Jonathan P. Dyke1,
Dolan Sondhi2, Henning Voss1, Dikoma C. Shungu1,
Xiangling Mao1, Kaleb Yohay3, Stefan Worgall3,
Neil Hackett2, Charleen Hollmann2, Mary Yeotsas2,
Annie Jeong2, Benjamin van de Graaf2, Ida Cao2,
Stephen Kaminsky2, Linda Heier1, Kyle Rudser4,
Mark Souweidane5, Michael Kaplitt5, Barry Kosofsky3,
Ronald Crystal2, Douglas Ballon1
1Radiology,
Weill Cornell Medical College, NY, United States; 2Genetic
Medicine, Weill Cornell Medical College, NY, United States; 3Pediatrics,
Weill Cornell Medical College, NY, United States; 4Division of
Biostatistics, University of Minnesota, Minneapolis, MN, United States; 5Neurological
Surgery, Weill Cornell Medical College, NY, United States
A quantitative noninvasive MR imaging based disease severity score for late infantile neuronal ceroid lipofuscinosis has been presented. The metric combines data from brain-water apparent diffusion coefficients, the volume percentage of CSF, and N-acetylaspartate-to-creatine metabolite ratios. The methods used used can be adapted to run on multiple scanner platforms in a straightforward manner.
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