Atypical pantothenate-kinase associated neurodegeneration [PKAN] in two Iranian patients

ABSTRACT

Pantothenate kinase- associated neurodegeneration [PKAN] or Hallervorden-Spatz syndrome is a rare autosomal recessive disorder characterized by dystonia, Parkinsonism, and iron accumulation in the brain. There are two types of this disease the classic disease which is characterized by an early onset and rapid progression, and the atypical disease which is characterized by later onset and slow progression. Clinical diagnosis is based on clinical and characteristic magnetic resonance imaging findings. We report two Iranian cases of atypical PKAN, the diagnosis of which was missed till MRI showed classic imaging findings: