G71.3 Mitochondrial myopathy, not elsewhere classified

Billable ICD-10-CM code, FY2026.

Excludes1 (never 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)

Applies from G71 Primary disorders of muscles

Excludes2 (not included here)

• arthrogryposis multiplex congenita (Q74.3)
• metabolic disorders (E70-E88)
• myositis (M60.-)

MS-DRG index

Defines the logic of DRG(s) 091-093 in MDC 01.

Approximate ICD-9-CM (GEMs)

• 359.89 Other myopathies (approximate)

Code annotations containing back-references to G71.3

Back-references to G71.3

• Type 1 Excludes: E88.4 Mitochondrial metabolism disorders ("Mitochondrial myopathy, NEC (G71.3)")

Back-references to G71

• Code First: M62.84 Sarcopenia ("primary disorders of muscles (G71.-)")

Diagnosis Index entries for G71.3

• Myopathy › mitochondrial NEC

Nearby codes

• G71.21 Nemaline myopathy
• G71.22 Centronuclear myopathy
• G71.220 X-linked myotubular myopathy
• G71.228 Other centronuclear myopathy
• G71.29 Other congenital myopathy
• G71.3 Mitochondrial myopathy, not elsewhere classified (this code)
• G71.8 Other primary disorders of muscles
• G71.9 Primary disorder of muscle, unspecified