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Fahr syndrome, also known as bilateral striatopallidodentate calcinosis is a rare neurological disorder, characterized by abnormal vascular calcium deposits in the basal ganglia, cerebellar dentate nuclei and white matter. Diagnostic criteria of Fahr’s disease are as follows: bilateral calcification of the basal ganglia on neuroimaging, progressive neurologic dysfunction, absence of biochemical abnormalities, and family history consistent with autosomal dominant inheritance r The term Fahr’s disease is used when primary familial brain calcification is present, and the term Fahr’s syndrome is used for secondary causes, Secondary causes include calcium metabolism disorders, endocrinopathies hypoparathyroidism, pseudohypoparathyroidism, autoimmune diseases vasculitis, infections. Symptoms may contain deterioration of motor function, movement disorders like parkinsonism, Korea and athetosis, dementia, ataxia, seizures, headache, dysarthria, spasticity and psychiatric disorders. Movement disorders, especially parkinsonism is the most common presentation followed by cognitive impairment and ataxia.

Fahr Syndrome with Fluctuation in Consciousness and Temporary Vision Loss