DR. SHADMA EQBAL

DR. RAKHI KUSUMESH

Abstract

Fahr’s syndrome refers to a rare syndrome which is characterized by symmetrical and bilateral intracranial calcification.It is associated with neuropsychiatric, extrapyramidal and cerebellar symptoms, convulsive seizures, parkinsonian features, dementia and speech disorders.Within the basal ganglia,the globus pallidus is the most frequent site of the calcification. A 17 year old female presented with bilateral diminution of vision. She had history of dystonia and distal limbs involvement with frequent falls in school.She had history of oligomenorrhea.On ocular examination, bilateral anterior and posterior subcapsular cataract was noted.Her CT scan revealed a large area of calcification over the basal ganglia, the thalamus and the cerebellum. The secondary causes of bilateral calcification were ruled out to make the clinical diagnosis of idiopathic bilateral striopallidodentate calcinosis, which is otherwise called as the Fahr’s syndrome