ICD-10 Code E75.1 – Other and unspecified gangliosidosis (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.1 – Other and unspecified gangliosidosis
What it is
E75.1 identifies gangliosidosis that is not otherwise specified or does not fit a more specific subtype. It is a rare inherited lysosomal storage disorder involving abnormal ganglioside accumulation.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include progressive neurologic decline, developmental delay or regression, and organ involvement, but the exact presentation depends on the subtype.
When to use this code
Use this code when the record states gangliosidosis and no more specific ICD-10-CM code is documented. It also fits when the provider documents an unspecified or mixed gangliosidosis diagnosis. Verify the note for a named subtype before assigning E75.1.
Do not use for
Do not use E75.1 when the documentation identifies a specific gangliosidosis subtype with its own code. Do not use it for unrelated storage disorders or nonspecific neurologic symptoms without a documented diagnosis.
Coding tip
Check the provider’s exact wording first; if the subtype is named, code that specific diagnosis instead of E75.1.