Neurology & Pain Management Coding Alert

Question: A newborn was diagnosed with Ohtahara syndrome. Is there an ICD-9 code for this?

Nevada Subscriber

Answer: There is no specific listing in the current ICD-9 alphabetic index for Ohtahara syndrome and for the forthcoming year. By following ICD-9 guidelines, however, you can use 779.0 (Convulsions in newborn).

The updated ICD-9-CM Official Guidelines for Coding and Reporting, effective Oct. 1, has a new general coding guideline when confronted with this coding quandary:

"Follow the alphabetic index guidance when coding syndromes. In the absence of index guidance, assign codes for the documented manifestations of the syndrome." Ohtahara syndrome is primarily a seizure disorder found in newborns. Therefore, you can use codes for other and ill-defined conditions originating in the perinatal period. You thus apply 779.0

Don't miss: EEG testing (95812-95822) is usuallyordered to confirm diagnosis of Ohtahara syndrome. The EEGs of infants with Ohtahara syndrome will reveal a characteristic "burst suppression pattern" -- a highvoltage spike wave discharge followed by little activity.

Ohtahara syndrome is also known as early infantile epileptic encephalopathy (EIEE) and is a form of progressive epileptic encephalopathy. This condition often results in severe mental retardation.

According to the National Institute of Neurologic Disorders and Stroke (NINDS), Ohtahara syndrome is a neurological disorder characterized by seizures (www.ninds.nih.gov/disorders/ohtahara/ohtahara.htm). The disorder affects newborns, usually within the first three months of life (most often within the first 10 days) in the form of epileptic seizures. Infants have primarily tonic seizures, but may also experience partial seizures, and rarely, myoclonic seizures.