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Leigh Disease (Leigh's Disease) Summary

Description: A group of metabolic disorders primarily of infancy characterized by the subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, dysphagia, and lactic acidosis. Pathological features include spongy degeneration of the neuropile of the basal ganglia, thalamus, brain stem, and spinal cord. Patterns of inheritance include X-linked recessive, autosomal recessive, and mitochondrial. Leigh disease has been associated with mutations in genes for the PYRUVATE DEHYDROGENASE COMPLEX; CYTOCHROME-C OXIDASE; ATP synthase subunit 6; and subunits of mitochondrial complex I. (From Menkes, Textbook of Child Neurology, 5th ed, p850).

Also Known As: Leigh's Disease; Leigh Syndrome; Subacute Necrotizing Encephalopathy; Subacute Necrotizing Encephalomyelopathy; Encephalomyelopathy, Subacute Necrotizing Show All >>

Networked: 402 relevant articles (5 outcomes, 25 trials/studies) for this Disease

Key Drugs and Agents for Leigh Disease

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Vitamins : 2 outcomes 1 study in 3 results : IBA
  2. Dichloroacetate (Dichloroacetic Acid) : 2 outcomes in 8 results : IBA
  3. Ubiquinone : 2 outcomes in 5 results : IBA
  4. Thiamine (Aneurin) : 1 outcome in 8 results : FDA 6
  5. Carnitine (L-Carnitine) : 1 outcome in 2 results : FDA 9 Generic
  6. idebenone : 1 outcome in 1 result : IBA
  7. Mitochondrial DNA (mtDNA) : 8 studies in 112 results : IBA
  8. Adenosine Triphosphatases (ATPase) : 2 studies in 20 results : IBA
  9. DNA (Deoxyribonucleic Acid) : 2 studies in 19 results : IBA
  10. Pyruvate Carboxylase : 2 studies in 5 results : IBA
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Diseases Related to Leigh Disease

  1. Respiratory Insufficiency (Respiratory Failure)
  2. Obstructive Sleep Apnea
  3. Mitochondrial Diseases (Mitochondrial Disease)
  4. Lactic Acidosis
  5. Ataxia (Dyssynergia)
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Key Therapies for Leigh Disease

Efficacy Chart >>
  1. Anesthesia : 3 results
  2. Ketogenic Diet : 1 result
  3. Optical Devices : 1 result
  4. Fundoplication : 1 result
  5. Kidney Transplantation : 1 result
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