E75.01 ICD-10-CM Code: Sandhoff disease
E75.01 is not a CMS-HCC payment code. MEAT criteria · RAF Calculator · free HCC coding tools
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E75.01
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceSandhoff disease
Sandhoff disease is a rare inherited metabolic disorder where the body cannot break down certain fatty substances in the brain and nerve cells, leading to progressive neurological damage and developmental problems. It typically appears in infancy and causes severe developmental delays, loss of motor skills, blindness, and seizures.
Buddy Insight
Sandhoff disease is a rare autosomal recessive lysosomal storage disorder caused by deficiency of both hexosaminidase A and B enzymes, leading to toxic accumulation of GM2 gangliosides and other substrates in neurons and peripheral tissues.
CMS-HCC V28
N/A—
Not mapped
CMS-HCC V24
HistoricalHistorical
Not used for CY2026 payment
ACA/HHS
MappedHCC 119
Code-level coefficient reference
ESRD/PACE
MappedHCC 52
Code-level coefficient reference
RXHCC
MappedHCC 41
Code-level coefficient reference
Code Book Path
Inclusion Terms
OfficialNo inclusion terms are included in this display for E75.01. Check the code and parent instructions in the Code Book.
Excludes 2
Official- Ehlers-Danlos syndromes (Q79.6-)Inherited from E70-E88
Related Codes
Includes
OfficialNo Includes notes are included in this display for E75.01. Check the code and parent instructions in the Code Book.
Excludes 1
Official- transitory endocrine and metabolic disorders specific to newborn (P70-P74)Inherited from E00-E89, E70-E88, E75
- androgen insensitivity syndrome (E34.5-)Inherited from E00-E89, E70-E88, E75
- congenital adrenal hyperplasia (E25.0)Inherited from E00-E89, E70-E88, E75
- hemolytic anemias attributable to enzyme disorders (D55.-)Inherited from E00-E89, E70-E88, E75
- Marfan syndrome (Q87.4-)Inherited from E00-E89, E70-E88, E75
- 5-alpha-reductase deficiency (E29.1)Inherited from E00-E89, E70-E88, E75
- mucolipidosis, types I-III (E77.0-E77.1)Inherited from E00-E89, E70-E88, E75
- Refsum's disease (G60.1)Inherited from E00-E89, E70-E88, E75
Code First
OfficialNo Code First sequencing instructions are included in this display for E75.01. Check the code and parent instructions in the Code Book.
Use Additional
OfficialNo Use Additional Code instructions are included in this display for E75.01. Check the code and parent instructions in the Code Book.
Code Also
OfficialNo Code Also instructions are included in this display for E75.01. Check the code and parent instructions in the Code Book.
Buddy Documentation Tip
MEAT Support
Audit Caution
Common Mistakes
Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →
Is E75.01 an HCC code?
E75.01 has no mapping under the current CMS-HCC V28 community payment model. E75.01 has a separate mapping under the CMS-HCC ESRD model (HCC 52 (Dementia Without Complication)) and the Part D RxHCC model (HCC 41 (Lysosomal Storage Disorders)); the applicable result needs member context. E75.01 also appears in the HHS-HCC commercial risk model (HCC 119 (HHS-HCC 119 adult, RAF varies by metal level)), which is a commercial market model rather than a Medicare Advantage payment mapping.
- Code
- E75.01
- Description
- Sandhoff disease
- HCC (V28)
- No CMS-HCC V28 mapping
- RAF reference coefficient
- —
- Billable
- Yes
- Payment year
- 2026
HCC Category Mapping
These are source-labeled model coefficients, not member totals. A category may still be removed by hierarchy or model cleanup rules. Weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member context, hierarchy and cleanup rules, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains available for historical review.
Work E75.01 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →
MEAT review for E75.01
For E75.01, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.
- MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
- EEvaluate: test results, medication response, or physical findings reviewed by the provider
- AAssess: explicit mention in the assessment or plan with acknowledgment of status
- TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis
Coder workflow notes
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What This Code Means
E75.01 is the ICD-10-CM diagnosis code for sandhoff disease. Sandhoff disease is a rare inherited metabolic disorder where the body cannot break down certain fatty substances in the brain and nerve cells, leading to progressive neurological damage and developmental problems. It typically appears in infancy and causes severe developmental delays, loss of motor skills, blindness, and seizures. E75.01 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).
E75.01 has no mapping under the current CMS-HCC V28 community payment model. E75.01 has a separate mapping under the CMS-HCC ESRD model (HCC 52 (Dementia Without Complication)) and the Part D RxHCC model (HCC 41 (Lysosomal Storage Disorders)); the applicable result needs member context. E75.01 also appears in the HHS-HCC commercial risk model (HCC 119 (HHS-HCC 119 adult, RAF varies by metal level)), which is a commercial market model rather than a Medicare Advantage payment mapping. Do not assign V28 risk adjustment value from this page; verify the applicable model and payment year before using this code for risk adjustment.
Sandhoff disease is a specific lysosomal storage disorder; ensure you are not confusing it with Tay-Sachs disease (E75.00), which is a related but distinct condition.
HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for E75.01 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •Sandhoff disease is a specific lysosomal storage disorder; ensure you are not confusing it with Tay-Sachs disease (E75.00), which is a related but distinct condition
- •This code requires documentation of the diagnosis and may need to be linked with codes for associated complications such as seizures (G40.-), developmental delays (F88), or vision loss (H54.-) depending on the patient's clinical presentation
Clinical Significance
Sandhoff disease is a rare autosomal recessive lysosomal storage disorder caused by deficiency of both hexosaminidase A and B enzymes, leading to toxic accumulation of GM2 gangliosides and other substrates in neurons and peripheral tissues. Unlike Tay-Sachs, Sandhoff also affects visceral organs. The infantile form is rapidly fatal, while late-onset forms have a more protracted course with progressive neurological decline.
Documentation Requirements
- ✓Confirmed diagnosis of Sandhoff disease
- ✓Enzyme assay showing deficiency of both hexosaminidase A and B
- ✓Genetic testing for HEXB gene mutations if performed
- ✓Form specification: infantile, juvenile, or adult/chronic onset
- ✓Documentation of neurological status and progression
- ✓Visceral organ involvement assessment (hepatosplenomegaly, cardiac)
Commonly Confused Codes
- •E75.02: Tay-Sachs disease: deficiency of hexosaminidase A only, not both A and B
- •E75.00: GM2 gangliosidosis, unspecified: do not use when Sandhoff is confirmed
- •E75.09: Other GM2 gangliosidosis: for GM2 activator protein deficiency
- •E75.22: Gaucher disease: different lysosomal storage disorder with glucocerebrosidase deficiency
- •E75.10: Unspecified gangliosidosis: much less specific