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Neuronal Ceroid-Lipofuscinoses (Neuronal Ceroid Lipofuscinosis) Summary

Description: A group of severe neurodegenerative diseases characterized by intracellular accumulation of autofluorescent wax-like lipid materials (CEROID; LIPOFUSCIN) in neurons. There are several subtypes based on mutations of the various genes, time of disease onset, and severity of the neurological defects such as progressive DEMENTIA; SEIZURES; and visual failure.

Also Known As: Neuronal Ceroid Lipofuscinosis; Batten Disease; Lipofuscinosis, Neuronal Ceroid; Jansky Bielschowsky Disease; Spielmeyer Vogt Disease Show All >>

Networked: 677 relevant articles (4 outcomes, 43 trials/studies) for this Disease

Key Drugs and Agents for Neuronal Ceroid-Lipofuscinoses

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Superoxides (Superoxide) : 2 outcomes in 4 results : IBA
  2. Iron : 1 outcome in 4 results : IBA
  3. Hydroxyl Radical : 1 outcome in 2 results : IBA
  4. Cysteamine (Mercaptamine) : 1 outcome in 2 results : FDA 1
  5. Acetylcysteine (Siran) : 1 outcome in 2 results : FDA 17 Generic
  6. Microtubule-Associated Proteins (Microtubule-Associated Protein 2) : 1 outcome in 1 result : IBA
  7. mitochondrial ATPase subunit c : 4 studies in 18 results : IBA
  8. lipopigments : 3 studies in 65 results : IBA
  9. Antibodies : 3 studies in 11 results : IBA
  10. Retinaldehyde (Retinal) : 2 studies in 31 results : IBA
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Diseases Related to Neuronal Ceroid-Lipofuscinoses

  1. Neuronal Ceroid-Lipofuscinoses (Neuronal Ceroid Lipofuscinosis)
  2. Neurodegenerative Diseases (Neurodegenerative Disease)
  3. Parkinson Disease (Parkinson's Disease)
  4. Mental Retardation (Idiocy)
  5. Dementia (Dementias)
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Key Therapies for Neuronal Ceroid-Lipofuscinoses

Efficacy Chart >>
  1. Enzyme Replacement Therapy : 1 study in 7 results
  2. Transplants (Transplant) : 1 study in 1 result
  3. Therapeutics : 5 results
  4. Bone Marrow Transplantation (Transplantation, Bone Marrow) : 4 results
  5. Anesthesia : 4 results
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