FAHR DISEASE: A CASE OF DIAGNOSTIC AND THERAPEUTIC CHALLENGE

Abstract

Fahr’s disease, also known as familial idiopathic basal ganglia calcification, is a rare and low prevailing neurological disorder in our environment, characterized by abnormal calcifications in several areas of the brain and seen primarily in the basal ganglia It exhibits a variable clinic of neuropsychiatric manifestations generally complemented by pseudohypoparathyroidism, a diagnosis that represents a challenge in the clinical practice and should be based in imaging and the compliance of diagnostic criteria.

A specific treatment is yet to be established, with symptomatic management being the best choice for those who suffer from it since they can benefit of good conditions despite a serious prognosis.
This article presents the case of a 79 year old woman who is admitted for a clinical profile of a three-day progress of drowsiness and delirium. Infection and hydroelectrolytic disorders are dismissed as an explanation to these symptoms. With CT scans and contrasted magnetic resonance imaging, Fahr disease is confirmed. Symptomatic treatment is started with a clinical progressive recovery of her neurological condition and a positive progress in the hospital discharge.