ICD-10 Code E75.0 – GM2 gangliosidosis (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.0 – GM2 gangliosidosis
What it is
E75.0 identifies GM2 gangliosidosis, a group of inherited lysosomal storage disorders that cause harmful GM2 ganglioside buildup in the nervous system. It is used for the diagnosis as documented, not for symptoms alone.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include progressive neurodevelopmental decline, hypotonia, motor regression, seizures, and vision loss. Some patients have an early-onset, rapidly progressive course.
When to use this code
Use E75.0 when the provider documents GM2 gangliosidosis, including Tay-Sachs disease or Sandhoff disease when specified under this diagnosis family. Code it from confirmed clinical documentation, genetic testing, or specialist assessment.
Choose this code when the record names the disorder rather than only listing nonspecific neurologic symptoms. If the chart is unclear, check documentation before assigning the code.
Do not use for
Do not use E75.0 for isolated developmental delay, seizures, or vision problems without a documented GM2 gangliosidosis diagnosis. Do not use it for other storage disorders or unspecified neurodegenerative disease.
Coding tip
Verify the exact inherited disorder named in the record, and code any documented subtype only if the documentation supports it.