RT Journal Article
SR Electronic
T1 Adrenoleukodystrophy: a scoring method for brain MR observations.
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 1761
OP 1766
VO 15
IS 9
A1 D J Loes
A1 S Hite
A1 H Moser
A1 A E Stillman
A1 E Shapiro
A1 L Lockman
A1 R E Latchaw
A1 W Krivit
YR 1994
UL http://www.ajnr.org/content/15/9/1761.abstract
AB PURPOSE To develop a scoring method for brain observations in patients with X-linked adrenoleukodystrophy. METHODS One hundred seventy-five brain MR scans in 83 male subjects less than 20 years of age with proved biochemical defects were reviewed. A severity score (0 to 34), based on a point system derived from location and extent of disease and the presence of focal and/or global atrophy, was calculated for each exam. RESULTS Fifty-five of the 83 patients showed MR findings consistent with adrenoleukodystrophy. Two major patterns were observed. A posterior pattern (mean score, 9; range, 0.5 to 25) was present in 80% of patients, and an anterior pattern (mean score, 10; range, 2 to 18) was present in 15% of patients. Serial MR imaging, positive for adrenoleukodystrophy in 34 patients (mean follow-up, 23 months; range, 2 months to 6 years 11 months), showed progressive disease in 52%, progressive disease with subsequent stabilization in 18%, stable disease in 24%, and minimal improvement in 6%. CONCLUSION The adrenoleukodystrophy MR severity scoring method is a measure that can be used with standard MR images. When used in conjunction with clinical parameters, this scoring method may help define better the natural history of adrenoleukodystrophy and monitor response to developing therapies.