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AJNR Am J Neuroradiol. 2016 Feb;37(2):367-72. doi: 10.3174/ajnr.A4500. Epub 2015 Oct 1.

Intensity of MRI Gadolinium Enhancement in Cerebral Adrenoleukodystrophy: A Biomarker for Inflammation and Predictor of Outcome following Transplantation in Higher Risk Patients.

Author information

1
From the Division of Pediatric Blood and Marrow Transplant (W.P.M., T.C.L., P.J.O.), Department of Pediatrics mill4991@umn.edu.
2
Division of Hematology and Oncology (L.F.M.), Department of Pediatrics, University of Sao Paulo, Sao Paulo, Brazil.
3
University of Minnesota Medical School (J.M.), Minneapolis, Minnesota.
4
Departments of Diagnostic Radiology (J.B.R., R.S.G., D.R.N.).
5
From the Division of Pediatric Blood and Marrow Transplant (W.P.M., T.C.L., P.J.O.), Department of Pediatrics.
6
Biostatistics Core (R.M.S.), Masonic Cancer Center, University of Minnesota, Minneapolis, Minnesota.
7
Neurology (G.V.R.), University of Minnesota Medical Center, Minneapolis, Minnesota.

Abstract

BACKGROUND AND PURPOSE:

Outcomes following hematopoietic stem cell transplantation for higher risk childhood-onset cerebral adrenoleukodystrophy are variable. We explored whether a brain MR imaging gadolinium intensity scoring system improves prediction of neurologic outcome.

MATERIALS AND METHODS:

We developed a 4-point scale of gadolinium intensity relative to the choroid plexus: 0 = no enhancement; 1 = hypointense; 2 = isointense; 3 = hyperintense. The interobserver concordance of the scale was assessed on 30 randomly chosen studies. Scores were generated for 64 evaluable patients and compared with CSF chitotriosidase levels, a known inflammatory marker correlating with outcomes following transplantation. For 25 evaluable higher risk patients (Loes ≥10), the gadolinium intensity score was compared with longer term posttransplantation clinical change.

RESULTS:

The gadolinium intensity scoring system showed good interobserver reproducibility (κ = 0.72). Of 64 evaluable boys, the score positively correlated with average concomitant CSF chitotriosidase activity in nanograms/milliliter/hour: 0: 2717, n = 5; 1: 3218, n = 13; 2: 6497, n = 23; and 3: 12,030, n = 23 (P < .01). For 25 evaluable higher risk patients, more intense pretransplantation brain MR imaging gadolinium enhancement predicted greater average loss on the adrenoleukodystrophy neurologic function scale following transplantation: 0/1: adrenoleukodystrophy neurologic function scale score difference = 4.3, n = 7; 2/3: adrenoleukodystrophy neurologic function scale score difference = 10.4, n = 18 (P = .05).

CONCLUSIONS:

Gadolinium enhancement intensity on brain MR imaging can be scored simply and reproducibly for cerebral adrenoleukodystrophy. The enhancement score significantly correlates with chitotriosidase. In boys with higher risk cerebral disease (Loes ≥10), the enhancement score itself predicts neurologic outcome following treatment. Such data may help guide treatment decisions for clinicians and families.

PMID:
26427835
PMCID:
PMC5177792
DOI:
10.3174/ajnr.A4500
[Indexed for MEDLINE]
Free PMC Article

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