ICD-10 Code G31.82 – Leigh’s Disease (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Leigh’s disease is G31.82.
2026 ICD-10-CM Diagnosis Code G31.82 – Leigh’s disease

What it is

G31.82 identifies Leigh’s disease, a rare progressive neurodegenerative disorder. It is used when documentation confirms this specific mitochondrial or metabolic encephalopathy.

Clinical signs

Clinical features vary; refer to documentation. Common findings include developmental regression, hypotonia, movement abnormalities, seizures, and brainstem or basal ganglia involvement on imaging.

When to use this code

Use G31.82 when the provider documents Leigh’s disease, Leigh syndrome, or a clearly equivalent diagnosis. It may also apply when records describe a confirmed mitochondrial disorder consistent with Leigh’s disease. If the chart only notes nonspecific encephalopathy, check documentation before coding.

Do not use for

Do not use this code for general developmental delay, unspecified neurodegeneration, or suspected mitochondrial disease without confirmation. If the diagnosis is uncertain, check documentation.

Coding tip

Match the code to the exact documented disease name, and query if the record says only “possible” or “rule out” Leigh’s disease.

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