Fahr syndrome

Case contributed by Bouhouche Abdeldjalil
Diagnosis almost certain

Presentation

Patient with a known history of anxio-depressive disorders presenting with seizures.

Patient Data

Age: 40 years
Gender: Male
mri
This study is a stack
Axial
T1
This study is a stack
Axial
Gradient Echo
This study is a stack
Axial
FLAIR
This study is a stack
Axial
T2
This study is a stack
Axial
DWI
This study is a stack
Axial
T1 C+
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Info

The MRI sequences demonstrate:

  • bilateral and symmetrical abnormal signal involvement of the caudate, lentiform nuclei, thalami and dentate nuclei as well as the deep and subcortical white matter which appear of high signal on T1 and iso to low signal on T2 with signal drop-out on GRE, in keeping with massive calcification rather than blood products

  • bilateral symmetrical T2/FLAIR high signal areas of the basal ganglia, the white matter of the internal capsules, corona radiata, centrum semiovale, and surrounding the dentate nuclei

  • no restricted diffusion on DWI/ADC or abnormal enhancement on postcontrast sequences

ct
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Info

A complementary CT was performed confirming the calcific nature of the aforementioned lesions.

Case Discussion

Fahr syndrome, also known as bilateral striatopallidodentate calcinosis, is characterized by abnormal vascular calcium deposition, particularly in the basal ganglia, cerebellar dentate nuclei, and white matter.

It can be either primary (usually autosomal dominant) or secondary to a large number of underlying illnesses or metabolic disturbances. 

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