Epilepsy in Tuberous Sclerosis Complex
Tuberous Sclerosis Complex (TSC) is an autosomal dominant multisystem disorder, characterized by the presence of hamartomatous lesions involving different organ systems, including the brain. Epilepsy is the most common presenting symptom, representing a m
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Contents General Features ........................................................................................................................ Pathophysiology .............................................................................................................. Clinical Phenotype .......................................................................................................... Central Nervous System.................................................................................................. Medical Treatment .......................................................................................................... Epilepsy Surgery ........................................................................................................................ Presurgical Workup ......................................................................................................... Surgical Procedures......................................................................................................... Cognitive and Behavioral Aspects ............................................................................................. Cognitive Impairment ..................................................................................................... Behavioural Sequelae ...................................................................................................... Conclusion Remarks .................................................................................................................. References ..................................................................................................................................
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Abstract Tuberous Sclerosis Complex (TSC) is an autosomal dominant multisystem disorder, characterized by the presence of hamartomatous lesions involving different organ systems, including the brain. Epilepsy is the most common presenting symptom, representing a major source of morbidity and mortality. Despite multiple antiepileptic drug combinations, in about two thirds of cases the patients present high-frequency drug-resistant epilepsy, and nonpharmacologic options may be considered. The aim of this work was to point out the current knowledge on epileptogenesis in TSC, the available medical therapies and diagnostic tools, and possible surgical strategies, with the intent to better understand the actual difficulties in controlling seizures and the results reported in the literature. There is also a section
F. Novegno, M.D. (*) • L. Massimi, M.D. • C. Di Rocco, M.D. Department of Pediatric Neurosurgery, Catholic University Medical School, Largo A. Gemelli 1, 00168 Rome, Italy e-mail: [email protected] N. Akalan, C. Di Rocco (eds.), Pediatric Epilepsy Surgery, Advances and Technical Standards in Neurosurgery, DOI 10.1007/978-3-7091-1360-8_6, © Springer-Verlag Wien 2012
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dedicated to the common association with cognitive impairment and the role of epilepsy control on its o
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