We report the case of a girl with hemimegalencephaly (HME) and Lennox-Gastaut syndrome (LGS) treated by callosotomy at 1 year of age. Over 10 years, her seizure frequency and severity decreased markedly. Hemispherectomy is the main surgical option for HME although HME appears to correlate with a less favorable seizure outcome. However, the clinical presentation of LGS and possible generalized cortical dysplasia, which is indicative of a secondarily generalized epilepsy, might predict a favorable surgical outcome of corpus callosotomy in patients of HME, as in our case.
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http://dx.doi.org/10.1016/j.braindev.2008.03.003 | DOI Listing |
Clin Genet
January 2025
Department of Human Genetics, Research Institute, National Center for Global Health and Medicine, Tokyo, Japan.
Heterozygous pathogenic variants in MBD5 (MIM*611472) and CCM2 (MIM*607929) cause autosomal dominant intellectual developmental disorder 1 (MIM#156200) and cerebral cavernous malformations-2 (MIM#603284), respectively. Both conditions may present with seizures, epilepsy, and status epilepticus. However, super-refractory status epilepticus, defined as seizures lasting more than 24 h, has not been described in either condition.
View Article and Find Full Text PDFJ Craniofac Surg
January 2025
Department of Neurosurgery, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Objective: To confirm the incidence of subcutaneous effusion secondary to cerebrospinal fluid leakage after craniotomy, analyze the risk factors for cerebrospinal fluid leakage leading to subcutaneous effusion, summarize the underlying causes of its occurrence and explore the corresponding treatment strategies.
Methods: A retrospective analysis was conducted on 757 patients who underwent craniotomy at our hospital from January to December 2023. The authors documented the sex, age, surgical characteristics, and history of chronic diseases for all patients, including those who developed subcutaneous effusion secondary to cerebrospinal fluid leakage.
Expert Rev Neurother
January 2025
Department of Paediatrics, Cambridge University Hospitals, Cambridge, UK.
Introduction: The seizures in Lennox-Gastaut syndrome are typically resistant to treatment. Seven antiseizure medications (ASMs) in the US (six in the UK/EU) are licensed for the treatment of seizures in LGS: lamotrigine, topiramate, rufinamide, clobazam, felbamate (not licensed in the UK/EU), cannabidiol and fenfluramine. Other options include neurostimulation, corpus callosotomy and dietary therapies, principally the ketogenic diet and its variants.
View Article and Find Full Text PDFLennox-Gastaut syndrome (LGS) is a severe developmental and epileptic encephalopathy (DEE) characterized by multiple types of drug-resistant seizures (which must include tonic seizures) with classical onset before 8 years (although some cases with later onset have also been described), abnormal electroencephalographic features, and cognitive and behavioral impairments. Management and treatment of LGS are challenging, due to associated comorbidities and the treatment resistance of seizures. A panel of five epileptologists reconvened to provide updated guidance and treatment algorithms for LGS, incorporating recent advancements in antiseizure medications (ASMs) and understanding of DEEs.
View Article and Find Full Text PDFFront Neurol
December 2024
Department of Neurological Surgery, University of Pittsburgh School of Medicine, Pittsburgh, PA, United States.
Minimally invasive surgical techniques, such as MR-guided laser interstitial thermal therapy (LITT), have emerged as promising alternatives to open disconnective surgeries in drug-resistant epilepsy (DRE). This review synthesizes current literature on the application of LITT for corpus callosal disconnection and functional hemispheric disconnection. Studies highlight LITT's effectiveness for achieving seizure control and functional outcomes, often with reduced complications compared to traditional open procedures.
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