Abstract
Introduction: Idiopathic, bilateral, and symmetric striatopallidodentate calcinosis (or Fahr’s disease) is a rare disease that usually presents between the fourth and sixth decades of life with a variable combination of abnormal movements, Parkinsonism, seizures, and cognitive dysfunction. Asymptomatic cases are being increasingly recognized because of the widespread use of brain CT scanning.
Case presentation: We report a 25-¬year-¬old female who had diffuse intra-cerebral calcification and normal calcium metabolism; her presentation was progressive frontal lobe-like cognitive decline over a period of 6 years; impairment in memory, mental slowness, irritability, apathy, poor night time sleep, and social isolation. She has generalized rigidity but no tremor. Over a period of 1 and a half year of follow-up, she had not developed seizures.
Conclusion: Fahr’s disease may present as an isolated frontal lobe-like cognitive dysfunction and that the brain radiological findings do not predict the clinical presentation and course.

Key words: Fahr disease; frontal lobe; cognitive dysfucntion; intracranial calcification