ICD10BillableValid for clinical use
ICD-10 Code G71.3: Mitochondrial myopathy, not elsewhere classified
Reviewed by HealthAssure Clinical TeamUpdated 26 May 2026
What is this code?
ICD-10 codes are diagnosis classification codes used in healthcare records, reporting, coding workflows, and billing support. This code sits within the broader ICD-10 area for Diseases of the nervous system (G00-G99).
When is it used?
- May be used when a clinician documents mitochondrial myopathy, not elsewhere classified in a patient's medical record.
- May appear in hospital records, claims, referrals, and clinical documentation.
- This code can be used as a clinically usable diagnosis entry in standardized coding workflows.
What it does not mean
- A code alone does not explain severity, treatment plan, or outcome.
- A medical code should not be treated as a substitute for a doctor's diagnosis or advice.
Code hierarchy
chapter
6Diseases of the nervous system (G00-G99)
block
G70-G73Diseases of myoneural junction and muscle
category
G71Primary disorders of muscles
currentG71
Official coding notes
Excludes1 (do not code together)
- Kearns-Sayre syndrome (H49.81)
- Leber's disease (H47.21)
- Leigh's encephalopathy (G31.82)
- mitochondrial metabolism disorders (E88.4.-)
- Reye's syndrome (G93.7)
Where you may see this code
You may see this code in hospital records, discharge summaries, insurance claims, encounter documentation, referrals, or other healthcare billing and coding records.
Related specialists
NeurologistGeneral Physician
Related codes
Sibling codes
Coverage-related procedures and services
CPT 95907high
NERVE CONDUCTION STUDIES; 1-2 STUDIES
Source: CMS coverage guidance
CPT 95912high
NERVE CONDUCTION STUDIES; 11-12 STUDIES
Source: CMS coverage guidance
CPT 95913high
NERVE CONDUCTION STUDIES; 13 OR MORE STUDIES
Source: CMS coverage guidance
CPT 95908high
NERVE CONDUCTION STUDIES; 3-4 STUDIES
Source: CMS coverage guidance
CPT 95909high
NERVE CONDUCTION STUDIES; 5-6 STUDIES
Source: CMS coverage guidance
CPT 95910high
NERVE CONDUCTION STUDIES; 7-8 STUDIES
Source: CMS coverage guidance
Mapped diagnoses and classifications
SNOMED_CT 725464001high
Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy
Source: SNOMED International ICD-10 map
SNOMED_CT 733599009high
Adult-onset multiple mitochondrial deoxyribonucleic acid deletion syndrome due to deoxyguanosine kinase deficiency
Source: SNOMED International ICD-10 map
SNOMED_CT 1222644009high
Autosomal dominant mitochondrial myopathy with exercise intolerance
Source: SNOMED International ICD-10 map
SNOMED_CT 773398005high
Congenital cataract, progressive muscular hypotonia, hearing loss, developmental delay syndrome
Source: SNOMED International ICD-10 map
SNOMED_CT 783057002high
DNA2-related mitochondrial DNA deletion syndrome
Source: SNOMED International ICD-10 map
SNOMED_CT 778029000high
FASTKD2-related infantile mitochondrial encephalomyopathy
Source: SNOMED International ICD-10 map
Coding guidelines
Compatibility
Legacy and official ICD code match exactly.
Legacy codes
G71.3
Common synonyms
Mitochondrial myopathy, not elsewhere classified
Frequently asked questions
About this content
This page is prepared by HealthAssure's clinical team using official coding standards from ICD-10. AI tools assist with drafting explanations, which are then reviewed and verified by healthcare professionals for accuracy. This content is for informational purposes and does not replace professional medical advice. Meet our team.