[Agyria--a clinico-pathological study (author's transl)]

Agyria is an extremely rare malformation of the brain caused by an arrest of neuronal migration towards the cortex. A case of nearly complete agyria is presented in a 2 8/12 year-old boy. The autopsy revealed complete absence of cerebral convolutions in the frontal, parietal and occipital lobes of both hemispheres and discrete convolutions in the temporal lobes. The cortex reached up to 22 mm and the central white matter was very thin, occipitally up to 2 mm. Additionally, hydrocephalus internus was observed. Clinical and pathoanatomical findings are presented and compared with other data published in the literature. The results are discussed in relation to a possible pathogenetic mechanism leading to this cerebral malformation.