39 cases of MOGAD adult CCE (36% females; average age of onset of 29 years). Among them, 85% had seizure, 82% had headache, 64% had cortical symptoms, 64% had fever, 54% had changes of consciousness, and 38% had ocular symptoms. All cases demonstrated cerebral cortical T2 fluid-attenuated inversion recovery (FLAIR) lesions on MRI. Of the 25 patients (with seizure or not) who had EEG reports, 76% of patients showed abnormal EEG. Cerebrospinal fluid (CSF) white blood cell count of 90% of patients and CSF total protein of 67% of patients were elevated. In 16 patients with available CSF cytology data, 11 (69%) had abnormal cytology findings with monocytic predominance. In the 15 cases for which MOG antibody IgG was tested in both serum and CSF, 14 (93%) demonstrated a higher positive MOG IgG titer in serum than CSF. The majority of patients were treated with immunosuppressive therapy (97% corticosteroids, 15% mycophenolate mofetil, 13% IVIg, 5% azathioprine, and 5% other). The majority of patients had a favorable prognosis after treatment, as exemplified by improved clinical symptoms and imaging. Two patients relapsed.
CONCLUSIONS
The clinical presentation and prognosis of adult CCE remain less understood in comparison to more common MOGAD phenotypes. It is important to consider MOGAD as an underlying etiology for adult CCE, as early detection and immunotherapy may improve outcomes.
Topics: Female; Male; Humans; Myelin-Oligodendrocyte Glycoprotein; Autoantibodies; Encephalitis; Seizures; Immunoglobulin G; Oligodendroglia
PubMed: 37520572
DOI: 10.3389/fimmu.2023.1203615 -
Frontiers in Behavioral Neuroscience 2024Neurexins, essential synaptic proteins, are linked to neurodevelopmental and neuropsychiatric disorders like autism spectrum disorder (ASD) and schizophrenia.
BACKGROUND
Neurexins, essential synaptic proteins, are linked to neurodevelopmental and neuropsychiatric disorders like autism spectrum disorder (ASD) and schizophrenia.
OBJECTIVE
Through this systematic review, we aimed to shed light on the relationship between neurexin dysfunction and its implications in neurodevelopmental and neuropsychiatric manifestations. Both animal and human-induced pluripotent stem cell (hiPSC) models served as our primary investigative platforms.
METHODS
Utilizing the PRISMA 2020 guidelines, our search strategy involved scouring articles from the PubMed and Google Scholar databases covering a span of two decades (2003-2023). Of the initial collection, 27 rigorously evaluated studies formed the essence of our review.
RESULTS
Our review suggested the significant ties between neurexin anomalies and neurodevelopmental and neuropsychiatric outcomes, most notably ASD. Rodent-based investigations delineated pronounced ASD-associated behaviors, and hiPSC models derived from ASD-diagnosed patients revealed the disruptions in calcium dynamics and synaptic activities. Additionally, our review underlined the integral role of specific neurexin variants, primarily NRXN1, in the pathology of schizophrenia. It was also evident from our observation that neurexin malfunctions were implicated in a broader array of these disorders, including ADHD, intellectual challenges, and seizure disorders.
CONCLUSION
This review accentuates the cardinal role neurexins play in the pathological process of neurodevelopmental and neuropsychiatric disorders. The findings underscore a critical need for standardized methodologies in developing animal and hiPSC models for future studies, aiming to minimize heterogeneity. Moreover, we highlight the need to expand research into less studied neurexin variants (i.e., NRXN2 and NRXN3), broadening the scope of our understanding in this field. Our observation also projects hiPSC models as potent tools for bridging research gaps, promoting translational research, and fostering the development of patient-specific therapeutic interventions.
PubMed: 38380150
DOI: 10.3389/fnbeh.2024.1297374 -
Epilepsy & Behavior : E&B Mar 2024Psychiatric comorbidity is a double burden among people with epilepsy. Since people with epilepsy are more vulnerable to psychiatric illnesses. So, the implementation of... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Psychiatric comorbidity is a double burden among people with epilepsy. Since people with epilepsy are more vulnerable to psychiatric illnesses. So, the implementation of an appropriate intervention to minimize the double burden of comorbidity is very important. Therefore, this systematic review and meta-analysis aimed to assess the prevalence and associated factors of psychiatric comorbidity among people with epilepsy in Ethiopia.
METHODS
This systematic review and meta-analysis followed the Preferred Reporting Item Review and Meta-analysis (PRISMA) guideline. Searching databases were PubMed, PsycINFO, Web of Science, Cochrane Library, Google Scholar, and HINARI.The quality of the included articles was assessed using the Newcastle-Ottawa Scale (NOS). The pooled meta-logistic regression was computed to estimate the pooled prevalence and the risk factors with a 95% CI.
RESULTS
The pooled prevalence of psychiatric comorbidity in people with epilepsy was 34.69 % (95 % CI: 29.27, 40.10). Frequent seizures (POR = 2.94: 95 % CI: 1.08, 8.00) and a history of divorce (POR = 2.00: 95 % CI: 1.09, 3.81) were associated factors of psychiatric comorbidity in people with epilepsy.
CONCLUSIONS
This systematic review and meta-analysis revealed that the pooled prevalence of psychiatric comorbidity among people with epilepsy was found to be higher compared with the general population. Therefore, among people with epilepsy, parallel psychiatric evaluation is very important along with neurological evaluation.
Topics: Humans; Ethiopia; Comorbidity; Epilepsy; Risk Factors; Mental Disorders; Prevalence
PubMed: 38277847
DOI: 10.1016/j.yebeh.2024.109648 -
Epilepsy & Behavior : E&B May 2024In recent years, adjunctive therapies for epilepsy management are being explored due to considerable side effects carried by antiepileptic drugs (AEDs) and widespread... (Review)
Review
OBJECTIVES
In recent years, adjunctive therapies for epilepsy management are being explored due to considerable side effects carried by antiepileptic drugs (AEDs) and widespread reports of drug-resistant epilepsy. One such approach is non-invasive musical neurostimulation. Within this context, Mozart's sonata K448 has received particular attention following reports of reduced seizure frequency and a decrease in epileptiform discharges during and after music exposure; often described as the 'Mozart effect'. However, controversy exists around the effectiveness of K448 in epilepsy and the strength and quality of the evidence supporting it. Therefore, this study aims to systematically review the available literature around the Mozart effect, in both adult and paediatric cases of epilepsy.
METHODS
We carried out a literature search on PubMed, Science Direct, Scopus and Web of Science using the query string ALL= (Mozart AND epileps*). Selected clinical studies were classified based on the age of the population studied, as paediatric (0-18 years), adult (19 years or older) or a combination of the two. All the studies were evaluated using the Johns Hopkins Nursing Evidence-Based Practice (JHNEBP) rating scale to determine the strength of the evidence (level) and the quality of the research evidence.
RESULTS
Out of 538 records, 25 studies were selected, grouped based on the age of the population studied and evaluated using the JHNEBP rating scale. Ten level 1 studies, which represent the strongest evidence, were identified, including six RCTs and three meta-analyses. Nine of these ten studies show a decrease in epileptiform discharges and in seizure frequency following exposure to Mozart's K448. One multiverse analysis reported lack of statistically significant evidence to support the use of K448 in epilepsy or any other medical condition.
CONCLUSIONS
A growing body of evidence supports the Mozart effect on epilepsy, with notable studies including RCTs and comprehensive meta-analyses. This review identified nine level 1 studies, conducted by research groups worldwide, which endorse the use of Mozart's music to reduce seizures and epileptiform discharges in adult and paediatric epilepsy patients. However, existing research exhibits limitations like varying protocols, small sample sizes and diverse treatment regimens. Additionally, studies that combine adult and paediatric patients fail to take account of developmental differences between these two groups - particularly with regards to brain maturation and neurophysiology - which could negatively impact upon the accuracy of findings by obscuring important age-related differences in response to intervention. Adequately addressing these limitations will be crucial to demonstrating proof of concept; otherwise, a potentially valuable, non-invasive, accessible, and affordable therapeutic option for drug-resistant epilepsy will remain on the medical fringe. Further research with larger samples and stricter protocols, particularly considering patient age and drug regimens, is required.
Topics: Humans; Drug Resistant Epilepsy; Music Therapy; Child; Adult; Adolescent; Child, Preschool
PubMed: 38636110
DOI: 10.1016/j.yebeh.2024.109743 -
Journal of Clinical Neuroscience :... Aug 2023Epilepsy surgery is an underutilised, efficacious management strategy for selected individuals with drug-resistant epilepsy. Natural language processing (NLP) may aid in... (Review)
Review
INTRODUCTION
Epilepsy surgery is an underutilised, efficacious management strategy for selected individuals with drug-resistant epilepsy. Natural language processing (NLP) may aid in the identification of patients who are suitable to undergo evaluation for epilepsy surgery. The feasibility of this approach is yet to be determined.
METHOD
In accordance with the PRISMA guidelines, a systematic review of the databases PubMed, EMBASE and Cochrane library was performed. This systematic review was prospectively registered on PROSPERO.
RESULTS
6 studies fulfilled inclusion criteria. The majority of included studies reported on datasets from only a single centre, with one study utilising data from two centres and one study six centres. The most commonly employed algorithms were support vector machines (5/6), with only one study utilising NLP strategies such as random forest models and gradient boosted machines. However, the results are promising, with all studies demonstrating moderate to high levels of performance in the identification of patients who may be suitable to undergo epilepsy surgery evaluation. Furthermore, multiple studies demonstrated that NLP could identify such patients 1-2 years prior to the treating clinicians instigating referral. However, no studies were identified that have evaluated the influence of implementing such algorithms on healthcare systems or patient outcomes.
CONCLUSIONS
NLP is a promising approach to aid in the identification of patients that may be suitable to undergo epilepsy surgery evaluation. Further studies are required examining diverse datasets with additional analytical methodologies. Studies evaluating the impact of implementation of such algorithms would be beneficial.
Topics: Humans; Natural Language Processing; Epilepsy; Algorithms; Drug Resistant Epilepsy; Random Forest
PubMed: 37354663
DOI: 10.1016/j.jocn.2023.06.010 -
Seizure Aug 2023To document epilepsy-related mortality in sub-Saharan Africa (SSA) and investigate possible associations with onchocerciasis endemicity. (Meta-Analysis)
Meta-Analysis
PURPOSE
To document epilepsy-related mortality in sub-Saharan Africa (SSA) and investigate possible associations with onchocerciasis endemicity.
METHODS
Systematic review with meta-analysis. Searches were performed in PubMed and Google Scholar (search terms: 'epilepsy'; 'mortality/death'; 'sub-Saharan Africa'). Included studies were classified as high-risk or low-risk for onchocerciasis based on documented endemicity data. Pooled mortality rates and annual case fatality rates (CFR) were calculated, and risk factors for mortality among persons with epilepsy (PWE) were investigated using meta-regression analysis.
RESULTS
The 28 eligible studies reported 30 epilepsy surveys, of which 9 (30.0%) were conducted in onchocerciasis high-risk sites. The pooled epilepsy mortality rate was 20.9 (95% CI: 5.9-74.4) per 100,000 person-years, and the pooled CFR was 36.2 (95% CI: 23.9-54.4) per 1,000 PWE per year, albeit with substantial between-study heterogeneity. Compared to onchocerciasis low-risk sites, high-risk sites had higher pooled mortality (342.9 versus 10.0 per 100,000 PY; p<0.001) and CFR (57.0 versus 26.6 per 1,000 PWE per year; p = 0.001). Mortality of PWE was almost five-fold that of people without epilepsy (mortality risk ratio: 4.9; 95% CI: 3.5-6.8). Studies in onchocerciasis high-risk sites and the study which recruited only PWE with nodding syndrome were associated with higher CFR (p = 0.044 and p = 0.002, respectively). The leading causes of epilepsy-related death were status epilepticus (58.5%), drowning (15.7%), and sudden unexpected death in epilepsy (10.1%).
CONCLUSION
Epilepsy mortality remains high in SSA. Most reported causes of death among PWE might be averted by improving seizure control. Better epilepsy prevention and care are urgently needed, particularly in onchocerciasis-endemic settings.
Topics: Humans; Onchocerciasis; Epilepsy; Seizures; Status Epilepticus; Africa South of the Sahara; Prevalence
PubMed: 37451075
DOI: 10.1016/j.seizure.2023.07.006 -
Neurology(R) Neuroimmunology &... Jan 2024We characterize clinical and neuroimaging features of SARS-CoV-2-related acute necrotizing encephalopathy (ANE).
BACKGROUND AND OBJECTIVES
We characterize clinical and neuroimaging features of SARS-CoV-2-related acute necrotizing encephalopathy (ANE).
METHODS
Systematic review of English language publications in PubMed and reference lists between January 1, 2020, and June 30, 2023, in accordance with PRISMA guidelines. Patients with SARS-CoV-2 infection who fulfilled diagnostic criteria for sporadic and genetic ANE were included.
RESULTS
From 899 articles, 20 cases (17 single case reports and 3 additional cases) were curated for review (50% female; 8 were children). Associated COVID-19 illnesses were febrile upper respiratory tract infections in children while adults had pneumonia (45.6%) and myocarditis (8.2%). Children had early neurologic deterioration (median day 2 in children vs day 4 in adults), seizures (5 (62.5%) children vs 3 of 9 (33.3%) adults), and motor abnormalities (6 of 7 (85.7%) children vs 3 of 7 (42.9%) adults). Eight of 12 (66.7%) adults and 4 (50.0%) children had high-risk ANE scores. Five (62.5%) children and 12 (66.7%) adults had brain lesions bilaterally and symmetrically in the putamina, external capsules, insula cortex, or medial temporal lobes, in addition to typical thalamic lesions of ANE. Hypotension was only seen in adults (30%). Hematologic derangements were common: lymphopenia (66.7%), coagulopathy (60.0%), or elevated D-dimers (100%), C-reactive protein (91.7%), and ferritin (62.5%). A pathogenic heterozygous c/.1754 C>T variant in was present in 2 children: one known to have this before SARS-CoV-2 infection, and a patient tested because the SARS-CoV-2 infection was the second encephalopathic illness. Three other children with no prior encephalopathy or family history of encephalopathy were negative for this variant. Fifteen (75%) received immunotherapy (with IV methylprednisolone, immunoglobulins, tocilizumab, or plasma exchange): 6 (40.0%) with monotherapy and 9 (60.0%) had combination therapy. Deaths were in 8 of 17 with data (47.1%): a 2-month-old male infant and 7 adults (87.5%) of median age 56 years (33-70 years), 4 of whom did not receive immunotherapy.
DISCUSSION
Children and adults with SARS-CoV-2 ANE have similar clinical features and neuroimaging characteristics. Mortality is high, predominantly in patients not receiving immunotherapy and at the extremes of age.
Topics: Adult; Child; Child, Preschool; Female; Humans; Infant; Male; Middle Aged; Brain Diseases; COVID-19; Methylprednisolone; SARS-CoV-2; Seizures; Aged
PubMed: 38086061
DOI: 10.1212/NXI.0000000000200186 -
Epilepsy & Behavior Reports 2024Exercise interventions in epilepsy have been shown to improve seizure frequency, physical capacity, quality of life, mood, and cognitive functioning. However, the... (Review)
Review
Exercise interventions in epilepsy have been shown to improve seizure frequency, physical capacity, quality of life, mood, and cognitive functioning. However, the effectiveness of exercise in improving sleep in epilepsy is less clear. The purpose of this report is to identify the published literature regarding exercise interventions in people with epilepsy to determine 1) what proportion of published clinical trials assess sleep as an outcome, and 2) what benefits of exercise interventions on sleep have been observed. We searched the PubMed, PsycINFO, and SCOPUS electronic databases using the search terms "epilepsy AND [exercise OR physical activity]" and identified 23 articles reporting on 18 unique clinical trials. Nine studies were conducted in adults, five in children, and four in adults and children with active seizures, controlled seizures, or both. Exercise modalities included aerobic exercise, strength training, walking, and yoga, among others, and some also included educational and motivational components. Exercise effects on sleep were tested in four studies, two of which only included indirect measures of sleep- and rest-related fatigue, with mixed results. Of the two reports assessing sleep directly, one reported marginal non-significant improvements in subjective sleep quality and no improvements in objective sleep quality in children after twelve weeks of walking, and the other reported no benefits in subjective sleep quality after twelve weeks of combined aerobic, strength, and flexibility training in adults. Given the health benefits of sleep and detrimental effects of sleep deprivation in epilepsy, epilepsy researchers need to assess the effects of exercise interventions on sleep.
PubMed: 38779424
DOI: 10.1016/j.ebr.2024.100675 -
Frontiers in Neurology 2023Magnetic resonance-guided laser interstitial thermal therapy (MRgLiTT) and stereoelectroencephalography-guided radiofrequency thermocoagulation (SEEG-RFTC) are two...
Magnetic resonance-guided laser interstitial thermal therapy vs. stereoelectroencephalography-guided radiofrequency thermocoagulation in epilepsy patients with focal cortical dysplasia: a systematic review and meta-analysis.
BACKGROUND
Magnetic resonance-guided laser interstitial thermal therapy (MRgLiTT) and stereoelectroencephalography-guided radiofrequency thermocoagulation (SEEG-RFTC) are two effective, minimally invasive treatments for epilepsy with focal cortical dysplasia (FCD). The purpose of this study is to conduct a meta-analysis to evaluate and compare the efficacy and safety of these two therapies in epilepsy patients with FCD.
METHODS
We searched PubMed, Embase, Cochrane, and other databases for articles published before March 2023. The primary objective was to compare the effectiveness and complications of MRgLiTT and SEEG-RFTC in epilepsy patients with FCD. The second objective was to determine which method provides a better prognosis for specific subgroup patients.
RESULTS
According to the inclusion and exclusion criteria, 18 studies were included, comprising 270 FCD patients including 37 patients from 6 MRgLiTT studies and 233 from 12 SEEG-RFTC studies. There were no significant differences between MRgLiTT and SEEG-RFTC groups in the seizure-freedom rate (59%, 95% CI 44-74%; 52%, 95% CI 47-57%, = 0.86) and the rate of ≥50% seizure-reduction of FCD (90%, 95% CI 80-100%; 90%, 95% CI 86-94%, = 0.42). Both methods had low complication rates (17.1%, 28/159) and long-term complication (2.5%, 4/159) rate, with no significant difference between them ( = 0.17).
CONCLUSION
Both MRgLiTT and SEEG-RFTC are safe and minimally invasive treatments for patients with FCD. They have comparable performance in terms of postoperative seizure-freedom rates in patients with FCD, and both can be used as treatment options for patients with FCD. Our study found that SEEG-RFTC had a better therapeutic effect in the FCD2b subgroup.
PubMed: 37928136
DOI: 10.3389/fneur.2023.1241763 -
Epilepsy Research May 2024Implantable brain recording and stimulation devices apply to a broad spectrum of conditions, such as epilepsy, movement disorders and depression. For long-term... (Meta-Analysis)
Meta-Analysis
Implantable brain recording and stimulation devices apply to a broad spectrum of conditions, such as epilepsy, movement disorders and depression. For long-term monitoring and neuromodulation in epilepsy patients, future extracranial subscalp implants may offer a promising, less-invasive alternative to intracranial neurotechnologies. To inform the design and assess the safety profile of such next-generation devices, we estimated extracranial complication rates of deep brain stimulation (DBS), cranial peripheral nerve stimulation (PNS), responsive neurostimulation (RNS) and existing subscalp EEG devices (sqEEG), as proxy for future implants. Pubmed was searched systematically for DBS, PNS, RNS and sqEEG studies from 2000 to February 2024 (48 publications, 7329 patients). We identified seven categories of extracranial adverse events: infection, non-infectious cutaneous complications, lead migration, lead fracture, hardware malfunction, pain and hemato-seroma. We used cohort sizes, demographics and industry funding as metrics to assess risks of bias. An inverse variance heterogeneity model was used for pooled and subgroup meta-analysis. The pooled incidence of extracranial complications reached 14.0%, with infections (4.6%, CI 95% [3.2 - 6.2]), surgical site pain (3.2%, [0.6 - 6.4]) and lead migration (2.6%, [1.0 - 4.4]) as leading causes. Subgroup analysis showed a particularly high incidence of persisting pain following PNS (12.0%, [6.8 - 17.9]) and sqEEG (23.9%, [12.7 - 37.2]) implantation. High rates of lead migration (12.4%, [6.4 - 19.3]) were also identified in the PNS subgroup. Complication analysis of DBS, PNS, RNS and sqEEG studies provides a significant opportunity to optimize the safety profile of future implantable subscalp devices for chronic EEG monitoring. Developing such promising technologies must address the risks of infection, surgical site pain, lead migration and skin erosion. A thin and robust design, coupled to a lead-anchoring system, shall enhance the durability and utility of next-generation subscalp implants for long-term EEG monitoring and neuromodulation.
Topics: Humans; Deep Brain Stimulation; Electrodes, Implanted; Electroencephalography; Seizures
PubMed: 38564925
DOI: 10.1016/j.eplepsyres.2024.107356