ICD-10 Code E75.00 – GM2 gangliosidosis, unspecified (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.00 – GM2 gangliosidosis, unspecified
What it is
GM2 gangliosidosis, unspecified is a lysosomal storage disorder in which GM2 gangliosides build up because of defective enzyme activity. Use this code when the record confirms GM2 gangliosidosis but does not identify a specific subtype.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include progressive neurologic decline, developmental delay or regression, hypotonia, and seizures. Some patients may also have vision loss or an exaggerated startle response.
When to use this code
Use E75.00 when the clinician documents GM2 gangliosidosis without naming Tay-Sachs disease, Sandhoff disease, or another specific form. It is appropriate for problem lists, discharge diagnoses, or referrals when the subtype remains unspecified.
If genetic, enzyme, or specialist notes later identify a precise GM2 variant, code the documented specific condition instead. Check documentation when the chart only mentions “gangliosidosis” or another broader storage disorder.
Do not use for
Do not use this code for a confirmed specific GM2 subtype, such as Tay-Sachs or Sandhoff disease. Also avoid it when the record describes another metabolic disorder without clear GM2 gangliosidosis documentation.
Coding tip
Choose the unspecified code only when the subtype is not stated anywhere in the available record.