Late adult onset chorea with typical pathology of Hallervorden-Spatz syndrome

Abstract

Senile chorea is a well recognised but poorly understood clinical entity characterised by a slowly progressive, generalised chorea in elderly people without mental deterioration or a clear underlying cause. The Hallervorden-Spatz syndrome is typically thought of as a paediatric condition with extrapyramidal features and dementia. However, it has been described in adults usually presenting with parkinsonism plus dementia. An elderly woman with slowly progressive chorea without dementia was found at postmortem to have the pathological features originally described by Hallervorden and Spatz. This association has not previously been reported.