G40.C01
BillableLafora progressive myoclonus epilepsy, not intractable, with status epilepticus
HCC Category Mapping
What This Code Means
Lafora disease is a rare, inherited progressive epilepsy that causes myoclonic seizures and progressive neurological decline, typically beginning in childhood. This code indicates the condition is medication-responsive but the patient is currently experiencing status epilepticus.
Coding Tips
- •Lafora disease is a progressive myoclonus epilepsy; ensure genetic or pathological confirmation is documented
- •Document presence of status epilepticus and that the condition is not intractable (responds to treatment)
Clinical Significance
Lafora progressive myoclonus epilepsy with status epilepticus is a rare, fatal autosomal recessive disorder caused by mutations in the EPM2A or NHLRC1 genes. Lafora disease causes progressive neurological decline with myoclonic seizures, cognitive deterioration, and visual hallucinations. Status epilepticus in Lafora disease is a medical emergency that may be particularly difficult to manage given the progressive nature of the disease.
Documentation Requirements
- ✓Confirmed Lafora disease diagnosis (genetic testing or skin biopsy showing Lafora bodies)
- ✓Active status epilepticus during the encounter
- ✓Statement that seizures currently respond to medication
- ✓Emergency interventions for status epilepticus
- ✓Current medication regimen
- ✓Documentation of disease progression stage