ICD-10-CM E88.4: Mitochondrial metabolism disorders

ICD-10-CM Diagnosis Code (2025)

Official Description for E88.4

Mitochondrial metabolism disorders

Coding Notes & Guidelines for E88.4

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)
Inclusion Terms

Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes

Inclusion Terms

Myoclonic epilepsy associated with ragged-red fibers

Code Also

progressive myoclonic epilepsy (G40.3-)

Parent Code (Less Specific):

About ICD-10-CM Code E88.4 (Mitochondrial metabolism disorders)

ICD-10-CM code E88.4 is a billable or specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2025 edition of ICD-10-CM E88.4 became effective on October 1, 2024.

This page provides detailed information about E88.4 - Mitochondrial metabolism disorders. It includes common terminology, coding guidelines such as "Includes," "Excludes1," and "Excludes2" notes, and information on required 7th characters. Utilizing this information correctly is essential for accurate medical coding and claims submission for conditions like Mitochondrial metabolism disorders.