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Involvement of the pontomedullary corticospinal tracts: a useful finding in the diagnosis of X-linked adrenoleukodystrophy. AJNR Am J Neuroradiol 1997 Jan;18(1):95-100



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Scopus ID

2-s2.0-0031032029   33 Citations


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.

Author List

Barkovich AJ, Ferriero DM, Bass N, Boyer R


Nancy Bass MD Professor in the Neurology department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Child, Preschool
Diagnosis, Differential
Magnetic Resonance Imaging
Medulla Oblongata
Neurologic Examination
Peroxisomal Disorders
Pyramidal Tracts
Retrospective Studies
Sex Chromosome Aberrations
X Chromosome