Adult onset leukodystrophy presents in the fourth or fifth decade of life and is characterized by early autonomic abnormalities, pyramidal and cerebellar dysfunction, and symmetric demyelination of the CNS.
On MRI patients have increased T2 signal in the upper corticospinal tract, cerebellar peduncles, with later development of confluent white matter changes in the frontoparietal area with relative sparing of the periventricular white matter