Neurodegeneration with brain iron accumulation

Case contributed by Dr Utkarsh Kabra

Presentation

Dystonia, muscular rigidity and spasticity.

Patient Data

Age: 25 years
Gender: Female

T2 hypointensity is seen involving bilateral globi pallidi and substantia nigra, which is better highlighted by GRE images.

Case Discussion

Above findings are suggestive of bilateral globi pallidi and substantia nigra mineralization, likely iron deposition secondary to neurodegeneration with brain iron accumulation.

This explains the patients symptoms of dystonia, muscular rigidity, and spasticity.

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