ICD-10 Code E75.01 – Sandhoff disease (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.01 – Sandhoff disease
What it is
E75.01 identifies Sandhoff disease, a rare inherited lysosomal storage disorder. It causes harmful buildup of GM2 gangliosides in the body, leading to progressive neurologic decline.
Clinical signs
Common findings include developmental delay or regression, hypotonia, weakness, and worsening neurologic impairment. Clinical features vary; refer to documentation.
When to use this code
Use E75.01 when the provider documents Sandhoff disease or confirms the diagnosis in the assessment. It is appropriate for encounters focused on evaluation, follow-up, or management of the disorder.
Code it from the documented diagnosis, not from family history or a nonspecific neurodegenerative workup. If the record only suggests a storage disorder, Check documentation.
Do not use for
Do not use this code for Tay-Sachs disease, GM2 gangliosidosis without further specification, or unrelated developmental delay. Check documentation when the chart does not clearly name Sandhoff disease.
Coding tip
Confirm the exact inherited disorder name in the provider note before assigning E75.01.