Childhood cerebral form of adrenoleukodystrophy: short-term effect of bone marrow transplantation on brain MR observations.
OBJECTIVE
To report the serial brain MR observations in patients with childhood-onset cerebral adrenoleukodystrophy 1 to 2 years after bone marrow transplantation.


METHODS
Eight boys with childhood-onset cerebral adrenoleukodystrophy have undergone successful transplantation at our institution. Seven patients (mean age, 8 years 10 months; range, 5 years 3 months to 11 years 9 months) had serial MR studies before and after transplantation. An MR severity score (0 to 34) based on disease location and the presence or absence of focal atrophy was calculated for each patient scan.


RESULTS
Posttransplantation serial MR showed improvement in two patients, stabilization in three patients, and worsening of MR signal changes in two patients. The patient with the most striking progression had systemic graft-versus-host disease. Although the adrenoleukodystrophy MR severity score did not change in three patients after transplantation, two of these patients did show improved margination of disease.


CONCLUSIONS
Bone marrow transplantation can affect brain MR observations in childhood-onset cerebral adrenoleukodystrophy. Although brain MR findings do not typically resolve, they do seem to stabilize, which is an improvement over the natural MR history of the disease.
University of Minnesota Hospital and Clinic, Department of Radiology, Minneapolis 55455.
D J
DJ
Loes
A E
AE
Stillman
S
S
Hite
E
E
Shapiro
L
L
Lockman
R E
RE
Latchaw
H
H
Moser
W
W
Krivit
D000326:Adrenoleukodystrophy / Q000175:diagnosis / Q000628:therapy*; D001284:Atrophy; D016026:Bone Marrow Transplantation / Q000473:pathology; D001921:Brain / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male