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Florian Eichler, MD; Asif Mahmood, MD; Daniel Loes, MD; Lena Bezman, MD; Doris Lin, MD; Hugo W. Moser, MD; Gerald V. Raymond, MD

Arch Neurol. 2007;64(5):659-664.

Background  An inherited disorder, X-linked adrenoleukodystrophy (X-ALD) is known to cause progressive inflammatory demyelination.

Objective  To analyze the adult pattern of disease progression in X-ALD.

Design, Setting, and Patients  We retrospectively assessed magnetic resonance (MR) images obtained in adult patients who had developed cerebral disease between January 1, 1985, and December 31, 2005. We identified 103 adult patients with X-ALD with lesions on their MR images. Of these, 56 had serial MR examinations at least 1 year apart and were included in this study.

Main Outcome Measure  Progression of X-ALD lesions on MR images.

Results  On initial presentation, 17 patients with X-ALD had corticospinal tract lesions without splenium or genu involvement, 24 had symmetric corticospinal tract lesions with additional involvement of the splenium or genu, and 15 did not have corticospinal tract involvement but had other white matter lesions. In 18 of 21 patients with progressive lesions, corticospinal tract involvement preceded or occurred concurrently with progressive inflammatory demyelination.

Conclusions  Brain MR imaging abnormalities in adults with X-ALD progress slower than those reported in childhood. The involvement of the corticospinal tracts is prominent and may at times represent a variant course of progressive inflammatory demyelination.

Author Affiliations: Department of Neurology and Martinos Center for Biomedical Imaging, Massachusetts General Hospital, Harvard Medical School, Boston (Dr Eichler); Kennedy Krieger Institute (Drs Mahmood, Bezman, Moser, and Raymond) and Departments of Neurology and Pediatrics (Drs Mahmood, Bezman, Moser, and Raymond) and Radiology and Radiological Sciences (Dr Lin), The Johns Hopkins University, Baltimore, Md; and Suburban Radiological Consultants Ltd, Minneapolis, Minn (Dr Loes).
Deceased.