[On pathology of the diffuse pachygyria (author's transl)]

A case report on a 4 year old boy with physical and mental retardation noted since the age of 7 months is presented. In the 10th month a hydrocephalus internus was diagnosed. The autopsy showed a cerebral dystrophy with diffuse pachygyria presumably caused by a disturbance in the neuroblast migration during the 2nd and the 5th month of gravidity. The 33 cases of diffuse pachygyria hitherto described in the literature are compared with our own one. In more than 80% of the cases pachygyria is associated with oligophrenia or idiotism, resp., and 75% with epilepsy. Paralysis is rarely observed. The average life expectancy is about 5 years. Etiologic factors are intrauterine hypoxemia and infectious diseases during the early pregnancy period as well as hereditary afflictions. In the case described here the teratogenic action of a drug probably was the etiologic factor.