Eye of the Tiger Sign in Pantothenate Kinase-Associated Neur
Neurodegeneration with brain iron accumulation (NBIA) is a group of diseases characterized by an abnormal accumulation of iron in the basal ganglia leading to various progressive disorders of movement and development. There are ten main types, including pantothenate kinase-associated neurodegeneration (PKAN), which is the most common form. The classic appearance helping in the diagnosis is the eye of the tiger on T2-weighted images (hypointensity of the globus pallidus with a central hyperintensity). An 11-year-old boy from a consanguineous marriage with no significant past medical history presents at the age of 9 psychomotor delays, upper limb tremor, and frequent falls. There was no family history of neurological diseases. Clinical examination revealed dysarthria, spastic paraparesis with rigidity, and dystonic movements of the upper member. Laboratory investigations and EEG were normal. An initial head CT showed bilateral calcifications of the globus pallidus.

A brain MRI was performed, revealing diffuse bilateral and symmetric hypointensity of both the globus pallidus with relatively central hyperintensity in T2-weighted images. These areas showed susceptibility artifacts (low signal) in T2 sequences. No other signal alterations were observed in other regions of the basal ganglia. These findings were consistent with the eye of the tiger sign that is characteristic of pantothenate kinase-associated neurodegeneration but not pathognomonic.

Source:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8121601/
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