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Pantothenate Kinase-Associated Neurodegeneration (Hallervorden-Spatz Disease) Summary

Description: 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; Pigmentary Pallidal Atrophy; Degeneration, Pigmentary Pallidal; Hallervorden Spatz Disease Show All >>

Networked: 193 relevant articles (3 outcomes, 6 trials/studies) for this Disease

Key Drugs and Agents for Pantothenate Kinase-Associated Neurodegeneration

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Iron : 1 outcome 3 studies in 77 results : IBA
  2. deferiprone (CP20) : 1 outcome 1 study in 4 results : IBA
  3. Botulinum Toxins (Botulinum Toxin) : 1 outcome in 2 results : IBA
  4. pantethine : 1 outcome in 2 results : IBA
  5. pantothenate kinase : 1 study in 40 results : IBA
  6. Baclofen : 1 study in 3 results : FDA 31 Generic
  7. Ferritins (Ferritin) : 1 study in 1 result : IBA
  8. Neurodegeneration with brain iron accumulation (NBIA) : 18 results : IBA
  9. Coenzyme A (CoA) : 12 results : IBA
  10. alpha-Synuclein : 10 results : IBA
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Diseases Related to Pantothenate Kinase-Associated Neurodegeneration

  1. Dystonia (Limb Dystonia)
  2. Parkinson Disease (Parkinson's Disease)
  3. Multiple System Atrophy
  4. Huntington Disease (Huntington's Disease)
  5. Neurosyphilis (General Paresis of the Insane)
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Key Therapies for Pantothenate Kinase-Associated Neurodegeneration

Efficacy Chart >>
  1. Ketogenic Diet : 1 outcome in 1 result
  2. Deep Brain Stimulation : 1 study in 11 results
  3. Spinal Injections : 1 study in 1 result
  4. Pallidotomy : 2 results
  5. Anesthesia : 2 results
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