We report a case with hemimegalencephaly and catastrophic epilepsy treated early at 4 months by functional hemispherectomy. The boy had intractable continuous epilepsy, with seizures every 10 min, hemiparesis and absence of psychomotor acquisition. Three years after hemispherectomy, the boy is seizure free and has a mild psychomotor delay. Hemiparesis and hemianopsia are unchanged. Early hemispherectomy, before 6 months of age, may control severe epilepsy and preserve the development of higher cortical functions in the nonhemimegalencephalic hemisphere.
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http://dx.doi.org/10.1159/000121265 | DOI Listing |
Neuropediatrics
January 2025
Neonatology, Leiden University, Leiden, Netherlands.
Background Hemimegalencephaly (HME) is a rare congenital disorder that is initiated during embryonic development with abnormal growth of one hemisphere. Tuberous sclerosis complex (TSC), a genetic disorder, is rarely associated with HME. Methods We present a case of a newborn with HME with a confirmed mutation in the TSC-1 gene and describe the clinical course, findings on (amplitude integrated) electroencephalography (aEEG), cranial ultrasound (CUS), MRI, and the postmortem evaluation.
View Article and Find Full Text PDFSeizure
November 2024
Surgical Division of Epilepsy Center, Shenzhen Children's Hospital, Shenzhen, China; Neurology Department, Shenzhen Children's Hospital, Shenzhen, China. Electronic address:
Objective: To investigate the clinical outcomes and safety of surgery in infants (< 12 months of age) with drug-resistant epilepsy, clarify surgical indications, and select appropriate surgical methods.
Methods: This was a retrospective analysis of infants with drug-resistant epilepsy who underwent epilepsy surgery and were followed up for > 6 months at the Epilepsy Center of Shenzhen Children's Hospital. Clinical data included etiology, seizure type, surgical procedure, preoperative auxiliary examinations, pathological findings, and intraoperative and postoperative complications.
Radiol Case Rep
November 2024
Loma Linda University Medical Center, Department of Radiology, 11234 Anderson St, Loma Linda, CA 92354, USA.
Rasmussen's encephalitis (RE) is a progressive inflammatory neurological process most commonly occurring in children characterized by drug-resistant focal epilepsy, hemiplegia, and cognitive decline, with brain atrophy and white matter changes typically localized to 1 hemisphere of the brain. Because the clinical course of RE is often indistinguishable from a variety of medical conditions, MRI has historically been the primary diagnostic tool. Here, we report both the clinical course and progression of neuroimaging findings of a 5-year-old female who had very subtle early cortical and white matter changes on MRI and was diagnosed with RE by correlating the clinical presentation, imaging, and electrographic findings.
View Article and Find Full Text PDFNeurology
July 2024
From the Department of Neurosurgery (S.W., X.W., J. Zhou, G.L., Y.G.), SanBo Brain Hospital, Capital Medical University; Department of Neurosurgery (S.W., K.Z., J. Zhang), Beijing Tiantan Hospital, Capital Medical University; Pediatric Epilepsy Center (Q.-Z.L., L.C.), Peking University First Hospital, Beijing; Department of Neurosurgery (R.Z.), Children's Hospital of Fudan University, Shanghai; Department of Neurosurgery (X.Z.), Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders; Epilepsy Center (S. Li), Guangdong Sanjiu Brain Hospital, Guangzhou; Department of Neurosurgery (Z.Y.), Xiangya Hospital, Central South University, Changsha, Hunan; Department of Neurosurgery (Y.S.), Xuanwu Hospital, Capital Medical University; Department of Neurosurgery (K.M., Y.L.), Capital Institute of Pediatrics, Beijing; Department of Neurosurgery (J.H.), Huashan Hospital, Fudan University, Shanghai; Department of Epilepsy Center (L.S.), The Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangdong; Department of Neurosurgery (H.C.), The First Affiliated Hospital of Anhui Medical University, Hefei; Department of Neurology (X.L.), Affiliated ZhongDa Hospital, Southeast University, Nanjing, Jiangsu; Department of Neurology (J.S.), Affiliated Children's Hospital of Xi'an Jiaotong University, Shaanxi; Department of Neurosurgery (M.Z.), Henan Sanbo Brain Hospital, Zhengzhou; Department of Neurology (M.W., T.L.), SanBo Brain Hospital; and Department of Functional Neurosurgery (J. Zhang), Beijing Neurosurgical Institute, Capital Medical University; Beijing Key Laboratory of Neurostimulation (J. Zhang); Functional Neurosurgery Department (S. Liang), Beijing Children's Hospital, Capital Medical University, National Center for Children's Health; Beijing Key Laboratory of Epilepsy (G.L., Y.G.); and Center of Epilepsy (G.L., Y.G.), Beijing Institute of Brain Disorders, Collaborative Innovation Center for Brain Disorders, Capital Medical University, China.
Background And Objectives: Surgery is widely performed for refractory epilepsy in patients with Sturge-Weber syndrome (SWS), but reports on its effectiveness are limited. This study aimed to analyze seizure, motor, and cognitive outcomes of surgery in these patients and to identify factors associated with the outcomes.
Methods: This was a multicenter retrospective observational study using data from patients with SWS and refractory epilepsy who underwent epilepsy surgery between 2000 and 2020 at 16 centers throughout China.
J Med Genet
August 2024
Department of Neurosurgery, KU Leuven University Hospitals, Leuven, Belgium.
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