Neuronal ceroid lipofuscinosis

This code identifies a group of inherited neurodegenerative disorders characterized by the accumulation of lipopigment (ceroid and lipofuscin) in neurons and other cells throughout the body. These lysosomal storage diseases lead to progressive neurological dysfunction, including seizures, vision loss, motor deterioration, and cognitive decline.

Assign this code for patients diagnosed with any form of neuronal ceroid lipofuscinosis (NCL), often referred to as Batten disease. Documentation should clearly state the diagnosis of NCL, Batten disease, or a specific subtype (e.g., infantile, late infantile, juvenile, adult NCL).

• Ensure the medical record specifies a diagnosis of neuronal ceroid lipofuscinosis rather than a general neurodegenerative disorder.
• Do not confuse this with other lysosomal storage disorders; this code is specific to NCL.
• Consider sequencing this code as a primary diagnosis when it is the reason for the encounter, or as a secondary diagnosis when managing complications or related symptoms.