G71.3 MITOCHONDRIAL MYOPATHY, NOT ELSEWHERE CLASSIFIED

Code Information

  • Diagnosis Code: G71.3
  • Short Description: Mitochondrial myopathy, not elsewhere classified
  • Long Description: Mitochondrial myopathy, not elsewhere classified
  • Code Version: 2025 ICD-10-CM

The code G71.3 is VALID for claim submission

Code Classification

Synonyms

  • Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy
  • Adult-onset multiple mitochondrial deoxyribonucleic acid deletion syndrome due to deoxyguanosine kinase deficiency
  • Autosomal dominant mitochondrial myopathy with exercise intolerance
  • Childhood myocerebrohepatopathy spectrum
  • Combined oxidative phosphorylation defect type 39
  • Congenital cataract, hypertrophic cardiomyopathy, mitochondrial myopathy syndrome
  • Cytochrome-c oxidase deficiency
  • Diabetes mellitus associated with genetic syndrome
  • DNA2-related mitochondrial DNA deletion syndrome
  • FASTKD2-related infantile mitochondrial encephalomyopathy
  • FBXL4-related encephalomyopathic mitochondrial DNA depletion syndrome
  • Hereditary cerebellar atrophy
  • Hypertrophic mitochondrial cardiomyopathy
  • Lethal infantile mitochondrial myopathy
  • Maternally inherited mitochondrial cardiomyopathy
  • Maternally inherited mitochondrial cardiomyopathy and myopathy
  • Maternally inherited mitochondrial myopathy
  • Mitochondrial deoxyribonucleic acid depletion syndrome myopathic form
  • Mitochondrial DNA depletion syndrome encephalomyopathic form
  • Mitochondrial DNA depletion syndrome encephalomyopathic form
  • Mitochondrial DNA depletion syndrome encephalomyopathic form
  • Mitochondrial DNA depletion syndrome encephalomyopathic form
  • Mitochondrial DNA depletion syndrome encephalomyopathic form with methylmalonic aciduria
  • Mitochondrial DNA depletion syndrome, encephalomyopathic form with variable craniofacial anomalies
  • Mitochondrial encephalomyopathy
  • Mitochondrial myopathy
  • Mitochondrial myopathy with reversible cytochrome C oxidase deficiency
  • Mitochondrial myopathy with sideroblastic anemia syndrome
  • Mitochondrial myopathy, cerebellar ataxia, pigmentary retinopathy syndrome
  • Mitochondrial myopathy, lactic acidosis, deafness syndrome
  • Mitochondrial neurogastrointestinal encephalomyopathy syndrome
  • Myopathy and diabetes mellitus
  • Progressive external ophthalmoplegia, myopathy, emaciation syndrome
  • Pure mitochondrial myopathy
  • RRM2B-related mitochondrial DNA depletion syndrome, encephalomyopathic form with renal tubulopathy
  • Severe X-linked mitochondrial encephalomyopathy
  • TK2-related mitochondrial deoxyribonucleic acid depletion syndrome myopathic form
  • TMEM70 related mitochondrial encephalo-cardio-myopathy

References to Index of Diseases and Injuries

The code G71.3 has the following ICD-10-CM references to the Index of Diseases and Injuries

  • Type 1 Excludes Notes:
    • 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*)

Crosswalk Information

ICD-10 Code ICD-9 Code ICD-9 Description
G71.3 Right Arrow 359.89 Myopathies NEC
This ICD-10 to ICD-9 data is based on the 2018 General Equivalency Mapping (GEM) files published by the Centers for Medicare & Medicaid Services (CMS) for informational purposes only. The data is not an ICD-10 conversion tool and doesn’t guarantee clinical accuracy.

Similar Codes

ICD-10 Code ICD-10 Description
G71 Primary disorders of muscles
G71.0 Muscular dystrophy
G71.00 Muscular dystrophy, unspecified
G71.01 Duchenne or Becker muscular dystrophy
G71.02 Facioscapulohumeral muscular dystrophy
G71.03 Limb girdle muscular dystrophies
G71.031 Autosomal dominant limb girdle muscular dystrophy
G71.032 Autosomal recessive limb girdle muscular dystrophy due to calpain-3 dysfunction
G71.033 Limb girdle muscular dystrophy due to dysferlin dysfunction
G71.034 Limb girdle muscular dystrophy due to sarcoglycan dysfunction
G71.0340 Limb girdle muscular dystrophy due to sarcoglycan dysfunction, unspecified
G71.0341 Limb girdle muscular dystrophy due to alpha sarcoglycan dysfunction
G71.0342 Limb girdle muscular dystrophy due to beta sarcoglycan dysfunction
G71.0349 Limb girdle muscular dystrophy due to other sarcoglycan dysfunction
G71.035 Limb girdle muscular dystrophy due to anoctamin-5 dysfunction
G71.036 Limb girdle muscular dystrophy due to fukutin related protein dysfunction
G71.038 Other limb girdle muscular dystrophy
G71.039 Limb girdle muscular dystrophy, unspecified
G71.09 Other specified muscular dystrophies
G71.1 Myotonic disorders
G71.11 Myotonic muscular dystrophy
G71.12 Myotonia congenita
G71.13 Myotonic chondrodystrophy
G71.14 Drug induced myotonia
G71.19 Other specified myotonic disorders
G71.2 Congenital myopathies
G71.20 Congenital myopathy, unspecified
G71.21 Nemaline myopathy
G71.22 Centronuclear myopathy
G71.220 X-linked myotubular myopathy
G71.228 Other centronuclear myopathy
G71.29 Other congenital myopathy
G71.8 Other primary disorders of muscles
G71.9 Primary disorder of muscle, unspecified
This page was last updated on: 10/1/2025