Presentation
Ataxic gait since childhood
Patient Data
The cerebellum is globally reduced in size with widening of the cerebellar sulci. The supratentorial brain, brainstem and cervical cord appear unremarkable.
Case Discussion
The normal shape of the cerebellum with global volume reduction is seen, consistent with global cerebellar hypoplasia; a type of congenital morphological cerebellar abnormality. An identical appearance can be seen in diffuse cerebellar atrophy, however, the long-standing history suggests that this is hypoplasia. Only long-term imaging showing stability over an extended time period can categorically distinguish between these two entities.