Late Onset Atypical Pantothenate-Kinase-Associated Neurodegeneration

Table 1

Clinical presentation of PKAN.

Features	Typical PKAN	Atypical PKAN
Onset	First decade	Second or third decade
Features	Gait impairment, focal dystonia, pyramidal dysfunction, pigmentary retinopathy, and cognitive impairment	Psychiatric symptoms, focal dystonia, ± parkinsonism or chorea, cognitive impairment, late gait dysfunction
Progression	Rapid progression; Periods of stability interspersed with periods of rapid progression.; Loss of ambulation occurs 10 to 15 years after onset	Slower progression; Loss of ambulation after 15 to 40 years after onset
Imaging	Eye-of-the-tiger	Eye-of-the-tiger