Description
This specimen shows two pieces of cerebellum; the grey matter appears intact but the white
matter is clearly abnormal, being pale in colour, and apparently collapsed. The subcortical U fibres
appear intact.
History
This child, who died at the age of two years, had suffered a progressive neurological
degenerative condition characterised by a gross ataxia and a lack of balance to such an extent
that she became upset when not adequately supported; she also showed a spastic quadriplegia
affecting her left side more than her right and her arms more than her legs. Histologically the
lesion was classified as sudanophilic leucodystrophy; another child in the family appears to be
affected in a similar manner.