Search Login Register

Canavan Disease (Leukodystrophy, Spongiform) Summary

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: Leukodystrophy, Spongiform; Spongy Degeneration of Infancy; Aminoacylase 2 Deficiency; Aspartoacylase Deficiency; Canavan Disease, Familial Form Show All >>

Networked: 144 relevant articles (3 outcomes, 8 trials/studies) for this Disease

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 80 results : IBA
  2. Lithium : 1 outcome 1 study in 2 results : IBA
  3. Thioctic Acid (Lipoic Acid) : 1 outcome 1 study in 1 result : IBA
  4. N-acetylaspartate (N-acetyl aspartate) : 1 study in 53 results : IBA
  5. DNA (Deoxyribonucleic Acid) : 1 study in 6 results : IBA
  6. Biological Markers (Surrogate Marker) : 1 study in 4 results : IBA
  7. Protons (Proton) : 1 study in 2 results : IBA
  8. Prostaglandins D (PGD) : 1 study in 1 result : IBA
  9. Aspartic Acid (Aspartate) : 11 results : FDA 7
  10. N-acetyl-1-aspartylglutamic acid (NAAGA) : 6 results : IBA
Show All >>

Diseases Related to Canavan Disease

  1. Nervous System Diseases (Neurological Disorders)
  2. Tremor (Tremors)
  3. Canavan Disease (Leukodystrophy, Spongiform)
  4. Neurodegenerative Diseases (Neurodegenerative Disease)
  5. Mental Retardation (Idiocy)
Show All >>

Key Therapies for Canavan Disease

Efficacy Chart >>
  1. Enzyme Replacement Therapy : 1 result
  2. Cell Transplantation : 1 result

CureHunter Inc. provides medical information and specifically does NOT provide medical advice.
© Copyright 2003-2016 CureHunter Inc., MeSH copyright NLM, Journal Articles copyright original owners.