Neurodegeneration with brain iron accumulation

Case contributed by Utkarsh Kabra
Diagnosis probable

Presentation

Progressive dystonia

Patient Data

Age: 40 years

There is disproportionate mineralization of bilateral globus palllidi and dentate nuclei evidenced by T2 and FLAIR hypointense signal and blooming on GRE.

Case Discussion

Although some mineralization of globus pallidi and dentate nuclei occurs normally with age, the above patient has disproportionate mineralization. These finding with clinical symptoms of progressive dystonia suggest likelihood of neurodegeneration with brain iron accumulation.

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