What is this radiological sign?
What is the diagnosis?
What is the cause of this findings and what part of the brain is affected?
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@BrownJHM @mohammadala33 @DrAkhilRaghavan @IhabFathiSulima
The eye-of-the-tiger-sign is known as a radiological sign, that refers to abnormal low signal intensity in the globus pallidus,with a central longitudinal zone of high signal, as seen on T2-weighted MRI images.
This low signal surrounds a central, small hyperintense area, producing the eye-of-the-tiger appearance.The sign is seen in Hallervorden-Spatz (HS) syndrome aka neurodegeneration with brain iron accumulation (NBIA) or pantothenate kinase II (PANC2)-associated neurodegeneration.
The HS syndrome is a neurodegenerative disorder associated with extrapyramidal dysfunction and dementia. It is a neuroaxonal dystrophy, with the pathologic triad of iron deposition, axonal spheroids,gliosis in the globus pallidi.
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