MR imaging appearance of childhood adrenoleukodystrophy with auditory, visual, and motor pathway involvement.

M. E. Jensen, R. W. Sawyer, I. F. Braun, W. B. Rizzo

Research output: Contribution to journalArticle

19 Scopus citations

Abstract

Childhood adrenoleukodystrophy is an X chromosome-linked disorder characterized by progressive demyelination of cerebral white matter and adrenal insufficiency. Magnetic resonance (MR) imaging was performed in 15 patients with symptomatic disease and three with presymptomatic disease. MR imaging findings were abnormal only in symptomatic patients. Major sites of disease were the occipital, parietal, and temporal lobes, with all patients showing involvement of occipital lobes, optic radiations, and splenium of the corpus callosum. Follow-up images obtained in four patients demonstrated a posterior-to-anterior progression of disease. Correlation of results from MR imaging with those from evoked potential studies indicated that MR imaging was the method of choice for detecting demyelination of visual, auditory, and motor systems in adrenoleukodystrophy.

Original languageEnglish (US)
Pages (from-to)53-66
Number of pages14
JournalRadiographics : a review publication of the Radiological Society of North America, Inc
Volume10
Issue number1
DOIs
StatePublished - Jan 1990

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

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