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Central African Journal of Medicine

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

H.A. Mujuru, T Maphosa, M Mangiza, L Hlatshwayo

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.




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