ICD-10 Code G40.C1 – Lafora progressive myoclonus epilepsy, intractable (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code G40.C1 – Lafora progressive myoclonus epilepsy, intractable
What it is
G40.C1 identifies Lafora progressive myoclonus epilepsy when it is documented as intractable. Use it for this rare, inherited epileptic syndrome marked by progressive neurologic decline and difficult-to-control seizures.
Clinical signs
Typical findings include myoclonic jerks, generalized seizures, and worsening neurologic function over time. Clinical features vary; refer to documentation for the specific seizure pattern and evidence that the epilepsy is intractable.
When to use this code
Use this code when the provider documents Lafora progressive myoclonus epilepsy and states it is intractable, refractory, or poorly controlled. It may also be supported by a clear history of persistent seizures despite appropriate therapy. Confirm the diagnosis is specifically Lafora disease, not another myoclonic epilepsy.
Do not use for
Do not use this code for generalized epilepsy, isolated myoclonus, or seizures without documentation of Lafora disease. If intractability is not documented, check documentation before assigning this code.
Coding tip
Code only when both the Lafora diagnosis and intractable status are clearly documented by the clinician.