Adrenoleukodystrophy: a scoring method for brain MR observations.
OBJECTIVE
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%.


CONCLUSIONS
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.
University of Minnesota Hospital and Clinic, Department of Radiology, Minneapolis 55455.
D J
DJ
Loes
S
S
Hite
H
H
Moser
A E
AE
Stillman
E
E
Shapiro
L
L
Lockman
R E
RE
Latchaw
W
W
Krivit
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000145:classification / Q000175:diagnosis*; D000328:Adult; D001284:Atrophy; D001921:Brain / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D003337:Corpus Callosum / Q000473:pathology; D005500:Follow-Up Studies; D005625:Frontal Lobe / Q000473:pathology; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009434:Neural Pathways / Q000473:pathology; D011149:Pons / Q000473:pathology; D013116:Spinal Cord / Q000473:pathology