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EncyclopediaSeeinfantile spasmWest syndrome
West syn·drome (west), an encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarrhythmia.
West syn·drome (west), an encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarrhythmia.An early onset condition characterised by a triad:
(1) Infantile spasms,
(2) Interictal EEG pattern termed hypsarrhythmia, and
(3) Mental retardation. Per the international classification, the diagnosis can be made in the presence of two of the three symptoms. The frequent onset of West syndrome in infancy suggests that CNS immaturity may play a key role in its pathogenesis. The clinical response to ACTH and glucocorticoids may be linked to suppression of corticotropin-releasing hormone productionWest syndrome Massive myoclonus Neurology An occasionally X-linked condition characterized by infantile spasms–seizures and 2º generalized epilepsy, hypsarrhythmia, encephalopathy with mental retardation and arrested psychomotor development, ± with immune dysfunction and death at an early age due to bronchopneumonia. See Salaam convulsions. West syn·drome (west sin'drōm) An encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarrhythmia. West, Charles, English physician, 1816-1898. West syndrome - an encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarhythmia. |