Hallervorden Spatz syndrome - eye of the tiger sign
3 year progressive left hemidystonia, decreased cognition and speech, orobuccal dyskinesia.
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T2 shows central area of globus pallidus hyperintensity, with surrounding low intensity, giving the 'eye of the tiger' sign.
Eye of the tiger sign is seen in Hallervorden Spatz syndrome, also known as pantothenate kinase-associated neurodegeneration (PKAN). This sign describes characteristic low signal surrounding a central region of high signal in the anteromedial globus pallidus on T2. Pathologically, the low signal corresponds to abnormal iron deposition, and the high signal corresponds to gliosis, vacuolisation and increased water content.