Cerebellar hypoplasia associated with systemic degeneration in early life.

Hypoplasia predominantly affecting the lateral lobes of the cerebellum has on several occasions been found associated with marked underdevelopment of the griseum pontis and middle peduncle (Vogt and Astwazaturow, 1912; Brun, 1917, 1918; Brouwer, 1924; Biemond, 1955). Brouwer named this condition " hypoplasia ponto-neocerebellaris " and emphasized the fact that the same neuronal system was affected in this prenatal malformation as in olivo-ponto-cerebellar atrophy. Support was thus given to the theory already put forward by Winkler (1923) that a congenital weakness of the derivatives of the cell bands of Essick (1912) underlay the brain-stem degenerations of olivo-pontocerebellar atrophy. The two cases described in this paper take this comparison a stage further. Both are examples of cerebellar hypoplasia occurring in infants who died during the first year of life. Detailed examination of the brain-stem in the first case has revealed a much closer resemblance to olivoponto-cerebellar atrophy than has hitherto been suspected. More remarkable, however, is that in both our cases there is evidence of degeneration having taken place in certain other nuclei known to be liable to involvement in the systemic degenerations of later life. A bond has thus been disclosed linking malformation with abiotrophy.