Abstract
An inherited disorder, X-linked adrenoleukodystrophy (X-ALD) is known to cause progressive inflammatory demyelination. To analyze the adult pattern of disease progression in X-ALD. 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. 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. 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.
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.