Pantothenate Kinase-Associated Neurodegeneration | Legal notice |
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Other terms
Neurodegeneration, Pantothenate Kinase-Associated,Pantothenate Kinase Associated Neurodegeneration,Pigmentary Pallidal Degeneration,Degeneration, Pigmentary Pallidal,Pigmentary Pallidal Atrophy,Pallidal Atrophy, Pigmentary,Hallervorden-Spatz Disease,Hallervorden Spatz Disease,Hallervorden-Spatz Syndrome,Hallervorden Spatz Syndrome
Description
Pantothenate Kinase-Associated Neurodegeneration: 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)
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