ICD-10-CM 2027
ICD-10 code for NARP syndrome
From the official ICD-10-CM alphabetic index entry “NARP (Neuropathy, Ataxia and Retinitis pigmentosa) syndrome”. Page updated September 29, 2026.
About coding NARP syndrome
The ICD-10-CM code for NARP syndrome is E88.49 (Other mitochondrial metabolism disorders).
Within E88.4, choose E88.49 (Other mitochondrial metabolism disorders) only when documentation doesn't support a more specific option: mitochondrial metabolism disorder, unspecified (E88.40), MELAS syndrome (E88.41), MERRF syndrome (E88.42) and disorders of mitochondrial tRNA synthetases (E88.43).
Use an additional code for: codes for associated conditions.
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
Common questions about NARP syndrome ICD-10 codes
What is the ICD-10 code for NARP syndrome?
- E88.49 — Other mitochondrial metabolism disorders.
Is E88.49 billable?
- Yes. E88.49 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E88.49 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E88.49 as a principal diagnosis.
Is E88.49 a CC or MCC?
- E88.49 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.