Cerebellar Hypoplasia in Werdnig-Hoffmann Disease

It is well established that the neuropathological changes in the Werdnig-Hoffmann disease are not confined to the spinal cord and brain stem, and that sensory as well as motor nuclei may be involved. In contrast to this wealth of degenerative change, reports of malformations in association with Werdnig-Hoffmann disease have been conspicuous by their absence, except for doubling of the central canal of the spinal cord in one of Werdnig's (1891) cases, and a few reports of minor neuronal heterotopias (Brandt, 1950). There is, however, an account by Radermecker and Liessens (1954) of a family in which the same sibship contained proved cases of Werdnig-Hoffmann disease and also a child who suffered from a non- progressive ataxia considered to be due to cere- bellar hypoplasia.