Adult-onset multiple mitochondrial DNA (deoxyribonucleic acid) deletion syndrome due to DGUOK (deoxyguanosine kinase) deficiency
Genetic syndromes of diabetes mellitus
Booth Haworth Dilling syndrome
Mitochondrial encephalomyopathy
Mitochondrial myoencephalopathy
Myopathy, lactic acidosis and sideroblastic anaemia
Mitochondrial DNA depletion syndrome encephalomyopathic form with methylmalonic aciduria
Mitochondrial deoxyribonucleic acid depletion syndrome, encephalomyopathic form with variable craniofacial anomalies
Mitochondrial neurogastrointestinal encephalomyopathy syndrome
Benign COX (cytochrome C oxidase) deficiency
PEO (progressive external ophthalmoplegia) myopathy emaciation syndrome
COX - Cytochrome C oxidase deficiency
Mitochondrial myopathy, cerebellar atrophy, pigmentary retinopathy syndrome
Mitochondrial myopathy with reversible cytochrome C oxidase deficiency
FBXL4-related encephalomyopathic mitochondrial DNA depletion syndrome
Mitochondrial DNA depletion syndrome encephalomyopathic form
GTP dependent ribosome recycling factor mitochondrial 2-related combined oxidative phosphorylation defect
TK2-related mitochondrial deoxyribonucleic acid depletion syndrome myopathic form
Progressive external ophthalmoplegia, myopathy, emaciation syndrome
FBXL4-related early onset mitochondrial encephalopathy
Reversible infantile respiratory chain deficiency
Mitochondrial complex IV deficiency
Mitochondrial DNA depletion syndrome, encephalomyopathic form with variable craniofacial anomalies
Hereditary cerebellar atrophy
Diabetes mellitus associated with genetic syndrome
Pure mitochondrial myopathy
Mitochondrial DNA depletion syndrome 2 myopathic type
Childhood myocerebrohepatopathy spectrum
Hypertrophic mitochondrial cardiomyopathy
Lethal infantile mitochondrial disease
Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy
Combined oxidative phosphorylation defect type 39
DNA replication helicase/nuclease 2-related mitochondrial deoxyribonucleic acid deletion syndrome
Ragged red myopathy
Mitochondrial myopathy with sideroblastic anemia syndrome
FASTKD2-related infantile mitochondrial encephalomyopathy
Myopathy and diabetes mellitus
Mitochondrial DNA depletion syndrome 8A encephalomyopathic type with renal tubulopathy
Mitochondrial encephalomyopathy due to combined oxidative phosphorylation deficiency 6
COXPD39 - combined oxidative phosphorylation defect type 39
Mitochondrial encephalomyopathy due to COXPD6 deficiency
Mitochondrial DNA depletion syndrome, encephalomyopathic form with renal tubulopathy
TK2-related mitochondrial DNA depletion myopathy
Congenital cataract, hypertrophic cardiomyopathy, mitochondrial myopathy syndrome
Mitochondrial DNA deletion syndrome with limb-girdle weakness
Mitochondrial DNA deletion syndrome with progressive myopathy
Sengers syndrome
Mitochondrial deoxyribonucleic acid depletion syndrome myopathic form
TMEM70 related mitochondrial encephalo-cardio-myopathy
Adult-onset CPEO (chronic progressive external ophthalmoplegia) with mitochondrial myopathy
Autosomal dominant mitochondrial myopathy with exercise intolerance
Mitochondrial deoxyribonucleic acid depletion syndrome encephalomyopathic form
F-box and leucine rich repeat protein 4 related mitochondrial deoxyribonucleic acid depletion syndrome encephalomyopathic form
Adult-onset multiple mitochondrial deoxyribonucleic acid deletion syndrome due to deoxyguanosine kinase deficiency
Mitochondrial myopathy, cerebellar ataxia, pigmentary retinopathy syndrome
Mitochondrial encephalomyopathy aminoacidopathy syndrome
GFM2-related combined oxidative phosphorylation defect
Mitochondrial myopathy with sideroblastic anaemia syndrome
Ribonucleotide reductase regulatory TP53 inducible subunit M2B-related mitochondrial deoxyribonucleic acid depletion syndrome encephalomyopathic form with renal tubulopathy
TK2-related mitochondrial DNA depletion syndrome myopathic form
mtDNA (mitochondrial deoxyribonucleic acid) depletion syndrome encephalomyopathic form with methylmalonic aciduria
Complex IV deficiency
Lethal infantile mitochondrial myopathy
FBXL4 (F-box and leucine rich repeat protein 4) related early onset mitochondrial encephalopathy
Mitochondrial myopathy, lactic acidosis, hearing loss syndrome
Cytochrome c oxidase deficiency
FAST kinase domains 2-related infantile mitochondrial encephalomyopathy
Mitochondrial DNA (deoxyribonucleic acid) maintenance syndrome due to MGME1 (mitochondrial genome maintenance exonuclease 1) deficiency
Severe X-linked mitochondrial encephalomyopathy
Mitochondrial encephalocardiomyopathy due to transmembrane protein 70 mutation
Mitochondrial myopathy with reversible COX (cytochrome C oxidase) deficiency
Mitochondrial myopathy, lactic acidosis, deafness syndrome
Infantile reversible cytochrome C oxidase deficiency myopathy
mtDNA (mitochondrial deoxyribonucleic acid) depletion syndrome, encephalomyopathic form with variable craniofacial anomalies
Mitochondrial DNA depletion syndrome 13 encephalomyopathic type
Myopathy, lactic acidosis and sideroblastic anemia
Maternally inherited mitochondrial cardiomyopathy and myopathy
Mitochondrial deoxyribonucleic acid depletion syndrome encephalomyopathic form with methylmalonic aciduria
RRM2B-related mitochondrial DNA depletion syndrome, encephalomyopathic form with renal tubulopathy
DNA2-related mitochondrial DNA deletion syndrome
Reversible infantile cytochrome C oxidase deficiency
Maternally inherited mitochondrial myopathy
Mitochondrial myopathy with reversible complex IV deficiency
Cytochrome-c oxidase deficiency
Adult-onset multiple mitochondrial DNA deletion syndrome due to DGUOK deficiency
Maternally inherited mitochondrial cardiomyopathy

Frequently Asked Questions

What is the ICD-10 code for mitochondrial myopathy, not elsewhere classified?

The ICD-10-CM code for mitochondrial myopathy, not elsewhere classified is G71.3. The full clinical description is "Mitochondrial myopathy, not elsewhere classified". G71.3 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code G71.3 mean?

ICD-10-CM code G71.3 represents “Mitochondrial myopathy, not elsewhere classified”. It is classified under Chapter 6: Diseases of the Nervous System and is a billable/specific code that can be used on a claim.

Is G71.3 a billable code?

Yes, G71.3 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.

What chapter is G71.3 in?

G71.3 is in Chapter 6: Diseases of the Nervous System (codes G00-G99).

What codes cannot be used with G71.3?

G71.3 has Excludes1 notes indicating codes that cannot be used together with it, including: Kearns-Sayre syndrome (H49.81); Leber's disease (H47.21); Leigh's encephalopathy (G31.82); and 2 more.

What SNOMED CT codes does G71.3 map to?

G71.3 maps to 35 SNOMED CT concepts: 725464001, 733599009, 1222644009, 784370005, 1197052008, and 30 more. SNOMED CT is a clinical terminology used in electronic health records.

What are the UMLS CUIs for G71.3?

G71.3 is linked to 1 UMLS Concept Unique Identifier: C0869052. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.

How does G71.3 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like mitochondrial myopathy, not elsewhere classified affect a person's functioning — body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.

What is the ICD-11 equivalent of G71.3?

G71.3 maps to the ICD-11 code: 8C73.Z (Mitochondrial myopathies, unspecified).

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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.