Chaos, balance, and development: thoughts on selected childhood epilepsy syndromes.
Age-specific epilepsy syndromes raise important questions about developmental susceptibility to seizures and epileptogenesis and about the effect of seizures on function. The diagnosis and treatment of these syndromes has been enhanced by the use of modern science and technology. Epidemiologic studies have changed our approach to febrile convulsions. This developmental seizure disorder is benign and self-limited. We have been forced to think carefully about threshold, therapy, and whether other seizures in childhood may be equally benign. This framework of developmental specificity can also be applied to West syndrome, especially with respect to neurophysiology, neurochemistry, neuroimaging, and epidemiology--the types of seizures, clustering, variations associated with sleep, PET scans, and therapy. Rasmussen's syndrome and other unilateral developmental epilepsies are progressive but remain confined to a single hemisphere. However, they usually are devastating to global neurologic function. They are models for examining the impact of epilepsy in one pathologic hemisphere on the function of the entire brain. Current therapy for this condition is hemispherectomy. Recovery of function after this major surgery is striking and provides clues to brain organization. The analysis of these three syndromes provides windows on the dynamic, changing central nervous system of the child and may lead to better understanding and therapy for other seizure disorders.
Vining
E P
EP
Department of Neurology and Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD.
D001921:Brain / Q000254:growth & development / Q000503:physiopathology; D002648:Child; D004660:Encephalitis / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D004828:Epilepsies, Partial / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D007223:Infant; D003294:Seizures, Febrile / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D013036:Spasms, Infantile / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy