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AJNR - American Journal of Neuroradiology

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Assessing Disease Severity in Late Infantile Neuronal Ceroid Lipofuscinosis Using Quantitative MR Diffusion-Weighted Imaging

J.P. Dyke^a,b, H.U. Voss^a,b, D. Sondhi^c, N.R. Hackett^c, S. Worgall^d, L.A. Heier^b, B.E. Kosofsky^d, A.M. Ulu^b, D.C. Shungu^a,b, X. Mao^a,b, R.G. Crystal^c and D. Ballon^a,b

^a Citigroup Biomedical Imaging Center, Weill Medical College of Cornell University, New York, NY
^b Department of Radiology, Weill Medical College of Cornell University, New York, NY
^c Department of Genetic Medicine, Weill Medical College of Cornell University, New York, NY
^d Department of Pediatrics, Weill Medical College of Cornell University, New York, NY

View larger version (103K): [in this window] [in a new window]   Fig 1. A, A diffusion-weighted image (b = 1000 s/mm2) and an image acquired without diffusion weighting (B) show enlarged sulci and dilated ventricles consistent with atrophic changes from a representative patient with LINCL.

View larger version (18K): [in this window] [in a new window]   Fig 2. A whole-brain ADC histogram from a representative patient with LINCL shows the dual Gaussian and partial volume functions that were used to fit the whole brain, partial volume, and CSF compartments.

View larger version (39K): [in this window] [in a new window]   Fig 3. Voxels associated with specific ranges of ADC values provide visual confirmation that compartments identified as whole brain, partial volume, and CSF correspond with specific regions as shown from a representative section of a patient with LINCL. ADC ranges displayed are described below. A, 1.0 ± 0.2 x 10–3 mm2/s. B, 1.5 ± 0.2 x 10–3 mm2/s. C, 2.0 ± 0.2 x 10–3 mm2/s. D, 2.5 ± 0.2 x 10–3 mm2/s. E, 3.0 ± 0.2 x 10–3 mm2/s. F, 3.5 ± 0.2 x 10–3 mm2/s.

View larger version (12K): [in this window] [in a new window]   Fig 4. The whole-brain ADC value is plotted versus age for all of the patients showing an increasing trend over time. Serial studies are connected by lines, and the CNS disability scale is shown in the legend. Previously published whole-brain ADC values from age-matched control subjects are plotted for comparison.