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Canavan Disease (Spongy Degeneration of Infancy) Summary

101 relevant articles (1 outcomes, 6 trials/studies) found for this Disease

Description: A rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demeylination that gives rise to a spongy appearance. Aspartoacylase deficiency leads to an accumulation of N-acetylaspartate in astrocytes. Inheritance may be autosomal recessive or the illness may occur sporadically. This illness occurs more frequently in individuals of Ashkenazic Jewish descent. The neonatal form features the onset of hypotonia and lethargy at birth, rapidly progressing to coma and death. The infantile form features developmental delay, DYSKINESIAS, hypotonia, spasticity, blindness, and megalencephaly. The juvenile form is characterized by ATAXIA; OPTIC ATROPHY; and DEMENTIA. (From Adams et al., Principles of Neurology, 6th ed, p944; Am J Med Genet 1988 Feb;29(2):463-71)

Also Known As: Spongy Degeneration of Infancy; Leukodystrophy, Spongiform; Canavan Disease, Familial Form; Canavan Disease, Infantile; Canavan Disease, Juvenile Show All >>

Key Drugs and Agents for Canavan Disease

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. aspartoacylase : 1 outcome 3 studies in 71 results : IBA
  2. N-acetylaspartate (N-acetyl aspartate) : 1 study in 33 results : IBA
  3. Biological Markers (Surrogate Marker) : 1 study in 4 results : IBA
  4. Protons (Proton) : 1 study in 2 results : IBA
  5. Prostaglandins D (PGD) : 1 study in 1 result : IBA
  6. Lithium : 1 study in 1 result : IBA
  7. Aspartic Acid (Aspartate) : 7 results : FDA 11
  8. N-acetyl-1-aspartylglutamic acid (NAAGA) : 5 results : IBA
  9. DNA (Deoxyribonucleic Acid) : 4 results : IBA
  10. Succinate-Semialdehyde Dehydrogenase (Succinic Semialdehyde Dehydrogenase) : 2 results : IBA
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Diseases Related to Canavan Disease

  1. Nervous System Diseases (Neurological Disorders)
  2. Tremor (Tremors)
  3. Neurodegenerative Diseases (Neurodegenerative Disease)
  4. Canavan Disease (Spongy Degeneration of Infancy)
  5. Mental Retardation (Idiocy)
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Key Therapies for Canavan Disease

Efficacy Chart >>

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