Fahr's Disease: A Differential to Be Considered for Various Neuropsychiatric Presentations

Cureus. 2018 Mar 11;10(3):e2304. doi: 10.7759/cureus.2304.

Abstract

Fahr's disease, also known as familial idiopathic basal ganglia calcification, is a neurodegenerative disorder affecting cerebral microvessels, mainly the basal ganglia, and presenting with diverse neuropsychiatric manifestations. It is considered to be mainly hereditary, with autosomal dominant inheritance. In light of its various presentations and incomplete penetrance, Fahr's disease is known to be underestimated and underdiagnosed. Here, an early-onset case of Fahr's disease is presented mainly with pure psychiatric symptoms. Given the diversity of the presenting symptoms, and variations in the age of onset, further investigation of organic etiologies in patients presenting with neuropsychiatric symptoms, family members of patients with Fahr's disease, and patients with unexplained cerebral calcification is recommended.

Keywords: basal ganglia calcification; fahr disease; familial basal ganglia calcification.

Publication types

  • Case Reports