icd 10 code for leigh syndrome

What is a Leigh syndrome?

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).

Why is it called Leigh syndrome?

Leigh syndrome (also called Leigh disease and subacute necrotizing encephalomyelopathy) is an inherited neurometabolic disorder that affects the central nervous system. It is named after Archibald Denis Leigh, a British neuropsychiatrist who first described the condition in 1951.

Is Leigh syndrome a neurodegenerative disease?

The most common pediatric mitochondrial disease is Leigh syndrome, an episodic, subacute neurodegeneration that can lead to death within the first few years of life, for which there are no proven general therapies.

What is maternally inherited Leigh syndrome?

Maternally inherited Leigh syndrome (MILS) and neuropathy, ataxia and retinitis pigmentosa (NARP) syndrome are rare genetic multisystem disorders that are part of a spectrum or continuum of disease caused by abnormalities affecting mitochondrial energy production.

How is Leigh syndrome diagnosed?

The signs and symptoms of Leigh syndrome are caused in part by patches of damaged tissue (lesions) that develop in the brains of people with this condition. A medical procedure called magnetic resonance imaging (MRI) reveals characteristic lesions in certain regions of the brain .

Is Leigh syndrome a infectious disease?

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.

Is Leigh's syndrome anaerobic or aerobic?

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.

How is Leigh syndrome related to cellular respiration?

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. Leigh's is a class I mitochondrial disease that involves defects directly in mitochondrial DNA (mtDNA).

What is the difference between nuclear DNA and mtDNA?

Mitochondrial DNA, unlike nuclear DNA, is inherited from the mother, while nuclear DNA is inherited from both parents. So this is very helpful sometimes in determining how a person has a certain disorder in the family. Sometimes a disease will be inherited through the mother's line, as opposed to both parents.

How does thiamine help with Leigh syndrome?

It is suggested that the therapeutic effect of high doses of thiamine given to patients who suffer from Leigh's disease is, at least in part, due to maintainance of the pyruvate dehydrogenase complex in its active form, thus facilitating the oxidation of pyruvate.

Can Leigh syndrome be prevented?

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.

When was Leigh syndrome discovered?

Definition and History Leigh syndrome (LS or subacute necrotizing encephalomyelopathy) was originally described in 1951 by Dr. Denis Leigh, a British neurologist, who reported a 6.5-month-old infant boy presenting with developmental regression that progressed quickly and led to death 6 weeks later. At autopsy, Dr.

Tabular List of Diseases and Injuries

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:

Index to Diseases and Injuries

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:

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

Clinical Information

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.

Convert G31.82 to ICD-9 Code

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.

Information for Patients

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.

What is the ICD code for neuropathy?

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.

What is the approximate match between ICd9 and ICd10?

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.

What is Leigh syndrome?

Leigh syndrome (also called Leigh disease and subacute necrotizing encephalomyelopathy) is an inherited neurometabolic disorder that affects the central nervous system. It is named after Archibald Denis Leigh, a British neuropsychiatrist who first described the condition in 1951.

How old is the average Leigh syndrome?

French Canadian Leigh syndrome has similar symptoms to other types of Leigh syndrome. The age of onset is, on average, 5 months and the median age of death is 1 year and 7 months.

How long does Leigh syndrome last?

The symptoms of Leigh syndrome are classically described as beginning in infancy and leading to death within a span of several years; however, as more cases are recognized, it is apparent that symptoms can emerge at any age—including adolescence or adulthood—and patients can survive for many years following diagnosis.

How is Leigh syndrome inherited?

Leigh syndrome caused by nuclear DNA mutations is inherited in an autosomal recessive pattern. This means that two copies of the mutated gene are required to cause the disease, so two unaffected parents, each of whom carries one mutant allele, can have an affected child if that child inherits the mutant allele from both parents.

What is the most common cause of death in people with Leigh syndrome?

However, respiratory failure is the most common cause of death in people with Leigh syndrome. Other neurological symptoms include peripheral neuropathy, loss of sensation in extremities caused by damage to the peripheral nervous system.

Where is Leigh syndrome found?

French Canadian Leigh syndrome. The type of Leigh syndrome found at a much higher rate in the Saguenay-Lac-Saint-Jean region of Quebec is caused by a mutation in the LRPPRC gene, located on the small ('p') arm of chromosome 2.

When was Leigh syndrome first described?

Leigh syndrome was first described by Denis Leigh in 1951 and distinguished from similar Wernicke's encephalopathy in 1954. In 1968, the disease's link with mitochondrial activity was first ascertained, though the mutations in cytochrome c oxidase and other electron transport chain proteins were not discovered until 1977.

What is the syndrome of cataracts?

A syndrome of congenital cataracts, hydrophthalmia, delayed growth and mental development, vitamin d-resistant rickets, aminoaciduria, and reduced production of ammonia by the kidneys. Additional manifestations include areflexia, hypotonia, glaucoma, corneal keloid, and noninflammatory arthropathy.

What does the title of a manifestation code mean?

In most cases the manifestation codes will have in the code title, "in diseases classified elsewhere.". Codes with this title are a component of the etiology/manifestation convention. The code title indicates that it is a manifestation code.