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Abstract #3385

Diffusion Tensor Imaging in Patients with Hallervorden-Spatz Syndrome and Their Siblings

Rishi Awasthi1, Richa Trivedi1, Vimal K. Paliwal2, Jitesh K. Singh3, Ram K S Rathore3, Rakesh K. Gupta1

1Department of Radiodiagnosis, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India; 2Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India; 3Department of Mathematics and Statistics, Indian Institute of Technology, Kanpur, India

DTI was performed on 4 patients with Hallervorden Spatz syndrome (HSS), their siblings (n=2) and age matched controls (n=5). Fractional anisotropy (FA) and mean diffusivity (MD) values were measured by placing region of interests (ROIs) on globus palllidus (GP), substantia nigra (SN), putamen, and caudate (CN) in all groups. In patient group hypointense and hyperintense components of eye of tiger sign visible on T2* GRE were automatically segmented to obtain DTI metrics. A significant difference in FA value was observed among all groups in CN, GP and SN. Significantly high FA with low MD value in hypointense compared to hyperintense region of eye of tiger sign was observed in patient group. We conclude that FA is a sensitive indicator for abnormal iron accumulation in these regions.