Epilepsy and Behavior Reports最新文献

We undertook a survey among epileptologists in China to explore their attitudes toward physical exercise and sports for persons with epilepsy (PWEs). A total of 288 epileptologists participated. Most recognized the potential benefits of physical exercise and sports for PWEs, including improved cognitive function (74.6 %), alleviation of mental disorders (73.2 %), and enhanced quality of life (83.8 %). Epileptologists overwhelmingly agreed on the importance of discussing and encouraging physical exercise and sports for PWEs (97.4 % and 95.2 %, respectively). Before engagement in physical exercise and sports, most epileptologists considered that the duration of seizure-free status could be shorter if the seizures were typically focal, non-motor, or without impaired awareness (p < 0.05). There was consensus (99.1 %) on the need to grade the risk of related activities. Opinions were divided regarding the use of health certificates for restricting PWEs (favored by 63.2 %). The majority (93.9 %) called for an expert consensus or clinical guidelines in China. In conclusion, epileptologists in China generally demonstrate a positive attitude toward physical exercise and sports for PWEs. Both benefits and risks of these activities have generally been acknowledged. It is recommended to prioritize activities with lower risks and higher benefits. However, the recommendations for PWEs with a lower likelihood of recurrence and less risky seizure types can be more liberal. Urgent development of normative guidance from governmental and professional bodies is warranted.

Functional neurological disorder (FND) is a common neurologic disorder associated with many comorbid symptoms including fatigue, pain, headache, and orthostasis. These concurrent symptoms lead patients to accumulate multiple diagnoses comorbid with FND, including fibromyalgia, chronic fatigue syndrome, postural orthostatic tachycardia syndrome, persistent post-concussive symptoms, and chronic pain. The role of physical activity and exercise has not been evaluated in FND populations, though has been studied in certain comorbid conditions. In this traditional narrative literature review, we highlight some existing literature on physical activity in FND, then look to comorbid disorders to highlight the therapeutic potential of physical activity. We then consider abnormalities in the autonomic nervous system (ANS) as a potential pathophysiological explanation for symptoms in FND and comorbid disorders and postulate how physical activity and exercise may provide benefit via autonomic regulation.

Zellweger Syndrome is a peroxisomal disorder that can lead to elevation of long chain fatty acids and epilepsy, which can be drug resistant. The treatment of drug resistant epilepsy can include the ketogenic diet in appropriately chosen patients. Typically, the ketogenic diet is contraindicated in individuals with defects in fatty acid metabolism because of the diet’s reliance on medium and long chain fatty acids. To our knowledge this is the first publication outlining the use of the ketogenic diet in patients with defects in beta oxidation of very long chain fatty acids. We present two patients with Zellweger Syndrome who were placed on a ketogenic diet for drug resistant epilepsy.

Safety and tolerance of the ketogenic diet in patients with Zellweger Syndrome.

We describe a case of focal epilepsy with a semiology consisting of behaviors indicating an enthusiastic desire for those around him to get along and engage in friendly relations, which we refer to as “amity seizures”. The patient was a 41-year-old right-handed male with seizures since age 26. Semiology consisted of stereotyped enthusiastic behaviors such as expressing “Peace! Peace!… Come on, we all on the same team, right?!”, and giving hugs, kisses, and high-fives to those around him. On SEEG evaluation, 2 independent areas of seizure onset were identified, the right hippocampus and right posterior orbitofrontal area. Locally confined seizures had bland manifestation. However, spread from right hippocampus to right orbitofrontal area, or vice versa, elicited his typical amity seizure semiology. To our knowledge this is the first report of the seizure semiology we have coined “Amity seizures”. While emotions were once thought to localize to discrete brain regions, they are now accepted to arise from networks across multiple brain regions. The fact that this behavior only occurred when seizures spread from either of 2 onset zones to the other suggests that this semiology results from network engagement between, and likely beyond, either onset zone.

KBG syndrome is characterised by developmental delay, dental (macrodontia of upper central incisors), craniofacial and skeletal anomalies. Since the identification of variants in the gene (ANKRD11) responsible for KBG syndrome, wider phenotypes are emerging. While there is phenotypic variability within many features of KBG syndrome, epilepsy is not usually markedly severe and movement disorders largely undocumented. Here we describe a novel early onset phenotype of dyskinetic epileptic encephalopathy in a male, who presented during infancy with a florid hyperkinetic movement disorder and developmental regression. Initially he had epileptic spasms and tonic seizures, and EEGs revealed a modified hypsarrhythmia. The epilepsy phenotype evolved to Lennox-Gastaut syndrome with seizures resistant to multiple anti-seizure therapies and the movement disorder evolved to choreoathetosis of limbs and head with oro-lingual dyskinesias. Previous extensive neurometabolic and imaging investigations, including panel-based exome sequencing were unremarkable. Later trio exome sequencing identified a de novo pathogenic heterozygous frameshift deletion of ANKRD11 (c.6792delC; p.Ala2265Profs*72). Review of the literature did not identify any individuals with such a hyperkinetic movement disorder presentation in combination with early-onset epileptic encephalopathy. This report expands the phenotype of ANKRD11-related KBG syndrome to include epileptic dyskinetic encephalopathy.

It is unclear whether a dominant hemispherectomy/hemispherotomy in someone with Rasmussen’s Encephalitis (RE) may produce a satisfactory outcome when performed over the age of 40 years. Important questions include whether RE may continue to evolve three decades after onset, and whether a hemispherectomy may adequately shift language function when performed in older ages. Two cases illustrate seizure, language, motor and functional outcomes after dominant hemispherotomies. The cases were selected from an epilepsy surgery database of procedures performed at a private hospital in Cape Town, South Africa, spanning the period 1998–2023. A man in his 40s with epilepsy since childhood and dominant hemisphere RE partially regained impaired comprehension and ambulation, while expressive language function did not recover post-hemispherotomy. By contrast, a young teenage patient with dominant hemisphere RE demonstrated considerable recovery of expressive and receptive language and ambulation post-surgery. Both remain seizure-free. These two cases demonstrate that a dominant hemispherotomy, when performed on a quadragenarian, may produce a satisfactory, albeit inferior, functional outcome in comparison to when performed in childhood. RE may cause progressive neurological dysfunction in the late thirties and older and should be considered in patients presenting with functional decline decades after disease onset.

In this paper, we discuss how artificial intelligence chatbots based on large-scale language models (LLMs) can be used to disseminate information about the benefits of physical exercise for individuals with epilepsy. LLMs have demonstrated the ability to generate increasingly detailed text and allow structured dialogs. These can be useful tools, providing guidance and advice to people with epilepsy on different forms of treatment as well as physical exercise. We also examine the limitations of LLMs, which include the need for human supervision and the risk of providing imprecise and unreliable information regarding specific or controversial aspects of the topic. Despite these challenges, LLM chatbots have demonstrated the potential to support the management of epilepsy and break down barriers to information access, particularly information on physical exercise.

Posterior quadrant epilepsy surgery, involving the occipital lobe, parietal lobe, or the posterior border of the temporal lobe, accounts for a small percentage of focal resections for medically refractory epilepsy. Prior studies investigating seizure control from posterior quadrant epilepsy surgery are limited. In this study, a retrospective database of patients undergoing surgery for left sided posterior cortex epilepsy at a single large level 4 epilepsy center was analyzed between August 2008 to April 2021 in order to characterize seizure control outcomes. Nine patients presented with epileptogenic foci in the left posterior cortex with a malformation of cortical development deemed as the etiology of seizures for all but one patient. Absolute seizure freedom (Engel I) was achieved in 4 of 9 patients, with the remaining 5 patients achieving an improvement in the frequency of seizures (Engel II/III). Complete resection of the anatomic and physiologic abnormalities was performed in 3 of 4 patients with Engel 1 outcomes and 1 of 5 patients with Class II/III outcomes. Five patients developed new right sided visual field defects, all of which were expected based on the sub-lobar, occipital localization and were viewed as acceptable by the patients and did not interfere with activities of daily living. Overall, our study demonstrates the potential for surgical resection to yield excellent seizure-control outcomes with anticipated, tolerable neurological deficits. This information is important for patients with disabling seizures who may not benefit sufficiently from palliative procedures.

Pediatric patients with epilepsy often have psychosocial and cognitive difficulties. Physical activity has emerged as a lifestyle modification that may reduce seizure burden, enhance brain plasticity, and improve cognitive and psychosocial comorbidities. We systematically reviewed published studies examining the effect of physical activity on cognitive and psychosocial function in children and adolescents with epilepsy. Studies were identified with PubMed and Emory Library databases. Eleven studies met inclusion criteria. Six of 10 studies related to psychosocial outcomes showed benefits of physical activity in children and adolescents with epilepsy, including improvements in internalizing symptoms, relationships, self-esteem, and psychological well-being, but four of the 10 studies showed no psychosocial benefits. Of the six studies evaluating cognitive outcomes, all six indicated that physical activity was associated with cognitive improvements in pediatric epilepsy, including areas of attention, processing speed, executive function, and memory. Our review was limited by the paucity of published studies on this topic, and the use of different measurement tools limited our ability to make direct comparisons between studies. Additional studies that compare pediatric epilepsy populations to non-epilepsy control groups are needed to better understand how physical activity affects seizure control and epilepsy-related comorbidities.