KCNQ2-related epilepsy, intractable, with status epilepticus

This code identifies a specific genetic form of epilepsy caused by a mutation in the KCNQ2 gene. It signifies that the seizures are difficult to control with standard treatment (intractable) and are currently presenting as status epilepticus, a prolonged or rapidly recurring seizure without full recovery of consciousness between episodes.

Apply this code when documentation clearly states a diagnosis of KCNQ2-related epilepsy, explicitly noting its intractable nature and the presence of status epilepticus. This is typically seen in pediatric neurology settings following genetic testing confirmation and acute management of prolonged seizures.

• Ensure documentation specifies "KCNQ2-related" and not just a general genetic epilepsy.
• Verify both "intractable" and "status epilepticus" are clearly documented; absence of either requires a different code.
• Sequence this code as the primary diagnosis when it is the reason for the encounter, followed by any associated manifestations or complications.