Fahr’s Disease in a 26 year-old female: A case report

Abstract

Background: Fahr’s disease is a rare, degenerative neurological-psychiatric disorder characterized by idiopathic symmetrical and bilateral intracranial calcification. Prevalence of <1:1,000,000, usually presents at middle age group, rarely in the young. Calcification are deposited in basal ganglia, thalamus, putamen, hippocampus, cerebral cortex, cerebellar subcortical white matter and dentate nucleus. Diagnosis requires certain clinical criteria: absence of biochemical abnormalities and somatic features; absence of infection, trauma, toxic cause; presence of progressive neuropsychiatric dysfunction with onset of symptoms at 4th – 5th decade of life, presence of bilateral basal ganglia calcification visualized on neuroimaging and family history with autosomal dominant inheritance or sporadic.