= PANTOTHENATE KINASE-ASSOCIATED NEURODEGENERATION

= rare familial neurodegenerative metabolic disorder with abnormal iron retention in basal ganglia

Cause: mutation of PANK2 gene encoding pantothenate kinase → neurodegeneration with iron accumulation

Age: 2^nd decade of life

Histo: hyperpigmentation and symmetrical destruction of globus pallidus + substantia nigra

Types:

1. classic early-onset rapidly progressive disease
2. atypical late-onset slowly progressive disease

• progressive gait impairment + rigidity of limbs
• slowing of voluntary movements, dysarthria
• choreoathetotic movement disorder, progressive dementia

CT:

• low- (= tissue destruction) / high-density (= dystrophic calcification) foci in globus pallidus

MR:

• “eye-of-the-tiger” sign = high-signal-intensity center surrounded by the more typical hypointensity in globus pallidus:
  • initially bilateral hypointense globus pallidus on T2WI (= iron accumulation)
  • later central hyperintense foci on T2WI (= tissue destruction + gliosis)