Leigh syndrome is a rare genetic neurometabolic disorder. It is characterized by the degeneration of the central nervous system (i.e., brain, spinal cord, and optic nerve).
Leigh's disease can be caused by mutations in mitochondrial DNA or by deficiencies of an enzyme called pyruvate dehydrogenase. Symptoms of Leigh's disease usually progress rapidly. The earliest signs may be poor sucking ability,and the loss of head control and motor skills.
The diagnosis of Leigh's syndrome is based on clinical features confirmed by imaging tests. The symptoms in younger patients include loss of head control (the infant can no longer hold up the head) and failure to suck normally.
Leigh syndrome has multiple causes, all of which imply a defect in aerobic energy production, ranging from the pyruvate dehydrogenase complex to the oxidative phosphorylation pathway.
Certain tissues that require large amounts of energy, such as the brain, muscles, and heart, seem especially sensitive to decreases in cellular energy. Cell death in the brain likely causes the characteristic lesions seen in Leigh syndrome, which contribute to the signs and symptoms of the condition.
The prognosis of Leigh disorder is very poor, with patients mostly living up to six to seven years after diagnosis. Although some patients may live until mid-teenage years. Leigh syndrome is a rare inherited genetic disorder that affects the central nervous system.
Leigh syndrome is a rare, inherited neurodegenerative condition. It usually becomes apparent in infancy, often after a viral infection. Signs and symptoms usually progress rapidly. Early symptoms may include poor sucking ability; loss of head control and motor skills; loss of appetite; vomiting; and seizures.
Leigh's syndrome is a severe mitochondrial disease. It is an inherited disease that causes a disruption in cellular respiration via mutations in electron transport chain (ECT) components.
The most common treatment for Leigh's disease is thiamine or Vitamin B1. Oral sodium bicarbonate or sodium citrate may also be prescribed to manage lactic acidosis. Researchers are currently testing dichloroacetate to establish its effectiveness in treating lactic acidosis.
If the genetic variant is present either in the nuclear or mitochondrial DNA then there is nothing that can be taken during pregnancy or given to the infant that will prevent Leigh syndrome occurring.
The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code G31.82:
The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code (s). The following references for the code G31.82 are found in the index:
The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:
LEIGH DISEASE-. 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.
The General Equivalency Mapping (GEM) crosswalk indicates an approximate mapping between the ICD-10 code G31.82 its ICD-9 equivalent. The approximate mapping means there is not an exact match between the ICD-10 code and the ICD-9 code and the mapped code is not a precise representation of the original code.
Degenerative nerve diseases affect many of your body's activities, such as balance, movement, talking, breathing, and heart function. Many of these diseases are genetic. Sometimes the cause is a medical condition such as alcoholism, a tumor, or a stroke. Other causes may include toxins, chemicals, and viruses. Sometimes the cause is unknown.
The ICD code G318 is used to code Neuropathy, ataxia, and retinitis pigmentosa. Neuropathy, ataxia, and retinitis pigmentosa, also known as NARP syndrome, is a rare disease with mitochondrial inheritance that causes a variety of signs and symptoms chiefly affecting the nervous system.
This is the official approximate match mapping between ICD9 and ICD10, as provided by the General Equivalency mapping crosswalk. This means that while there is no exact mapping between this ICD10 code G31.82 and a single ICD9 code, 330.8 is an approximate match for comparison and conversion purposes.