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Our cases of Fahr's disease and review of the literature

Sukran Kaygisiz.



Abstract
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Fahr's disease is defined as idiopathic basal ganglia calcification. Calcinosis develops in the basal ganglia and cerebral cortex due to impaired calcium and phosphorus metabolism. Calcium deposition or mineralization usually develops in vascular and perivascular areas. It results in vascular obliteration, perivascular neuronal degeneration, and gliosis in the brain. Intracranial calcifications are symmetrical and occur primarily in the basal ganglia, dentate nucleus, thalamus, and centrum semiovale. If intracranial calcifications develop due to a known cause, it is called Fahr syndrome. It is an inherited or sporadic neurological disorder and is thought to be rare. It is more common in males than females and occurs mainly in the fourth decade of life. The disease may present with clinical signs such as slurred speech, gait instability, involuntary movements, or muscle cramps. It may be accompanied by neuropsychiatric symptoms such as psychosis and personality changes. Because of the symptoms, it may be confused with other neurological diseases and misdiagnosed. This retrospective study was planned to evaluate the frequency of patients in our region and their clinical findings.

Key words: Neurologic disorder, Fahr's disease, Fahr's syndrome, intracranial calcification







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