Hallervorden Spatz Disease-A Rare Case Report

This patient was referred to us for second opinion with clinical features of dystonia and non-specific initial MRI report. On MRI we noted bilaterally symmetric hyperintense signal changes in anterior medial globus pallidus with surrounding hypointensity in the globus pallidus on T2-weighted images. These imaging features are fairly diagnostic of HSD and have been termed the “eye-of-the-tiger” sign.The hyperintensity represents pathologic changes including gliosis, demyelination, neuronal loss, and axonal swelling, and the surrounding hypointensity is due to loss of signal secondary to iron deposition.

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