Pantothenate Kinase-Associated Neurodegeneration

**Definition:** 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)

**Tree numbers:** - C10.228.140.079.800 - C10.228.140.744.320 - C10.228.662.575 - C10.574.500.700 - C16.320.400.650

**Synonyms:** - Hallervorden-Spatz Disease - Hallervorden-Spatz Syndrome - Neurodegeneration With Brain Iron Accumulation 1 - Pigmentary Pallidal Atrophy - Pigmentary Pallidal Degeneration - PKAN Neuroaxonal Dystrophy, Juvenile-Onset - Neuroaxonal Dystrophy, Juvenile-Onset - Neurodegeneration with Brain Iron Accumulation Type 1