Hallervorden-Spatz Syndrome (HSS) is a rare, familial, progressive, and ultimately fatal disorder characterized by extrapyramidal rigidity, dysarthria, pyramidal tract involvement, and dementia, with pathological evidence of iron deposition in the globus pallidus and substantia nigra. The diagnosis is usually made using magnetic resonance imaging (MRI), where bilateral high signal intensity in the globus pallidus surrounded by low signal intensity areas is found. This is called an eye-of-the-tiger appearance and is considered specific to this disorder. Psychiatric aspects of this syndrome are common and include cognitive decline, personality changes with impulsivity and violent outbursts, depression and emotional lability. Here we report two cases of Hallervorden-Spatz Syndrome with psychiatric presentation.
Key words: Hallervorden Spatz, neuropsychiatry, psychosis
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