Analysis of MRI patterns aids prediction of progression in X-linked adrenoleukodystrophy.
BACKGROUND
X-linked adrenoleukodystrophy (X-ALD) has variants with widely different outcomes, hampering clinical counseling and evaluation of therapies.


OBJECTIVE
To evaluate the degree to which MRI patterns can predict lesion progression.


METHODS
Two hundred six boys and men with cerebral X-ALD (median age 12.2 years, mean age 18.5 years, age range 1.7 to 73.8 years) were studied. In 140 individuals, follow-up MRI were available. Data after bone marrow transplantation (BMT) were excluded. The patterns of MRI abnormalities were subdivided into five groups based on the anatomic location of the initial T2 signal hyperintensity (pattern 1: parieto-occipital white matter, pattern 2: frontal white matter, pattern 3: corticospinal tract, pattern 4: cerebellar white matter, pattern 5: concomitant parieto-occipital and frontal white matter). The X-ALD MRI Severity Scale, a 34-point scale previously described, was used in the analysis.


RESULTS
Pattern 1 patients had rapid progression if contrast enhancement was present and if the MRI abnormality manifested at an early age. The latter was also true for pattern 2 patients. Based on these variables, predictive formulas were constructed for these two patterns using multiple regressions. MRI progression was much slower in pattern 3 and 4 patients, whereas in the few pattern 5 patients, it was more rapid than in any other of the patterns. Patterns 1 and 5 occurred mainly in childhood, patterns 2 and 4 in adolescence, and pattern 3 in adults.


CONCLUSIONS
MRI progression in X-ALD depends on patient age, initial MRI Severity Scale score, and anatomic location of the lesion. When used in combination, these data aid the prediction of disease course and the selection of patients for BMT.
Loes
D J
DJ
Suburban Radiology Consultant Ltd., Minneapolis, MN, USA.
Fatemi
A
A
Melhem
E R
ER
Gupte
N
N
Bezman
L
L
Moser
H W
HW
Raymond
G V
GV
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000175:diagnosis* / Q000188:drug therapy; D000328:Adult; D000367:Age Factors; D000368:Aged; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004338:Drug Combinations; D004883:Erucic Acids / Q000627:therapeutic use; D005500:Follow-Up Studies; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007223:Infant; D016014:Linear Models; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011379:Prognosis; D012720:Severity of Illness Index; D014304:Triolein / Q000627:therapeutic use
D004338:Drug Combinations; D004883:Erucic Acids; C079420:Lorenzo's oil; D014304:Triolein