Fahr’s syndrome is a rare clinical entity that presents mainly with extrapyramidal signs and accompanied with metabolic, biochemical, neuroradiological and neuro-psychiatric situations at the same time. Bilateral intracranial calcifications are usually encountered as an incidental radiological finding. In this study, we discuss two cases of Fahr’s syndrome in the light of the literature; one of them idiopathic and the other one occurring secondary to hypoparathyroidism. Clinically, one of the cases presented with seizures and the other one with imbalance of the coordination system. Cranial computerized scans of both patients revealed intracranial diffuse bilateral calcifications in thalamus, basal ganglia and cerebellum.

Fahr’s syndrome, although encountered rarely, should also be taken into account in the differential diagnosis of cases with abnormal intracranial calcifications along with other familial, congenital and metabolic diseases and syndromes.©2007, Firat University, Medical Faculty