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Abstract #2097

Spatial Gradient of White Matter Microstructure Discriminates Progressive vs Non-Progressive Cerebral Adrenoleukodystrophy

Eric Mallack1, Aoife Jeffries2, Jie Lin3, Stephanie van de Stadt4, Marc Engelen5, Otto A Rapalino 6, Patricia L Musolino7, Florian S Eichler7, and Sumit Narayan Niogi3
1Kennedy Kreiger Institute, Johns Hopkins University School of Medicine, Baltimore, MD, United States, 2Northeastern University, Boston, MA, United States, 3Department of Radiology, Weill Cornell Medicine, New York, NY, United States, 4Department of Pediatric Neurology, Amsterdam University Medical Centers, Amsterdam, Netherlands, 54Department of Pediatric Neurology, Amsterdam University Medical Centers, Amsterdam, Netherlands, 6Department of Radiology, Harvard Medical School, Boston, MA, United States, 7Department of Neurology, Harvard Medical School, Boston, MA, United States

Synopsis

Keywords: Rare Disease, Rare disease

Motivation: The emergence of demyelination, prediction of progression, appropriate disease-staging, and neurological prognosis post-treatment for cerebral adrenoleukodystrophy are significant challenges.

Goal(s): Quantify the spatial change in white matter microstructure across a lesion into distal normal appearing white matter to discriminate between disease progression vs arrest.

Approach: A region of interest analysis was performed along the corpus callosum from proximal to the lesion to the distal end in 36 DTI datasets. The slope of change in FA was calculated and compared between progressors vs non-progressors.

Results: There is a significant difference between the FA slope of the progressor group vs non-progressors.

Impact: This analytic approach demonstrated differences in change in microstructure between patients with ongoing disease vs those with disease arrest, indicating utility as a clinical imaging tool to guide imaging surveillance, prognosis, and appropriate patient selection for treatment in cerebral adrenoleukodystrophy.

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Keywords