EYE OF THE TIGER sign is the MRI changes seen in Globus Pallidus in Pantothenate kinase associated neurodegenration. .The Globus Pallidus in T2W MRI shows medial high signal and lateral low signal.
Pantothenate kinase associated neurodegenration
- Most common form of neurodegeneration with iron accumulation in the brain.
- Mutation of pantothenate kinase 2 gene is the etiology
- Best imaging manifestation is the “eye of the tiger sign” owing to the symmetric bilateral T2 hypo intensity adjacent to medial foci of increased T2 signal.
- T2 hypo intensity is secondary to iron deposition within the brain
- Clinical manifestations are rapid onset dystonia, dysarthria, rigidity and choreoathetosis.
- Classically presents with a young child with postural deficits and gait problems.