Abstract

Pantothenate Kinase-Associated Neurodegeneration (PKAN), a subtype of neurodegeneration with brain iron accumulation (NBIA), presents with distinctive radiological features, notably the "eye of the tiger" sign. We report the case of a 7-year-old boy presenting with progressive developmental regression, dystonia, and dysarthria. Neuroimaging via MRI revealed bilateral and symmetric hyperintensity within the globus pallidus, surrounded by a hypointense area, indicative of the characteristic "eye of the tiger" sign, although not pathognomonic. PKAN, caused by mutations in the PANK2 gene, results in impaired coenzyme A synthesis, leading to neuronal destruction and iron accumulation in the basal nuclei. While the "eye of the tiger" sign is highly suggestive of PKAN, it can also be observed in other conditions, necessitating correlation with clinical findings and genetic testing for confirmation. This case underscores the importance of recognizing radiological patterns in guiding diagnosis and management of neurodegenerative disorders like PKAN.