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 progessive cerebellar ataxia and spastic paraparesis. CT showed atrophy of the cerebellum and brain stem. MRI disclosed lesion s, enhancing partially with gadolinium, in the dentate nuclei and the pyramidal tracts from the internal capsule to the brain stem. The bioc hemical findings were compatible with typical adrenoleukodystrophy, bu t the distribution of the lesions on MRI was distinctly different. Aft er steroid administration, the patient's symptoms improved slightly an d contrast enhancement of the lesions was markedly reduced.