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Pantothenate Kinase-Associated Neurodegeneration (Hallervorden-Spatz Disease)

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; Hallervorden Spatz Syndrome; Neurodegeneration, Pantothenate Kinase-Associated; Pallidal Atrophy, Pigmentary; Pantothenate Kinase Associated Neurodegeneration; Pigmentary Pallidal Degeneration
Networked: 193 relevant articles (3 outcomes, 6 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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)

Experts

1. Hayflick, Susan J: 13 articles (12/2015 - 01/2003)
2. Hayflick, S J: 8 articles (02/2009 - 08/2001)
3. Garavaglia, Barbara: 6 articles (09/2015 - 12/2010)
4. Schneider, Susanne A: 6 articles (08/2012 - 09/2007)
5. Gregory, Allison: 5 articles (12/2015 - 01/2005)
6. Bhatia, Kailash P: 5 articles (06/2012 - 09/2007)
7. Tiranti, Valeria: 4 articles (09/2015 - 03/2012)
8. Levi, Sonia: 4 articles (09/2015 - 12/2010)
9. Dusi, Sabrina: 4 articles (09/2015 - 03/2012)
10. Nardocci, Nardo: 4 articles (01/2013 - 12/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pantothenate Kinase-Associated Neurodegeneration:
1. IronIBA
2. deferiprone (CP20)IBA
3. Botulinum Toxins (Botulinum Toxin)IBA
4. pantethineIBA
5. pantothenate kinaseIBA
6. BaclofenFDA LinkGeneric
7. Ferritins (Ferritin)IBA
8. Neurodegeneration with brain iron accumulation (NBIA)IBA
9. Coenzyme A (CoA)IBA
10. alpha-SynucleinIBA

Therapies and Procedures

1. Ketogenic Diet
2. Deep Brain Stimulation
3. Spinal Injections
4. Pallidotomy
5. Anesthesia