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Mitochondrial protein-associated neurodegeneration (MPAN): MRI

T2/FLAIR hypointensity in the region of globus pallidus & substansia nigra. These findings may suspicious for neurodegeneration with brain iron accumulation particularly mitochondrial protein-associated neurodegeneration (MPAN). 

This recently described form of NBIA features hypointensity of the globus pallidus and substantia nigra, with relative preservation of the remainder of the cortex, cerebellum and brainstem. Clinical findings include generalized dystonia, with a prominent orobuccolingual component. 

Mitochondrial protein-associated neurodegeneration (MPAN): MRI Reviewed by Sumer Sethi on Thursday, June 22, 2017 Rating: 5

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