Atypical neurological decline in a teenager: A case of pantothenate kinase-associated neurodegeneration


Article PDF :

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Article type :

Case Report

Author :

Hardik Shah, Naiya Bhavsar, Rajeshri Mehta, Ashok Bhandari, Nainesh Gorvadia

Volume :

8

Issue :

2

Abstract :

Pantothenate Kinase-Associated Neurodegeneration (PKAN), formerly Hallervorden-Spatz disease, is a rare autosomal recessive neurodegenerative disorder characterized by progressive movement abnormalities and iron accumulation in the basal ganglia. A 13-year-old male presented with a history of progressive gait disturbances, involuntary limb movements, and developmental regression. Imaging showed the pathognomonic "eye-of-the-tiger" sign in the globus pallidus. Supportive treatment was initiated. This case demonstrates typical clinical and radiological findings of adolescent-onset PKAN. Early diagnosis can facilitate timely symptomatic intervention.

Keyword :

Extrapyramidal sign, Hallervorden-Spatz disease, Dystonia, Dementia.