G40.833
BillableDravet syndrome, 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
Dravet syndrome (a severe genetic epilepsy starting in infancy) that does not respond well to medication, currently experiencing a prolonged seizure state.
Coding Tips
- •Verify genetic testing or clinical diagnosis of Dravet syndrome is documented
- •Confirm status epilepticus is present and document the intractable nature with failed medication trials
Clinical Significance
Dravet syndrome (severe myoclonic epilepsy of infancy) is a rare, catastrophic genetic epilepsy caused primarily by SCN1A gene mutations. Intractable Dravet syndrome with status epilepticus is a life-threatening emergency, as these patients are highly susceptible to prolonged febrile and afebrile seizures. Dravet syndrome carries risk of sudden unexpected death in epilepsy (SUDEP) and requires lifelong specialized neurological care.
Documentation Requirements
- ✓Confirmed Dravet syndrome diagnosis (clinical or genetic confirmation of SCN1A mutation)
- ✓Documentation of intractability with list of failed antiepileptic drugs
- ✓Active status epilepticus during the encounter
- ✓Emergency interventions provided
- ✓Genetic testing results if available
- ✓Current medication regimen (noting contraindicated sodium channel blockers)
- ✓SUDEP risk discussion documentation
Commonly Confused Codes
G40.834 — Dravet syndrome, intractable, WITHOUT status epilepticusG40.823 — Epileptic spasms, intractable, with status epilepticus; different seizure typeG40.813 — Lennox-Gastaut, intractable, with status epilepticus; different syndromeG40.411 — Other generalized epilepsy, intractable, with status epilepticus; use when Dravet is not confirmedG40.42 — CDKL5 Deficiency; different genetic epilepsy
Code Hierarchy
└G40Epilepsy and recurrent seizures└G40.8Other epilepsy and recurrent seizures└G40.83Dravet syndrome└G40.833Dravet syndrome, intractable, with status epilepticus
└G40.833Dravet syndrome, intractable, with status epilepticus