(2) Pronounced peripheral motor and sensory neuropathy begins in infancy, and the disease progresses centrally with cognitive impairment, seizures and pyramidal tract signs
. (1) Patients become wheelchair bound in the second decade and eventually bedridden with severe polyneuropathy, ataxia and dementia.
A neurological evaluation revealed bilateral pyramidal tract signs
, cerebellar ataxia, and severe visual impairment (0.01/0.3), with a central scotoma in the right eye (Figure 1(b)).
(3) In classic PKAN the symptoms usually start to appear before age 6 on average around age 3 with profound dystonia, dysarthria, spasticity and pyramidal tract signs
and pigmentary retinopathy leading to night blindness and visual field constriction.
 Other neurological manifestations include pyramidal tract signs
, sensory deficits, involuntary movements and cerebellar ataxia.