ICD-10 Code G40.C01 – Lafora Progressive Myoclonus Epilepsy, Not Intractable, with Status Epilepticus (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Lafora progressive myoclonus epilepsy, not intractable, with status epilepticus is G40.C01.
2026 ICD-10-CM Diagnosis Code G40.C01 – Lafora progressive myoclonus epilepsy, not intractable, with status epilepticus

What it is

This code identifies Lafora progressive myoclonus epilepsy with status epilepticus, and it specifies that the seizures are not documented as intractable. Use it when the record clearly names this rare progressive epilepsy syndrome.

Clinical signs

Typical documentation includes progressive myoclonic seizures, generalized seizure activity, and episodes of status epilepticus. Clinical features vary; refer to documentation.

When to use this code

Use this code when the provider documents Lafora progressive myoclonus epilepsy and also documents status epilepticus. The chart should show that the epilepsy is not intractable. If the note only says Lafora epilepsy without seizure status detail, check documentation.

Do not use for

Do not use this code if status epilepticus is not documented, or if the epilepsy is described as intractable. Do not assign it for other seizure disorders or myoclonic epilepsies without Lafora disease.

Coding tip

Confirm both the Lafora diagnosis and the status epilepticus documentation before assigning G40.C01.

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