A Rare Case Presentation of Fahr's Syndrome

Abstract

Fahr's syndrome is a rare, genetically dominant, inherited, neurological disorder characterised by abnormal deposits of calcium in areas of the brain that control movement, including the basal ganglia and the cerebral cortex. Symptoms of the disorder may include deterioration of motor functions, dementia, seizures, headache, blurring of vision, spasticity (stiffness of limbs) and athetosis (involuntary, writhing movements). The most common site of calcification is the globus pallidus. However additional areas of calcification are putamen, caudate nucleus, internal capsule, dentate nucleus, thalamus, cerebellum and cerebral white matter. We are presenting a case of a 30 year old male, who complained of stiffening of upper limbs and blurring of vision followed by giddiness which was associated with slowness of movements. His CT scan revealed a symmetrical large area of calcification over the basal ganglia. Secondary causes of this disease were ruled out to make the clinical diagnosis of Bilateral Striopallidodentate Calcinosis, [BSPDC] which is otherwise called as the Fahr's syndrome.