ICD-10 Code E88.49 – Other mitochondrial metabolism disorders (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E88.49 – Other mitochondrial metabolism disorders
What it is
E88.49 identifies mitochondrial metabolism disorders that do not fit a more specific ICD-10-CM category. Use it for documented mitochondrial dysfunction or inherited mitochondrial disease when the record does not name a narrower code.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings in mitochondrial disorders include muscle weakness, exercise intolerance, neurologic involvement, and multisystem disease, but the exact presentation depends on the specific condition.
When to use this code
Use this code when the provider documents an “other” mitochondrial metabolism disorder and no more specific diagnosis is available. It also fits confirmed mitochondrial disease entries that are not otherwise classified in ICD-10-CM. Check the note for the exact disorder name before coding.
Do not use for
Do not use E88.49 for a more specific mitochondrial disorder that has its own code. Do not assign it when the chart only suggests mitochondrial disease without clear provider documentation; check documentation.
Coding tip
Look for the provider’s exact wording, because mitochondrial disorders are often coded more specifically when named.