ICD-10-CM 2027 diagnosis code
E75.01Sandhoff disease
E75.01 is a valid, billable ICD-10-CM code for sandhoff disease. It can be reported on claims for dates of service from October 1, 2026 – September 30, 2027.
Looking up by condition? See ICD-10 code for Sandhoff's disease.
Code unchanged since ICD-10-CM took effect on October 1, 2015. Source: official FY2027 ICD-10-CM release from CDC/NCHS and CMS. Page updated September 29, 2026. About our data
About E75.01
E75.01 is the ICD-10-CM diagnosis code for Sandhoff disease. It belongs to category E75 (disorders of sphingolipid metabolism and other lipid storage disorders), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.
Within E75.0 (GM2 gangliosidosis), E75.01 is specifically for Sandhoff disease. Related codes cover unspecified (E75.00), Tay-Sachs disease (E75.02) and other GM2 gangliosidosis (E75.09).
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
In MS-DRG v44.0, it is part of the grouping logic for DRG 056 (Degenerative Nervous System Disorders with MCC, relative weight 2.2655) and DRG 057 (Degenerative Nervous System Disorders without MCC, relative weight 1.2838), in MDC 01 (Diseases and Disorders of the Nervous System), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.
Before ICD-10, this condition was coded in ICD-9-CM as 330.1 (cerebral lipidoses).
Notes that apply from higher levels
Instructions written at a parent level also apply to E75.01.
Broader instructions also apply from E70-E88 Metabolic disorders and Chapter 4: Endocrine, nutritional and metabolic diseases.
Alphabetic index entries
2 entriesTerms in the official ICD-10-CM index that lead to E75.01.
MS-DRG v44.0 grouping
All DRGs →Inpatient MS-DRGs this diagnosis can group to, as the principal or a secondary diagnosis, depending on the rest of the claim.
MDC 01 · Diseases and Disorders of the Nervous System
ICD-9-CM equivalent
Converter →- 330.1Cerebral lipidosesapproximate
From the CMS 2018 General Equivalence Mappings (GEMs), the final GEMs release.
Code history
- 2016
- 2017
- 2018
- 2019
- 2020
- 2021
- 2022
- 2023
- 2024
- 2025
- 2026
- 2027
- FY2016 (effective 10/1/2015): Added (first year of ICD-10-CM)
- No changes since FY2016.
Common questions about E75.01
Is E75.01 billable?
- Yes. E75.01 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E75.01 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E75.01 as a principal diagnosis.
Is E75.01 a CC or MCC?
- E75.01 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.
What DRG does E75.01 group to?
- E75.01 is used in the MS-DRG v44.0 logic for MS-DRG 056 (Degenerative Nervous System Disorders with MCC) and MS-DRG 057 (Degenerative Nervous System Disorders without MCC). Which one applies depends on whether it is the principal diagnosis and on the rest of the claim.
What is the ICD-9 code for E75.01?
- The CMS General Equivalence Mappings map E75.01 to ICD-9-CM 330.1.