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Fahr syndrome

Case contributed by Danilo Ferreira Maia
Diagnosis almost certain

Presentation

16-years-old male patient presenting with headache and vomiting 12 years after excision of medulloblastoma.

Patient Data

Age: 16 years
Gender: Male

CT Brain

ct

Extensive symmetric calcifications involving caudate, lentiform nucleus, thalamus, and subcortical white matter.

Case Discussion

Fahr syndrome, also called estriatopalidodendate calcinosis, is characterized by extensive calcification of the base ganglia, dentate nucleus, thalamus, and subcortical white matter. It may be primary, familial, or secondary, such as HIV, hypoparathyroidism, hyperparathyroidism, sequelae of chemotherapy or radiotherapy, among others.

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