E88.4

Non-Billable
ChapterE00–E89
Ranksubcategory
Official Description

Mitochondrial metabolism disorders

Metadata & Insights
  • •2026 ICD-10-CM Edition - Effective October 1, 2025.
  • •Non-billable category (requires more specific sub-code).
  • •Chapter 4: Endocrine, Nutritional and Metabolic Diseases (E00-E89).

Instructional Notes

Excludes1(Not coded here)
  • disorders of pyruvate metabolism (E74.4)
  • Kearns-Sayre syndrome (H49.81)
  • Leber's disease (H47.22)
  • Leigh's encephalopathy (G31.82)
  • Mitochondrial myopathy, NEC (G71.3)
  • Reye's syndrome (G93.7)
General instructions are available for the parent sections:
View CH4 NotesView E70-E88 NotesView E88 Notes

Clinical Hierarchy

CH4Endocrine, nutritional and metabolic diseases (E00-E89)
E70-E88Metabolic disorders (E70-E88)
E88Other and unspecified metabolic disorders
E88.4Mitochondrial metabolism disorders
E88.40Mitochondrial metabolism disorder, unspecified
E88.41MELAS syndrome
E88.42MERRF syndrome
E88.43Disorders of mitochondrial tRNA synthetases
E88.49Other mitochondrial metabolism disorders

Applicable Ancestor Context

The following parent sections contain instructions or notes that apply to E88.4:

categoryE88
Other and unspecified metabolic disorders
View context instructions
blockE70-E88
Metabolic disorders (E70-E88)
View context instructions
chapterCH4
Endocrine, nutritional and metabolic diseases (E00-E89)
View context instructions

Alphabetic Index Mentions

These are Diagnosis Index lookup paths that route to E88.4. Use them as entry terms for search, then confirm final coding instructions in the Tabular List and notes above.

21 raw paths
Disorder (of)8 unique routes
metabolism NOS(7 variants)
mitochondrial→E88.4(7 variants)
aminoacyl-tRNA synthetase→E88.4
ARS2-related→E88.4
MELAS syndrome→E88.4
MERRF syndrome (myoclonic epilepsy associated with ragged-red fibers)→E88.4
other specified→E88.4
tRNA synthetases→E88.4
mitochondrial metabolism→E88.4
LBSL (leukoencephalopathy with brainstem - spinal cord involvement - lactate elevation)1 unique routes
Direct index entry→E88.4
Leukoencephalopathy2 unique routes
with(2 variants)
brainstem - spinal cord involvement - lactate elevation→E88.4
thalamus - brainstem involvement - high lactate→E88.4
LTBL (leukoencephalopathy with thalamus - brainstem involvement - high lactate)1 unique routes
Direct index entry→E88.4
MELAS syndrome1 unique routes
Direct index entry→E88.4
MERRF syndrome (myoclonic epilepsy associated with ragged-red fiber)1 unique routes
Direct index entry→E88.4
MNGIE (Mitochondrial Neurogastrointestinal Encephalopathy) syndrome1 unique routes
Direct index entry→E88.4
NARP (Neuropathy, Ataxia and Retinitis pigmentosa) syndrome1 unique routes
Direct index entry→E88.4
Syndrome5 unique routes
Fukuhara→E88.4
MELAS→E88.4
MERRF (myoclonic epilepsy associated with ragged-red fibers)→E88.4
MNGIE (Mitochondrial Neurogastrointestinal Encephalopathy)→E88.4
NARP (Neuropathy, Ataxia and Retinitis pigmentosa)→E88.4

MS-DRG Classification

2026

No direct MS-DRG triggers identified.

Cross Reference

Find Procedures (ICD-10-PCS)

Find surgical and procedural codes related to Mitochondrial metabolism disorders.

Search PCS Codes