Pantothenate Kinase-Associated Neurodegeneration

Overview

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Is a:

Disease or Syndrome

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)

Synonyms:

Degeneration, Pigmentary Pallidal
Hallervorden Spatz Disease
Hallervorden Spatz Syndrome
Hallervorden-Spatz Disease
Hallervorden-Spatz Syndrome
Neurodegeneration, Pantothenate Kinase-Associated
Pallidal Atrophy, Pigmentary
Pantothenate Kinase Associated Neurodegeneration
Pigmentary Pallidal Atrophy
Pigmentary Pallidal Degeneration

Broader Topics:

Narrower Topics:

None

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