MRI of adrenoleukodystrophy involving predominantly the cerebellum and brain stem

A case of adult-onset adrenoleukodystrophy involving predominantly the cerebellum and brain stem is described. The patient was a 30-year-old man with no family history of adrenoleukodystrophy. His neurological symptoms were progressive cerebellar ataxia and spastic paraparesis. CT showed atrophy of the cerebellum and brain stem. MRI disclosed lesions, enhancing partially with gadolinium, in the dentate nuclei and the pyramidal tracts from the internal capsule to the brain stem. The biochemical findings were compatible with typical adrenoleukodystrophy, but the distribution of the lesions on MRI was distinctly different. After steroid administration, the patient's symptoms improved slightly and contrast enhancement of the lesions was markedly reduced.