Hallervorden spatz disease: MR and pathological findings of a rare case.

M C Sharma, N Aggarwal, M Bihari, V Goyal, S Gaikwed, S Vaishya, Chitra Sarkar

Journal: Neurology India 2005;53(1):102-4

PMID: 15805666

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Abstract

We describe a child with pathologically proven Hallervorden Spatz disease. He presented with extrapyramidal symptoms and characteristic "eye-of-the-tiger" sign on magnetic resonance imaging. He was given the possible benefit if any of deep brain stimulation with no much improvement. Pathological examination of the brain showed iron deposition in bilateral globus pallidi, spongiform change and neuron axonal degeneration (spheroids).

Address: Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110-029, India.

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MeSH Terms: Child, Fatal Outcome, Globus Pallidus, Humans, Magnetic Resonance Imaging, Male, Pantothenate Kinase-Associated Neurodegeneration