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Neuroaxonal Dystrophies (Neuroaxonal Dystrophy, Late Infantile) Summary

Description: A nonspecific term referring both to the pathologic finding of swelling of distal portions of axons in the brain and to disorders which feature this finding. Neuroaxonal dystrophy is seen in various genetic diseases, vitamin deficiencies, and aging. Infantile neuroaxonal dystrophy is an autosomal recessive disease characterized by arrested psychomotor development at 6 months to 2 years of age, ataxia, brain stem dysfunction, and quadriparesis. Juvenile and adult forms also occur. Pathologic findings include brain atrophy and widespread accumulation of axonal spheroids throughout the neuroaxis, peripheral nerves, and dental pulp. (From Davis & Robertson, Textbook of Neuropathology, 2nd ed, p927)

Also Known As: Neuroaxonal Dystrophy, Late Infantile; Neuroaxonal Dystrophy; Adult Neuroaxonal Dystrophy; Infantile Neuroaxonal Dystrophy; Juvenile Neuroaxonal Dystrophy Show All >>

Networked: 123 relevant articles (2 outcomes, 4 trials/studies) for this Disease

Key Drugs and Agents for Neuroaxonal Dystrophies

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Lactic Acid : 1 outcome in 2 results : FDA 4 Generic
  2. Lysosomal Alpha-N-Acetylgalactosaminidase Deficiency : 1 outcome in 2 results : IBA
  3. alpha-N-Acetylgalactosaminidase (N-Acetyl-alpha-D-Galactosaminidase) : 2 studies in 9 results : IBA
  4. NAD (NADH) : 1 study in 37 results : IBA
  5. Sialoglycoproteins : 1 study in 1 result : IBA
  6. Crystallins (Crystallin) : 1 study in 1 result : IBA
  7. Iron : 15 results : IBA
  8. Phospholipases A2 (Phospholipase A2) : 7 results : IBA
  9. Streptozocin (Streptozotocin) : 7 results : FDA 1
  10. Neurodegeneration with brain iron accumulation (NBIA) : 6 results : IBA
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Diseases Related to Neuroaxonal Dystrophies

  1. Angiokeratoma
  2. Neuroaxonal Dystrophies (Neuroaxonal Dystrophy, Late Infantile)
  3. Neurodegenerative Diseases (Neurodegenerative Disease)
  4. Pantothenate Kinase-Associated Neurodegeneration (Hallervorden-Spatz Disease)
  5. Diabetic Neuropathies (Diabetic Neuropathy)
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Key Therapies for Neuroaxonal Dystrophies

Efficacy Chart >>
  1. Transplantation (Transplant Recipients) : 2 results
  2. Denervation : 1 result

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