Case Report: An Incidental Finding of Fahr's Disease in a Patient with Hypochondria

Abstract

Idiopathic basal ganglia calcification (IBGC), commonly referred to as Fahr's disease, is a rare neurological disorder characterized by the abnormal, symmetrical, and bilateral calcification of the basal ganglia and other brain regions. Patients typically present in their forties and fifties with various neurologic and/or psychiatric symptoms, including movement disorders, Parkinsonism, psychosis, and depression. The pathophysiology of this disease is not completely understood; however, several gene mutations have been identified in the pathogenesis of Fahr's disease. These mutations display an autosomal dominant inheritance pattern. Furthermore, the regional phenotypic expression of calcifications differs greatly from patient to patient, as do their clinical presentations. Here, we describe a patient who presented with psychiatric manifestations and imaging consistent with Fahr's disease.

Keywords: basal ganglia calcification; bilateral striopallidodentate calcinosis; fahr's disease; hypochondria; movement disorder; pdgfrb; slc20a2.

Figure 1. CT images displaying calcifications of the basal ganglia and cerebellum

A. Axial CT image showing bilateral, linear calcifications of the cerebellum. B. Axial CT image showing bilateral calcifications of the putamen and the head and tail of the caudate nucleus. C. Coronal CT image showing bilateral calcifications of the caudate nucleus and putamen. D. Coronal CT image showing bilateral calcifications of the putamen. E. Sagittal CT image showing calcification of the head of the caudate nucleus. F. Sagittal CT image showing calcifications throughout the basal ganglia and cerebellum. CT: computed tomography