Fahr's syndrome - Idiopathic Bilateral Striopallidodentate Calcinosis: a rare cause of seizures in a young African adolescent girl

Abstract

Fehr's disease, also known as Idiopathic Calcification of the Basal Ganglia (ICBG) or nonarteriosclerotic cerebral calcification, striopallidodentate calcinosis or cerebrovascular ferrocalcinosis (and many others), is a rare sporadic or familial neurological disorder whose etiology is unknown and is characterized by extensive symmetrical calcification of the basal ganglia and dentate nuclei in the cerebellum.¹ The average age at onset of symptoms is 40 to 60 years.² Hoque et al reported a case of Fahr's disease presenting with epilepsy, an uncommon presentation of this condition, in a 40 year old man.¹ Since its the basal ganglia that is commonly involved, most cases present with extrapyramidal symptoms. We report a case of an 11 year old African child presenting with seizures, bilateral basal ganglia calcifications and normal calcium metabolism.