Developmental and epileptic encephalopathy

This code represents a severe group of neurological disorders characterized by both developmental regression or stagnation and intractable epilepsy. These encephalopathies often manifest in early childhood, leading to significant cognitive and motor impairments due to the underlying brain dysfunction caused by the epileptic activity.

Apply this code for patients diagnosed with a developmental and epileptic encephalopathy, where the epileptic activity itself is believed to contribute to the developmental impairment. Documentation should clearly indicate both the presence of epilepsy and the associated developmental delay or regression, often with a specific type of encephalopathy (e.g., Lennox-Gastaut syndrome, Dravet syndrome) mentioned.

• Ensure documentation explicitly links the epilepsy to the developmental issues, rather than two co-occurring but unrelated conditions.
• Do not use this code for epilepsy without documented developmental regression or for developmental delays without an associated epileptic encephalopathy.
• Always sequence this code as a primary diagnosis when it is the main reason for the encounter, or as a secondary diagnosis when it is a significant co-morbidity impacting care.