Neuromuscular Ultrasound Findings of Myokymia in a Young Woman With Generalized Anti-LGI-1 and Anti-Caspr2 Antibodies Negative Isaac Syndrome.

ABSTRACT: Isaac syndrome is one of the rare peripheral nerve hyperexcitability (PNH) syndromes, which manifests with gross fasciculations, muscle undulation, twitching, and cramps, with or without autonomic and sensory symptoms. The diagnosis relies on characteristic electromyogram findings and the presence of anti-leucine-rich glial inactivated 1 and anti-contactin-associated protein 2 antibodies in the serum. Here, we report the case of a 21-year-old woman, who presented with extremities and tongue myokymia whose electromyogram findings were compatible with PNH, albeit seronegative for antibodies. Neuromuscular ultrasound was performed showing high-frequency rotatory, to-and-fro, high-amplitude movement of superficial and deep muscle fascicles, more prominent in the proximal than distal muscles. Neuromuscular ultrasound may be a useful adjunct in the diagnosis of PNH.

Infodemiology of autoimmune encephalitis, autoimmune seizures, and autoimmune epilepsy: An analysis of online search behavior using Google Trends.

BACKGROUND: Patients and their caregivers, including clinicians and educators, use web-based search engines to access healthcare-related information from the internet. Online search behavior analysis has been used to obtain insights on health information demand. OBJECTIVES: We aimed to describe the online search behavior for autoimmune encephalitis, autoimmune seizures, and autoimmune encephalitis (AE) worldwide over time through the analysis of search volumes made on Google. METHODS: In this infodemiological study, we retrieved search volume indices for the keyword "autoimmune encephalitis (search term)", "autoimmune seizures (search term)", and "autoimmune encephalitis (search term)" based on worldwide search data from January 01, 2004 to October 31, 2021, using Google Trends. We performed a descriptive analysis of search volume patterns, including related topics and queries. RESULTS: There was a progressive increase in search volume numbers over time for the keyword "autoimmune encephalitis", "autoimmune seizures", and "autoimmune epilepsy" with no annual seasonal variation. Peak search volumes for these keywords were recorded in July 2018, February 2005, December 2012, respectively. The greatest search volume for "autoimmune encephalitis" was recorded in Singapore, followed by Australia, the United States of America, the Philippines, and New Zealand, whereas it was highest in the United States for "autoimmune seizures" and "autoimmune epilepsy". The most searched topics were related to definition, causes, symptoms, diagnosis, and treatment. All related topics and queries increased in volume by more than 5000-fold over time. CONCLUSIONS: This study showed an uptrend in the online search interest on autoimmune encephalitis, autoimmune seizures, and autoimmune epilepsy over time, which may reflect the increased awareness of the condition by the public and the medical community. Information on online health information-seeking behavior may be obtained from Google Trends data despite its limitations.

Perampanel for amyotrophic lateral sclerosis: A systematic review and meta-analysis.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a fatal and incurable neurodegenerative disease. There is still no established cost-effective treatment that can improve functional status and survival of ALS patients. Perampanel, by inhibiting neuronal calcium ion influx and preventing dyslocalization of nuclear proteins, has the potential to ameliorate ALS neurodegeneration. OBJECTIVES: This study aims to determine the efficacy and safety of perampanel among ALS patients in terms of improvement in functional status using a review of relevant studies. METHODS: MedLine, Cochrane Central Register for Controlled Trials, Scopus, Embase, Literatura Latino-Americana e do Caribe em Ciências da Saúde, ClinicalTrials.gov website, and HERDIN databases were searched from inception to August 2021 for relevant studies. RESULTS: The search yielded 132 articles; 3 studies were included in the analysis. Pooled evidence shows that perampanel compared to placebo significantly improves cortical motor hyperexcitability but not the ALS functional rating scale-revised score. Perampanel is associated with adverse events such as aggression, somnolence, anger, and dysarthria. CONCLUSION: There is no sufficient evidence to support the role of perampanel in improving functional status of ALS patients. Although it can ameliorate motor cortical hyperexcitability, its clinical benefit has not yet been elucidated. Perampanel is not well tolerated among ALS patients as it is associated with adverse events such as aggression, somnolence, anger, and dysarthria. Further studies investigating the role of perampanel early in the ALS disease course, excluding ALS patients with frontotemporal lobe degeneration features and C9ORF72 repeat expansion, and using gradual drug titration schedule are needed to evaluate the potential benefit of perampanel in ALS.

Patterns of Google search behavior for epilepsy and seizures in the Philippines: An infodemiological study.

BACKGROUND: The internet has been established as a rich source of health information that is well utilized by the public. Online health information-seeking behavior may reflect public awareness in particular health-related topics, including epilepsy and seizures. OBJECTIVE: This study aimed to describe online search behavior for epilepsy and seizures in the Philippines through the examination of trends of Philippine-based Google search queries for epilepsy and seizure-related terms. METHODS: The search volume indices (SVIs) for the keywords "epilepsy (disorder)" and "seizure (disease)" based on Philippine-based Google searches from January 2004 to July 2021 were generated using Google Trends. Descriptive analysis of search volume patterns, including related topics and queries, was performed. RESULTS: There was a recent relative increase in Philippine-based searches related to the term "seizure" with a concurrent relative decline in searches related to the term "epilepsy" over time. The Philippines had the fifth-highest SVI for "seizure" based on worldwide trends. Most searches centered on the definition, symptomatology, etiologies, classification, and treatment of seizures and epilepsy. Queries containing seizure-related terms rose in volume by more than 5000% during the seventeen-year period. Search volume tended to peak during the National Epilepsy Awareness Week in September but slumped during holiday months. CONCLUSIONS: Online search behavior in the Philippines for seizure-related terms appears to be on the rise and may reflect increasing public awareness in epilepsy. Factors including fears surrounding epileptic seizures, the stigma surrounding the term "epilepsy", and seasonal events including a regularly held epilepsy awareness week may influence online search behavior.

Treatment gaps and challenges in epilepsy care in the Philippines.

OBJECTIVE: Epilepsy is a neurologic disease that carries a high disease burden and likely, a huge treatment gap especially in low-to-middle income countries (LMIC) such as the Philippines. This review aimed to examine the treatment gaps and challenges that burden Philippine epilepsy care. MATERIALS & METHODS: Pertinent data on epidemiology, research, health financing and health systems, pharmacologic and surgical treatment options, cost of care, and workforce were obtained through a literature search and review of relevant Philippine government websites. RESULTS: The estimated prevalence of epilepsy in the Philippines is 0.9%. Epilepsy research in the Philippines is low in quantity compared with the rest of Southeast Asia (SEA). Inequities in quality and quantity of healthcare services delivered to local government units (LGUs) have arisen because of devolution. Programs for epilepsy care by both government and nongovernment institutions have been implemented. Healthcare expenditure in the Philippines is still largely out-of-pocket, with only partial coverage from the public sector. There is limited access to antiseizure medications (ASMs), mainly due to cost. Epilepsy surgery is an underutilized treatment option. There are only 20 epileptologists in the Philippines, with one epileptologist for every 45,000 patients with epilepsy. In addition, epilepsy care service delivery has been further impeded by the coronavirus disease of 2019 (COVID-19) pandemic. CONCLUSION: There is a large treatment gap in epilepsy care in the Philippines in terms of high epilepsy disease burden, socioeconomic limitations and inadequate public support, sparse clinico-epidemiologic research on epilepsy, inaccessibility of health care services and essential pharmacotherapy, underutilization of surgical options, and lack of specialists capable of rendering epilepsy care. Acknowledgment of the existence of these treatment gaps and addressing such are expected to improve the overall survival and quality of life of patients with epilepsy in the Philippines.

Efficacy and tolerability of intravenous brivaracetam for status epilepticus: A systematic review.

BACKGROUND: Status epilepticus (SE) is an emergent neurologic condition that carries a high risk of morbidity and mortality. Intravenous brivaracetam (IV BRV) may be an alternative anticonvulsant against status epilepticus, although the sparseness of controlled studies on the topic limits its recommendation for this indication. OBJECTIVES: This systematic review aimed to determine the efficacy and safety of IV BRV in the treatment of status epilepticus. METHODS: A comprehensive literature search was conducted until December 2019 through several electronic databases (PubMed, Google Scholar, Scopus, OpenGrey, ScienceDirect, HERDIN, Epistemonikos, CENTRAL, ClinicalTrials.gov) to identify relevant studies. Studies that involved adult patients with SE who were given IV BRV were considered for inclusion in this review. RESULTS: From a total of 34 studies identified, 5 uncontrolled studies with 77 patients were included in this review. Thirty-seven out of 77 patients (48%) with SE responded to IV BRV. Reported time to seizure cessation may be immediate from a few minutes to several hours after IV BRV treatment. Patients manifested with significant disability on Glasgow outcome scale (Median: 3) and modified Rankin scale (Mode: 5). Six patients [somnolence (5), worsening seizures (1)] had treatment emergent adverse events. CONCLUSIONS: Limited evidence from 5 uncontrolled studies involving a limited number of patients suggests that IV BRV may be efficacious and safe in terminating seizures among patients with SE or refractory SE. Further studies employing either prospective, controlled trials or registry-based study designs are essential to determine the definitive role of IV BRV in patients with SE.