Hallervorden Spatz syndrome - eye of the tiger sign

Case contributed by Dr Yune Kwong


3 year progressive left hemidystonia, decreased cognition and speech, orobuccal dyskinesia.

Patient Data

Age: 60 years
Gender: Female

T2 shows central area of globus pallidus hyperintensity, with surrounding low intensity, giving the 'eye of the tiger' sign.

Case Discussion

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.

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Case information

rID: 41008
Published: 11th Nov 2015
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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