AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2020 Issue 4; New/Revised ICD-10-CM Codes

Dravet Syndrome

Two new codes have been created to identify Dravet syndrome, intractable, with status epilepticus (G40.833) and without status epilepticus (G40.834).Dravet syndrome is a rare genetic encephalopathy that presents in the first year of life. It was previously known as severe myoclonic epilepsy in infancy and represents approximately 7% of all severe epilepsies starting before the age of three years. During the first year of life, the condition appears as frequent febrile seizures. As the condition progresses, typically other types of seizures, including myoclonus and status epilepticus, occur. Around the age of two years, intellectual development begins...

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