Magnetic resonance imaging detection of lesion progression in adult patients with X-linked adrenoleukodystrophy.
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.


METHODS
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.
Eichler
Florian
F
Department of Neurology, Martinos Center for Biomedical Imaging, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Mahmood
Asif
A
Loes
Daniel
D
Bezman
Lena
L
Lin
Doris
D
Moser
Hugo W
HW
Raymond
Gerald V
GV
D000326:Adrenoleukodystrophy / Q000473:pathology* / Q000503:physiopathology; D000328:Adult; D001931:Brain Mapping; D003711:Demyelinating Diseases / Q000209:etiology / Q000473:pathology; D018450:Disease Progression; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011712:Pyramidal Tracts / Q000473:pathology; D012189:Retrospective Studies