Tominaga I, Yanai K, Kashima H, Kato Y, Sekiyama S, Yokochi A, Miura I
Rev Neurol (Paris). 1986;142(5):524-9.
Clinico-pathological studies of West syndrome are rather rare. A case of sequelae of acute encephalopathy which involved a nine-month-old boy with post-mortem data is reported. Birth and postnatal development had been normal until the onset of illness. Laboratory examinations ruled out bacterial or viral meningo-encephalitis and metabolic disorders. After the recovery from a coma lasting several days, spastic quadriplegia, severe mental retardation and intractable epileptic attacks were present. The latter were made of tonic spasms and myoclonic seizures. EEG records showed hypsarrhythmia. Neuropathological examination revealed almost symmetrical bilateral cystic cavities in the pontine tegmentum and lateral nuclei of both thalami. The corpus callosum was very thin. No finding suggested a congenital anomaly. In a search of the pathological basis for infantile spasms, this case was compared with the published data. It would appear that the lesions of the pontine tegmentum play a significant role in the pathogenesis of hypsarrhythmia or infantile spasms.
