Neocerebellar hypoplasia with systemic combined olivo-ponto-dentatal degeneration in a 9-day-old baby: contribution to the problem of relations between malformation and systemic degeneration in early life.

Three different cerebral alterations, apparently formed consecutively, were observed in a 9-day-old baby. Marked cortico-neocerebellar hypoplasia was seen in a relatively well-developed paleocortex. Its teratological stage was apparently the 3rd fetal month. Almost total nerve cell loss and marked proliferation of protoplasmic and fibrous astrocytes were found in the nuclei pontis and inferior olive. Perihypoglossal and pararaphal nuclei, which are related to the cerebellum, were also affected. This degenerative process must have resulted from neuronal deprivation or inactivity, as the pertinent cortico-cerebellar area was hypoplastic, and therefore any neuronal imput was impossible (olivo-ponto-dentatal degeneration due to cortico-cerebellar hypoplasia). Massive symmetrical necrosis in the cerebral white and grey matter, basal ganglia, midbrain and bulbus, is interpreted as hypoxic damage due to perinatal convulsive attacks and cessation of respiration.