ICD-10 Code E75.4 – Neuronal ceroid lipofuscinosis (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.4 – Neuronal ceroid lipofuscinosis
What it is
E75.4 identifies neuronal ceroid lipofuscinosis, a group of inherited lysosomal storage disorders. It causes harmful pigment-like material to build up in nerve cells and other tissues, leading to progressive neurologic decline.
Clinical signs
Clinical features vary; refer to documentation. Common findings include progressive vision loss, seizures, developmental regression, movement problems, and cognitive decline. Diagnosis is usually confirmed by genetics, enzyme testing, or characteristic clinical and pathologic findings.
When to use this code
Use E75.4 when the record documents neuronal ceroid lipofuscinosis, Batten disease, or a clearly stated NCL subtype. Code it for confirmed disease, not for isolated neurologic symptoms alone. If the subtype is specified, follow documentation and coding guidance.
Do not use for
Do not use this code for nonspecific seizures, developmental delay, or vision loss without a documented NCL diagnosis. Check documentation if the chart names another storage disorder or a different hereditary neurodegenerative condition.
Coding tip
Capture the exact subtype or genetic confirmation when documented, because it may support more precise coding and medical record clarity.