Image of the month: Diffuse intracranial calcinosis Fahr's Syndrome

A 44-year-old woman presented with expressive dysphasia on a background of epilepsy. Her computed tomography (CT) head scan demonstrated extensive calcification. Having excluded metabolic causes, the diagnosis was Fahr's syndrome. This is a rare, autosomal dominant disorder characterised by extensive calcification in the basal ganglia and cerebral cortex. Symptoms may include seizures, decline in motor function and movement disorders.
Non-contrast CT head scan, demonstrating florid multifocal -calcification including the pons and cerebellar hemispheres bilaterally. The differential diagnoses might include multiple calcified cavernous angiomata, infections such as neurocysticercosis or TB, previous brain injury, a variety of inherited or acquired metabolic conditions, or previous radiation or chemotherapy. CT = computed tomography; TB = tuberculosis.
Non-contrast CT head scan demonstrating florid multifocal calcification of the basal ganglia and at the grey-white matter interface of both cerebral hemispheres. CT = computed tomography.
- © 2014 Royal College of Physicians
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