Objective Proteus syndrome is a rare overgrowth disorder including bone, soft tissue, and skin. Central nervous system manifestations were reported in about 40% of the patients including hemimegalencephaly and the resultant hemicranial hyperplasia, convulsions and mental deficiency. We report a 1-month-old male baby referred to Pediatric Neurology Clinic Soetomo Hospital, Surabaya, Indonesia in 2014 presented recurrent seizures since birth with asymmetric dysmorphic face with the right side larger than the left, subcutaneous mass and linear nevi. Craniocervical MRI revealed hemimegalencephaly right cerebral hemisphere. Triple antiepileptic drugs were already given as well as the ketogenic diet, but the seizures persisted. The seizure then was resolved after hemispherectomy procedure.
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Daily executive functioning in adults with pediatric hemispherectomy.
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Fuller Graduate School of Psychology, Travis Research Institute, Pasadena, CA 91101, United States; International Research Consortium for the Corpus Callosum and Cerebral Connectivity (IRC5), Pasadena, CA 91106, United States; California Institute of Technology, Division of Humanities and Social Sciences, Pasadena, CA 91125, United States. Electronic address:
Background And Aims: For young children with intractable epilepsy caused by congenital abnormalities or acquired cortical lesions, pediatric hemispherectomy surgery (pHS) may offer the only path to seizure remediation. Although some sensory and motor outcomes of pHS are highly predictable, the long-term cognitive and functional sequelae of pHS are far more variable. With the aim of identifying potential post-pHS intervention targets, the current study examined daily executive functioning and self-awareness in adults with pHS and broadly intact cognitive outcomes (indicated by average or above performance on intelligence tests).
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Epileptic spasms (ES) are a unique seizure type typically presenting in the form of infantile epileptic spasms syndrome (IESS) with characteristic hypsarrhythmia on scalp EEG and a preponderance with developmental delay or regression. While pharmacotherapy is the mainstay of treatment, surgical options, including disconnective or resective procedures, are increasingly recognized as viable therapeutic options for recurrent or persistent ES. However, limited data on safety, effectiveness, and prognostic factors hinder informed decision-making regarding surgery indications, timing, and intervention type.
View Article and Find Full Text PDFEpileptiform discharges in the context of self-limited pediatric focal epilepsy (EDSelFEC) in pediatric hemispherotomy patients: Role of white matter abnormalities.
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Center for Pediatric Neurology and Neurorehabilitation, Epilepsy Center for Children and Adolescents, Schoen Clinic Vogtareuth, Vogtareuth, Germany.
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Department of Neurosurgery, SanBo Brain Hospital, Capital Medical University, Beijing, 100093, People's Republic of China.
Article Synopsis
- Sturge-Weber syndrome (SWS) is a rare neurological and skin disorder often linked to drug-resistant epilepsy, which this study investigates in relation to HMGB1 protein translocation.
- The research involved eight SWS patients who underwent hemispherectomy; brain tissue analysis showed increased levels of cytoplasmic HMGB1, particularly in microglia and neurons, while astrocytes mainly retained nuclear localization.
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