Fahr disease

Case contributed by Fakhry Mahmoud Ebouda
Diagnosis almost certain


Acute confusion. Rule out CVA.

Patient Data

Age: 65 years
Gender: Male

Bilateral symmetrical dense calcifications are seen bilaterally within the basal ganglia, corona radiata, centrum semiovale, cerebral peduncles, as well as in the deep white matter of the cerebellum, particularly involving the dentate nuclei.

There is also prominence of the of the lateral ventricles with no midline shift. Prominence is also noted of the cortical sulci, cerebellar folia and extra axial CSF spaces. These changes suggest generalized cerebral atrophy.

Overall, the plain CT findings are consistent with Fahr syndrome.

Case Discussion

Fahr disease is characterized by abnormal vascular calcium deposition, particularly in the basal ganglia, cerebellar dentate nuclei, and white matter, with subsequent atrophy.

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