ICD-10 Code E75.09 – Other GM2 gangliosidosis (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.09 – Other GM2 gangliosidosis
What it is
E75.09 identifies other GM2 gangliosidosis, a rare inherited lysosomal storage disorder caused by GM2 ganglioside buildup in the nervous system. Use it when the record names a GM2 gangliosidosis that is not specifically Tay-Sachs or Sandhoff disease.
Clinical signs
Clinical features vary; refer to documentation. Typical findings may include progressive neurologic decline, developmental regression, hypotonia, seizures, and vision or hearing loss in affected patients.
When to use this code
Use this code when the provider documents “other GM2 gangliosidosis,” “GM2 gangliosidosis NOS,” or a related GM2 storage disorder not otherwise classified. It also fits when the diagnosis is confirmed clinically or genetically and the chart does not specify a more exact GM2 subtype.
Do not use for
Do not use E75.09 for Tay-Sachs disease or Sandhoff disease, which have their own specific codes. If the documentation only mentions a nonspecific metabolic disorder, check documentation before assigning a GM2 gangliosidosis code.
Coding tip
Code the most specific GM2 diagnosis documented; if the subtype is unclear, query for clarification rather than defaulting to E75.09.