ICD-10 Code E88.41 – MELAS syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E88.41 – MELAS syndrome
What it is
E88.41 identifies MELAS syndrome, a mitochondrial disorder that affects energy production in the body. It is used when the record specifically documents MELAS syndrome.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include stroke-like episodes, seizures, muscle weakness, migraine-like headaches, and lactic acidosis.
When to use this code
Use E88.41 when the clinician diagnoses MELAS syndrome or documents it as the confirmed cause of neurologic or metabolic findings. Code it from the final assessment, problem list, or discharge summary when the diagnosis is established.
Do not assign it for nonspecific mitochondrial disease unless MELAS is clearly named. If the chart only suggests the condition, check documentation before coding.
Do not use for
Do not use E88.41 for isolated seizures, stroke-like symptoms, or lactic acidosis without a documented MELAS diagnosis. Do not substitute it for other mitochondrial disorders.
Coding tip
Look for the exact phrase “MELAS syndrome” in provider documentation before assigning E88.41.