2026 ICD-10-CM Diagnosis Code E75.01Sandhoff disease

ICD-10-CM Codes›E00–E89›E70-E88›E75

ICD-10-CM E75.01
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E75.01 is a billable ICD-10-CM diagnosis code for sandhoff disease. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as adult chronic GM2 gangliosidosis. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other nervous system disorders (often hereditary or degenerative) and Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E75.01
Billable Status
Yes — Valid for Submission
Code Describes
Sandhoff disease
Short Description
Sandhoff disease
Same as the full description in the CMS dataset.
Parent Code
E75.0
GM2 gangliosidosis

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE75Disorders of sphingolipid metabolism and other lipid storage disorders
This CodeE75.01Sandhoff disease

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Adult chronic GM2 gangliosidosis
  • Infantile GM2 gangliosidosis
  • Juvenile GM2 gangliosidosis
  • Sandhoff disease
  • Total hexosaminidase deficiency - adult
  • Total hexosaminidase deficiency - infantile
  • Total hexosaminidase deficiency - juvenile

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Gangliosidosis
      • GM2
        • Sandhoff disease
    • Sandhoff's disease

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR NVS006
Other nervous system disorders (often hereditary or degenerative)
Default principal diagnosis: inpatient No · outpatient No
CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Sandhoff Disease

    an autosomal recessive neurodegenerative disorder characterized by an accumulation of g(m2) ganglioside in neurons and other tissues. it is caused by mutation in the common beta subunit of hexosaminidase a and hexosaminidase b. thus this disease is also known as the o variant since both hexosaminidase a and b are missing. clinically, it is indistinguishable from tay-sachs disease.

Patient EducationClinical

Tay-Sachs Disease

Tay-Sachs disease is a rare, inherited disease. It is a type of lipid metabolism disorder. It causes too much of a fatty substance to build up in the brain. This buildup destroys nerve cells, causing mental and physical problems.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert E75.01 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
330.1 Cerebral lipidoses
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E75.01Overview

Is E75.01 (GM2 gangliosidosis) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report sandhoff disease on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E75.01?

Under the General Equivalence Mappings, sandhoff disease converts to ICD-9-CM 330.1 (cerebral lipidoses). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.