Evaluation of Neurodegeneration in a Mouse Model of INCL by MRI and Spectroscopic Analyses
Heffer A, Munasinghe J, Mukherjee A, Koretsky A, Hofmann S, Zhang Z
National Institutes of Health
Infantile Neuronal Ceroid Lipofuscinosis (INCL) is a childhood neurodegenerative storage disorder. Although children with INCL are normal birth, they are blind by two years of age and brain-dead by four. Rapid brain atrophy and altered chemical composition are characteristic of INCL. Using a mouse model of INCL that mimics the human disease, we analyzed the brains of these mice at 3, 4, 5 and 6 months of age using quantitative MRI, T2, ADC, relative cerebral blood flow, cerebral blood volume, Gd2+ infusion and MRS. The results suggest that these methods are useful in evaluating novel therapeutic interventions in this disease.