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Fahr syndrome with cortical laminar necrosis

Case contributed by Fatiha Kharroubi
Diagnosis almost certain

Presentation

Confusion and visual disturbances.

Patient Data

Age: 75 years
Gender: Female

Bilateral calcifications of the basal ganglia, subcortical white matter, and cerebellar dentate nuclei are noted with associated gyriform hyperdensities in the occipital lobes suggestive of cortical laminar necrosis.

Annotated images

ct
  • green arrow: bilateral basal ganglia calcification

  • orange arrow: occipital cortical hyperdensity

  • blue arrow: cerebellar dentate nuclei calcification

Case Discussion

Fahr disease is characterized by bilateral and symmetric calcifications in special sites like the basal ganglia, dentate nuclei, thalami, and the subcortical white matter as a result of abnormal vascular calcium deposition.

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