Pantothenate Kinase-Associated Neurodegeneration

Synonyms

Degeneration, Pigmentary Pallidal

Dystrophies, Juvenile-Onset Neuroaxonal

Dystrophy, Juvenile-Onset Neuroaxonal

Hallervorden Spatz Disease

Hallervorden Spatz Syndrome

Hallervorden-Spatz Disease

Hallervorden-Spatz Syndrome

Juvenile-Onset Neuroaxonal Dystrophies

Juvenile-Onset Neuroaxonal Dystrophy

Neuroaxonal Dystrophies, Juvenile-Onset

Neuroaxonal Dystrophy, Juvenile Onset

Neuroaxonal Dystrophy, Juvenile-Onset

Neurodegeneration With Brain Iron Accumulation 1

Neurodegeneration with Brain Iron Accumulation Type 1

Neurodegeneration, Pantothenate Kinase-Associated

PKAN Neuroaxonal Dystrophy, Juvenile Onset

PKAN Neuroaxonal Dystrophy, Juvenile-Onset

Pallidal Atrophy, Pigmentary

Pantothenate Kinase Associated Neurodegeneration

Pigmentary Pallidal Atrophy

Pigmentary Pallidal Degeneration

A rare autosomal recessive degenerative disorder which usually presents in late childhood or adolescence. Clinical manifestations include progressive MUSCLE SPASTICITY; hyperreflexia; MUSCLE RIGIDITY; DYSTONIA; DYSARTHRIA; and intellectual deterioration which progresses to severe dementia over several years. (From Adams et al., Principles of Neurology, 6th ed, p972; Davis & Robertson, Textbook of Neuropathology, 2nd ed, pp972-929)