Involvement of the pontomedullary corticospinal tracts: a useful finding in the diagnosis of X-linked adrenoleukodystrophy.

PURPOSE To determine whether pontomedullary corticospinal tract involvement is a common and specific finding of adrenoleukodystrophy on MR images.

METHODS MR images of 10 patients with biochemically proved adrenoleukodystrophy who were examined during the last 6 years were reviewed retrospectively to determine the frequency of corticospinal tract involvement in the medulla, pons, mesencephalon, internal capsules, and corona radiata. MR images of 10 patients with other leukodystrophies (three with Krabbe disease, two with Alexander disease, two with metachromatic leukodystrophy, two with Pelizaeus-Merzbacher disease, and one with Canavan disease) were reviewed with specific attention to the pontomedullary corticospinal tracts.

RESULTS Medullary and pontine corticospinal tract involvement was present in eight of the 10 patients with adrenoleukodystrophy. Mesencephalic and internal capsular involvement was present in three patients. The coronal radiata portion of the corticospinal tracts was not involved in any of the 10 patients. No pontomedullary corticospinal tract involvement was identified in any of the 10 patients with other leukodystrophies. The difference in the frequency of pontomedullary corticospinal tract involvement between the two groups was highly significant.

CONCLUSION Pontomedullary corticospinal tract involvement is a common finding in adrenoleukodystrophy and is unusual in other leukodystrophies. Awareness of this finding can facilitate the radiologic diagnosis of this disease and may expedite management of affected patients.