NEURODEGENERATION WITH IRON ACCUMULATION TYPE1

Neurodegeneration with iron accumulation type 1 is a rare degenerative disorder presenting with dementia and progressive extrapyramidal dysfunction. A 10 yrs old girl reported with complaints of difficulty in speech and involuntary movements. MRI Brain showed ‘eye of tiger appearance’ which is suggestive of neurodegeneration with iron accumulation type 1. Treatment is symptomatic and chelating agents have no effect. The disease is progressivelyfatal