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)
Also Known As:
Hallervorden-Spatz Disease; Hallervorden-Spatz Syndrome; Neuroaxonal Dystrophy, Juvenile-Onset; Neurodegeneration With Brain Iron Accumulation 1; Neurodegeneration with Brain Iron Accumulation Type 1; PKAN Neuroaxonal Dystrophy, Juvenile-Onset; Pigmentary Pallidal Atrophy; Degeneration, Pigmentary Pallidal; Dystrophies, Juvenile-Onset Neuroaxonal; Dystrophy, Juvenile-Onset Neuroaxonal; Hallervorden Spatz Disease; Hallervorden Spatz Syndrome; Juvenile-Onset Neuroaxonal Dystrophies; Juvenile-Onset Neuroaxonal Dystrophy; Neuroaxonal Dystrophies, Juvenile-Onset; Neuroaxonal Dystrophy, Juvenile Onset; Neurodegeneration, Pantothenate Kinase-Associated; PKAN Neuroaxonal Dystrophy, Juvenile Onset; Pallidal Atrophy, Pigmentary; Pantothenate Kinase Associated Neurodegeneration; Pigmentary Pallidal Degeneration