{"title":"Off-label use of cannabidiol in genetic epileptic and developmental encephalopathies: A case report","authors":"Elisa Mannini , Francesco Misirocchi , Stefania Lazzari , Giulia Balella , Dario Bottignole , Maddalena Frapporti , Lucia Zinno , Irene Florindo , Liborio Parrino , Carlotta Mutti","doi":"10.1016/j.ebr.2024.100687","DOIUrl":"https://doi.org/10.1016/j.ebr.2024.100687","url":null,"abstract":"<div><p>Developmental Epileptic encephalopathies (DEEs) are severe neurological conditions where cognitive functions appear modulated by both seizure and interictal epileptiform activity. Cannabidiol (CBD) has been shown to be highly effective in the treatment of drug-resistant seizures in patients with DEEs. Along with its antiseizure effects, CBD demonstrated clinical beneficial effects in patients’ quality of life, sleep and numerous adaptive behaviors. However, based on the available phase III studies, the indications for this treatment have so far been restricted to Lennox-Gastaut syndrome (LGS), Dravet syndrome (DS) and tuberous sclerosis complex (TSC) by regulatory authorities. We present the case of a 30-year-old girl with a rare genetic DEE, experiencing relevant seizure frequency reduction together with striking improvement in sleep quality, mood, behavior, language and motor skills after introducing off-label CBD.</p></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"27 ","pages":"Article 100687"},"PeriodicalIF":1.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2589986424000443/pdfft?md5=fd3325f0c7ff8b1d3fa9b7438c288400&pid=1-s2.0-S2589986424000443-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141483961","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Halley B. Alexander , Heidi M. Munger Clary , Hossam A. Shaltout , Nathan B. Fountain , Pamela Duncan , Peter Brubaker , Jason Fanning
{"title":"Developing optimized physical activity interventions for drug-resistant epilepsy: Challenges and lessons learned from a remote exercise intervention pilot trial","authors":"Halley B. Alexander , Heidi M. Munger Clary , Hossam A. Shaltout , Nathan B. Fountain , Pamela Duncan , Peter Brubaker , Jason Fanning","doi":"10.1016/j.ebr.2024.100693","DOIUrl":"https://doi.org/10.1016/j.ebr.2024.100693","url":null,"abstract":"<div><p>People with epilepsy (PWE) stand to benefit significantly from increasing their physical activity, but promotion of physical activity is difficult in any population; a challenge compounded by the unique barriers encountered by PWE, especially those with drug-resistant epilepsy (DRE). This study explores the feasibility of a remotely delivered, 12-week aerobic exercise program based on social cognitive theory principles in adults with DRE. This line of research is nested within the Multiphase Optimization Strategy (MOST), a framework that emphasizes iterative early pilot work (preparation phase research), followed by iterative optimization phase research. Ten participants were recruited, and four out of ten completed the study, resulting in 3.8 % recruitment from those preliminarily eligible by chart review, and 40 % retention. While acceptability was high among those who completed the study, recruitment, retention, and uptake were low. Three key related lessons learned emerged: 1) low appeal of an exercise intervention in our population of DRE 2) barriers related to comorbid mental health struggles, and 3) fear of seizures. How to best approach physical activity promotion in PWE, particularly DRE, will require a somewhat novel approach involving iterative pilot work and optimization before large scale efficacy trials and implementation can be achieved.</p></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"27 ","pages":"Article 100693"},"PeriodicalIF":1.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2589986424000509/pdfft?md5=e958391ad122f560e6ac2f1b39378629&pid=1-s2.0-S2589986424000509-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141540085","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Thorsten Wildermann , Felicitas Becker , Sarah Jesse , Hartmut Baier , Jan Wagner
{"title":"Successful use of Fenfluramine in super-refractory status epilepticus in a patient with tuberous sclerosis complex and Lennox-Gastaut syndrome","authors":"Thorsten Wildermann , Felicitas Becker , Sarah Jesse , Hartmut Baier , Jan Wagner","doi":"10.1016/j.ebr.2024.100697","DOIUrl":"10.1016/j.ebr.2024.100697","url":null,"abstract":"<div><p>A 24-year-old female patient with pre-existing refractory epilepsy caused by tuberous sclerosis (TSC) and electroclinical features of Lennox-Gastaut syndrome (LGS) was referred to our hospital from an external clinic. Upon arrival, she presented with super-refractory status epilepticus (SRSE) since anaesthetics had already been used in the referring clinic. Despite various changes in ASM-treatment and continuous administration of anaesthetics for more than two weeks, SRSE could not be terminated. On treatment day 24, we started Fenfluramin (FFA) which was soon titrated to a dose of 0,7 mg/kg/day. A few days after beginning the treatment with FFA, EEG and clinical situation improved dramatically. The following 6 weeks of treatment went without reported seizures. This case illustrates the successful use of FFA in SRSE in TSC and LGS and, to the best of our knowledge, represents the first report of FFA in this clinical context.</p></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"27 ","pages":"Article 100697"},"PeriodicalIF":1.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2589986424000546/pdfft?md5=c61ed2100f6345d7bc0aebf87e561214&pid=1-s2.0-S2589986424000546-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141838425","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kjell Heuser , Louis Romundstad , Jugoslav Ivanovic , Arild Egge , Erik Sætre , Kristin Alfstad , Line Sveberg , Line Bedos Ulvin , Erik Taubøll
{"title":"A case of acute functional hemispherotomy in a young woman with hemimegalencephaly and super-refractory status epilepticus","authors":"Kjell Heuser , Louis Romundstad , Jugoslav Ivanovic , Arild Egge , Erik Sætre , Kristin Alfstad , Line Sveberg , Line Bedos Ulvin , Erik Taubøll","doi":"10.1016/j.ebr.2024.100728","DOIUrl":"10.1016/j.ebr.2024.100728","url":null,"abstract":"<div><div>Status epilepticus (SE) is a critical medical emergency that demands immediate and effective intervention. We report a unique case involving a 21-year-old woman with left hemimegalencephaly who was hospitalized for super-refractory status epilepticus (SRSE) that persisted for 8 weeks. Despite extensive treatment efforts including multiple anti-seizure medications, anesthetics, high-dose methylprednisolone, anakinra, magnesium infusion, and a ketogenic diet, her condition remained unchanged. Ultimately, a left functional hemispherotomy was performed, informed by MRI findings and EEG results showing predominantly left-sided epileptic activity. This decision was made collaboratively when all other therapeutic options had been exhausted. Postoperatively, the patient recovered with manageable neurological deficits and a satisfactory quality of life. To our knowledge, this is the first reported case of acute functional hemispherotomy in an adult with hemimegalencephaly and SRSE.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"28 ","pages":"Article 100728"},"PeriodicalIF":1.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142699693","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Tobias M. Redecker , Haang Jeung-Maarse , Christian Brandt
{"title":"Panic disorder in epilepsy","authors":"Tobias M. Redecker , Haang Jeung-Maarse , Christian Brandt","doi":"10.1016/j.ebr.2024.100646","DOIUrl":"10.1016/j.ebr.2024.100646","url":null,"abstract":"<div><p>A 51-year-old woman showed structural epilepsy following an atypical, nontraumatic intracranial hemorrhage in the right frontal area. Despite successful seizure control with lamotrigine, she developed severe morning anxiety and panic attacks, leading to agoraphobia, social withdrawal, and psychogenic nonepileptic seizures. Neuropsychiatric and psychological assessments confirmed an anxiety disorder with no significant symptoms of depression. The patient received various psychopharmacological treatments with limited success. This case report illustrates that managing panic disorder in patients with structural epilepsy requires a comprehensive treatment approach that includes pharmacotherapy and psychotherapy. Differential diagnosis and accurate treatment are crucial because of the symptom overlap between panic attacks and <em>peri</em>-ictal fear. Screenings instruments such as the Panic and Agoraphobia Scale (PAS) can aid in assessing anxiety-related symptoms. First-line pharmacotherapy with selective serotonin reuptake inhibitors, especially sertraline, or venlafaxine can effectively reduce panic attacks and can be recommended in patients with epilepsy. Psychotherapy, particularly cognitive-behavioral therapy, is the treatment of choice. Referral to a psychiatrist is indicated when symptoms are severe or refractory to treatment.</p></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"25 ","pages":"Article 100646"},"PeriodicalIF":1.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2589986424000030/pdfft?md5=a6b77f84b1750f711464c1080c36e929&pid=1-s2.0-S2589986424000030-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139456448","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Do psychotropic drugs cause seizures?","authors":"Margaret Gopaul , Hamada Altalib","doi":"10.1016/j.ebr.2024.100679","DOIUrl":"https://doi.org/10.1016/j.ebr.2024.100679","url":null,"abstract":"<div><p>Patients with epilepsy often present with concurrent psychiatric disorders, posing unique challenges for healthcare providers. This review explores the intricate relationship between psychiatric comorbidities, epilepsy, and psychotropic medications to inform clinical decision-making. The bidirectional association between epilepsy and psychiatric conditions complicates treatment, with psychiatric symptoms preceding or following seizure onset. The review discusses the seizure risks associated with antidepressants, CNS stimulants, and antipsychotics, shedding light on both historical perspectives and recent empirical evidence. Antidepressants, particularly tricyclic antidepressants (TCAs), are known to pose seizure risks, while newer agents like selective serotonin reuptake inhibitors (SSRIs) exhibit lower incidences and even potential anticonvulsant effects. Contrary to common beliefs, CNS stimulants used in attention-deficit/hyperactivity disorder (ADHD) treatment show efficacy without significantly increasing seizure risk. However, the association between ADHD and seizures warrants careful consideration. Among antipsychotics, clozapine stands out for its heightened seizure risks, especially during titration and at high doses, necessitating close monitoring and individualized approaches. Understanding the nuanced seizure risks associated with different psychotropic medications is crucial for optimizing patient care and minimizing iatrogenic seizures in this vulnerable population. By recognizing the complexities of psychiatric comorbidities in epilepsy and considering the unique challenges they pose, healthcare providers can make informed decisions to enhance patient safety and treatment outcomes. This review offers practical insights to guide clinicians in navigating the intricate landscape of managing psychiatric comorbidities in patients with epilepsy.</p></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"27 ","pages":"Article 100679"},"PeriodicalIF":1.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2589986424000364/pdfft?md5=4c0846505b8a91437d3a7a00ab40be9e&pid=1-s2.0-S2589986424000364-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141243080","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Low-dose fenfluramine as an effective treatment option for ‘atypical’ Dravet syndrome","authors":"Akihiro Iguchi , Tokito Yamaguchi , Tomona Yabe , Mitsuhiro Miyashita , Satoshi Mizutani , Hideyuki Otani , Rie Miyata , Katsumi Imai","doi":"10.1016/j.ebr.2024.100714","DOIUrl":"10.1016/j.ebr.2024.100714","url":null,"abstract":"<div><div>Dravet syndrome (DS) is characterized by recurrent convulsive seizures, including status epilepticus, and intellectual disability as a comorbidity. Seizures associated with DS are commonly resistant to antiseizure medications. Typical features of DS are recurrent episodes of status epilepticus, the presence of genetic mutations, and no abnormal magnetic resonance imaging (MRI) findings. Here, we report a rare case of DS in a 14-year-old girl who was negative for genetic mutations, had experienced status epilepticus only once, and had abnormal findings on brain MRI. Although our patient’s case features are atypical of DS, they do not contradict the diagnostic criteria. Despite the difficulty in diagnosing DS because of the negative genetic testing results, we started our patient on fenfluramine (FFA). Long-term treatment with low-dose FFA effectively controlled our patient’s seizures and resulted in cognitive and functional improvements.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"28 ","pages":"Article 100714"},"PeriodicalIF":1.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142427179","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Should neurologists treat common psychiatric comorbidities in patients with epilepsy?","authors":"Andres M. Kanner , Heidi M. Munger Clary","doi":"10.1016/j.ebr.2024.100725","DOIUrl":"10.1016/j.ebr.2024.100725","url":null,"abstract":"<div><div>Psychiatric comorbidities are common and highly impactful among people with epilepsy, yet they are underrecognized and undertreated due to challenges including shortage of specialty mental health providers and lack of neurologist education to enable management by neurologists. The purpose of this special issue is to address these gaps by providing a practical resource for neurologists to safely manage comorbidities via pharmacotherapy for common comorbidities. In this introductory article, we summarize key categories of psychiatric problems in epilepsy and a broad overview of management strategies. These include reactive psychiatric symptoms, interictal psychiatric disorders, <em>peri</em>-ictal psychiatric episodes, and iatrogenic causes of psychiatric symptoms. Reactive psychiatric symptoms can be addressed via epilepsy education and neurologist acknowledgement of the loss of predictability inherent in epilepsy. Interictal psychiatric disorders can be identified via standardized screening instruments and managed using evidence-based pharmacotherapy with a similar approach to the general population. Peri-ictal psychiatric episodes have a consistent temporal relation to seizure occurrence and are primarily managed via prevention through seizure treatment. Patients with personal or family history of psychiatric disorders are at particular risk for iatrogenic psychiatric effects; neurologists should take care in treatment selection among these individuals and be ready to respond to manage iatrogenic effects if they arise. Management of specific psychiatric conditions are addressed in more depth in topic-focused articles throughout the remainder of the special issue.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"28 ","pages":"Article 100725"},"PeriodicalIF":1.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142573486","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alena Stasenko , Erik Kaestner , Adam Schadler , Evan Brady , Jonathan Rodriguez , Rebecca W. Roth , Ezequiel Gleichgerrcht , Jonathan L. Helm , Daniel L. Drane , Carrie R. McDonald
{"title":"Exercise, memory, and the hippocampus: Uncovering modifiable lifestyle reserve factors in refractory epilepsy","authors":"Alena Stasenko , Erik Kaestner , Adam Schadler , Evan Brady , Jonathan Rodriguez , Rebecca W. Roth , Ezequiel Gleichgerrcht , Jonathan L. Helm , Daniel L. Drane , Carrie R. McDonald","doi":"10.1016/j.ebr.2024.100721","DOIUrl":"10.1016/j.ebr.2024.100721","url":null,"abstract":"<div><div>Physical exercise is an emerging target for improving cognition in aging and neurological disease. Due to the beneficial impact of exercise on hippocampal health and the vulnerability of the hippocampus in medication-resistant temporal lobe epilepsy (TLE), exercise could present a promising intervention in TLE. We investigated whether exercise engagement is associated with verbal memory function and hippocampal integrity in 29 young to middle-aged adults with refractory TLE and 21 demographically matched controls. Participants completed a self-reported questionnaire of weekly exercise, three tests of verbal memory, and a subset (<em>n</em> = 44) underwent structural MRI. Individuals with TLE self-reported lower exercise scores than controls across all levels of exercise intensity (<em>p</em> < 0.001). In TLE, greater exercise engagement was associated with better verbal memory (word-list recall and associative learning; <em>rho</em> = 0.46–0.47; <em>p</em>s <sub>FDR</sub> < 0.05), and with larger contralateral hippocampal volumes (<em>rho</em> = 0.61; <em>p</em> < 0.01). These effects remained significant when controlling for epilepsy-related and demographic factors. Within the limitations of a cross-sectional observational study, these findings suggest that exercise may be a cognitive reserve factor in TLE, potentially mitigating memory decline by enhancing contralateral hippocampal integrity. With future replication and longitudinal studies to clarify the causal pathways of these relationships, exercise holds promise as a low-cost, accessible, and modifiable lifestyle target for improving cognitive health in individuals with refractory TLE.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"28 ","pages":"Article 100721"},"PeriodicalIF":1.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142573487","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sudhindra Vooturi , Bathula Siri , Sai Sirisha , Sita Jayalakshmi
{"title":"Socio economic factors and adherence to home based exercise in people with epilepsy","authors":"Sudhindra Vooturi , Bathula Siri , Sai Sirisha , Sita Jayalakshmi","doi":"10.1016/j.ebr.2024.100698","DOIUrl":"10.1016/j.ebr.2024.100698","url":null,"abstract":"<div><p>Exercise is a well-established component in the management of chronic illness both as a primary prevention and secondary intervention. The assumption that in otherwise healthy individuals, higher socioeconomic status (SES) is positively associated with physical activity (PA) has been debated. We report the influence of SES on adherence to home-based exercise program in people with epilepsy (PWE) from a developing country. Participants’ response to self-reported Social Needs Screening Tool of the American Academy of Family Physicians was collected. The current study is a secondary follow-up and post-hoc analysis of data from patients we have previous published. The average age of the study population was 26.93 ± 10.20 years with 57.8 % men. Among the 116 study participants, 31 (26.72 %) were adherent to the exercise program. Unemployment (14.1 % vs. 0.0 %; p = 0.034) was higher, fewer people had least high school education (76.6 % vs 93.5 %; p = 0.050) in PWE who did not adhere to exercise program. A significantly higher number of PWE who were not adherent to exercise reported that their family members or anyone else never physically hurt them (97.6 % vs 80.6 %; p = 0.05), never threaten (94.1 % vs 74.2 %; p = 0.007) and/or never scream at them (90.6 % vs 74.2 %; p = 0.011). In PWE education and employment are associated with adherence to home-based exercise programs. The role of family support and personal safety in adherence to exercise should be evaluated in detail.</p></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"27 ","pages":"Article 100698"},"PeriodicalIF":1.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2589986424000558/pdfft?md5=e360f79c0f4df2c225321a53e7bc315a&pid=1-s2.0-S2589986424000558-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141852874","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}