West Syndrome

West Syndrome

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 production

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West 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.