Presentation
Dystonia, muscular rigidity and spasticity.
Patient Data
Age: 8 years
Gender: Male
From the case:
Neurodegeneration with brain iron accumulation
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T2 hypointensity is seen involving bilateral globi pallidi and substantia nigra, which is better highlighted by GRE images.
Cerebellar atrophy is seen with prominence of cerebellar folia.
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.