G40.C11
BillableLafora progressive myoclonus epilepsy, intractable, with status epilepticus
HCC Category Mapping
V28HCC 201 — Seizure Disorders and Convulsions
0.262V24HCC 79 — Seizure Disorders and Convulsions
0.244ESRDHCC 79 — Seizure Disorders and Convulsions
0.000RxHCCHCC 163 — Seizure Disorders, Intractable Epilepsy
0.000What This Code Means
Lafora disease that does not respond adequately to medication, with the patient currently experiencing status epilepticus, a serious condition of prolonged or repeated seizures.
Coding Tips
- •Document intractability and confirm Lafora disease diagnosis through genetic or pathological evidence
- •Status epilepticus requires emergency intervention; ensure acute care documentation is present
Clinical Significance
Lafora progressive myoclonus epilepsy, intractable, with status epilepticus is an extremely severe and life-threatening presentation of this fatal genetic disease. At this stage, patients have refractory seizures with status epilepticus on a background of progressive neurological decline. Management is largely palliative, focusing on comfort and seizure mitigation rather than cure.
Documentation Requirements
- ✓Confirmed Lafora disease diagnosis
- ✓Documentation of intractability with failed medication trials
- ✓Active status epilepticus during the encounter
- ✓Emergency and palliative care interventions
- ✓Disease stage and functional status
- ✓Goals of care discussion documentation
Commonly Confused Codes
G40.C01 — Lafora disease, NOT intractable, with status epilepticusG40.C19 — Lafora disease, intractable, WITHOUT status epilepticusG40.813 — Lennox-Gastaut, intractable, with status epilepticus; non-progressive in the same wayG40.833 — Dravet syndrome, intractable, with status epilepticus; different genetic conditionG40.911 — Unspecified epilepsy, intractable, with status epilepticus; much less specific
Code Hierarchy
└G40Epilepsy and recurrent seizures└G40.CLafora progressive myoclonus epilepsy└G40.C1Lafora progressive myoclonus epilepsy, intractable└G40.C11Lafora progressive myoclonus epilepsy, intractable, with status epilepticus
└G40.C11Lafora progressive myoclonus epilepsy, intractable, with status epilepticus