Fahr’s disease: familial idiopathic basal ganglia calcification with and without extrapyramidal disorders

Abstract

Fahr’s disease (FD), also known as familial idiopathic basal ganglia calcification, is a neurodegenerative disease affecting cerebral micro vessels, mainly in the basal ganglia. It mostly presents with movement disorders, dementia and behavioral abnormalities. It is considered hereditary with an autosomal dominant transmission. Fahr’s disease is often underestimated and under diagnosed. We reported the clinical differences found in two patients with Fahr’s Disease. In particular, we described a case of Fahr’s disease with behavioral alteration with extrapyramidal movement disorders, and a rare case of Fahr’s disease with cognitive and behavioral alterations in absence of extrapyramidal movement disorders.