Fahr’s syndrome: a rare case report

Abstract

Fahr’s syndrome is a rare neurodegenerative disorder characterized by bilateral basal ganglia calcification. The exact incidence of Fahr’s syndrome is still unknown but estimated to be <1/1,000,000. On routine non-contrast-CT scan examination, 0.3-1.2% cases of intracranial calcification can be incidentally detected. It is caused by several medical conditions, with hypothyroidism as the most common one. Patient may come with neurologic or psychiatric symptoms. CT scan is the main supportive examination; it is used to determine the location and extension of calcification. Therapy is directed to the underlying etiology and usually symptomatic. A female, 46 years old, came with stiffness on both hands. This symptom appeared multiple times during past decade but with mild intensity. CT scan revealed calcification in both cerebral hemisphere and cerebellum. Blood test showed low calcium concentration. Patient was diagnosed with Fahr’s syndrome and given anticonvulsant and calcium supplementation. She was getting better during hospitalization and then discharged 4 days later with oral home medication.

Fahr syndrome, rare cause of neuropsychiatric manifestations in children: A two cases report

“…Fahr syndrome is a rare entity, the exact incidence of which is unknown, but it is estimated to be <1/1000000 [9]. It associates bilateral and symmetrical intracerebral calcifications of the central gray nuclei (NGC) mainly, of the serrated nuclei of the cerebellum and of the cerebral white matter with phosphocalcic metabolic disturbances [8,10].…”

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