Document Type

Case Report


Hallervorden Spatz disease, also known as pantothenate kinase associated neuro-degeneration, is a rare, progressive neurological disorder usually seen in first decade of life. It is associated with extrapyramidal effects, dysarthria and dementia. Hallervorden Spatz Disease is also associated with psychiatric symptoms, depression and behavioral changes. Affected patients are disabled predominantly by dystonia. MRI, in later stage of the disease, shows “eye of the tiger’ appearance which is fairly diagnostic of Hallervorden Spatz Disease. Response to drugs is often poor and of limited value to these patients. This report highlights a classical case of Hallervorden Spatz disease that presented as an outpatient with dystonia and psychotic symptoms and was diagnosed on the basis of clinical and radiological evidence.

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