مرکز منطقه ای اطلاع رساني علوم و فناوري

Abstract :

Fahr’s disease is a rare idiopathic bilateral and symmetrical calcification of the basal ganglia, thalami, subcortical hemispheric white matter, and deep cerebellar nuclei that usually presents between the 4nd and 6th decade of life with a variable combination of involuntary movements, Parkinsonism, presenile subcortical dementia, seizures, and ataxia. This longitudinal observational case series was conducted at the neurology outpatients’ department of Sulaimaniya general teaching hospital, Iraq. Three patients were diagnosed with Fahr’s disease. Their chief presenting complaint and other coexistent clinical features were noted and followed-up for at least one year. The ages of those consecutive patients were 25, 34, and 21 years, respectively. Two were females and the other patient was a male. The chief presenting complaint among the 3 patients was heterogeneous; cognitive impairment, seizures, and chorea, respectively. At the time of diagnosis, Parkinsonism and cognitive decline were present in all patients. The 3 patients never developed dystonia, dyskinesia, or athetosis and one patient only had mild cerebellar ataxia. Seizures were the presenting feature in one patient and they never developed in the other 2 patients. All patients had a variable degree of intracerebral calcification. Fahr’s disease has heterogeneous phenotypes and the brain radiological findings do not predict the clinical presentation and course. Although Parkinsonism was not the presenting feature, it was found in all patients at the time of diagnosis. Involuntary movements and cerebellar dysfunction were uncommon and the cognitive impairment was of the frontal lobe subcortical dysfunction.