What is ICD-10 code E75.4?

E75.4 is the ICD-10-CM diagnosis code for Neuronal ceroid lipofuscinosis. A group of rare inherited neurological disorders characterized by progressive accumulation of lipofuscin (a fatty pigment) in nerve cells, causing progressive dementia and loss of function. E75.4 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. E75.4 is part of the FY2026 ICD-10-CM code set published by the CMS National Center for Health Statistics and used on Medicare, Medicaid, and commercial claims in the United States.

Yes. E75.4 is a billable ICD-10-CM code that can be reported on professional and institutional claims as a primary or secondary diagnosis when the provider documentation supports the specific condition described. Billable codes in ICD-10-CM are coded to the highest level of specificity available for the documented diagnosis. E75.4 is current for fiscal year 2026 and remains in the active CMS ICD-10-CM code set published by the National Center for Health Statistics.

Which HCC does E75.4 map to under V28?

E75.4 does not map to a CMS-HCC V28 payment category. Check the current CMS-HCC V28 model file for payment year 2026 rather than an older V24 tip sheet.

What does E75.4 pay in RAF?

E75.4 has no separately stated V28 community RAF weight. If it is not a V28 payment HCC, it adds nothing to a community RAF score.

What is the V28 vs V24 difference for E75.4?

CMS phased in the V28 risk adjustment model over payment years 2024 (33%), 2025 (67%), and 2026 (100%), replacing V24. V28 consolidates HCC categories, removes some lower-acuity conditions, and recalibrates RAF weights across the entire model. E75.4 was a payment HCC under V24 (Dementia Without Complication) but the V28 recalibration removed it from the risk adjustment model. Both models can still appear in legacy data and in payer-specific lookback periods.

2026 ICD-10-CM E75.4: Neuronal ceroid

E75.4 is not a CMS-HCC payment code. MEAT criteria · RAF calculator · free HCC coding tools

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Code lookupE75.4Effective periodFY2026 Apr Update (Apr 1-Sep 30)

FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)

E75.4

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Neuronal ceroid lipofuscinosis

A group of rare inherited neurological disorders characterized by progressive accumulation of lipofuscin (a fatty pigment) in nerve cells, causing progressive dementia and loss of function.

Buddy Insight

CMS-HCC V28

N/A

—

Not risk-adjusted for this model/period.

Not mapped

CMS-HCC V24

Mapped

HCC 52

Dementia Without Complication

RAF 0.346

ACA/HHS

Mapped

HCC 119

HHS-HCC 119

Varies by metal level

ESRD/PACE

Mapped

HCC 52

Dementia Without Complication

RAF 0.042

RXHCC

Mapped

HCC 41

Lysosomal Storage Disorders

RAF 3.081

Code Book Path

Official

E75Disorders of sphingolipid metabolism and other lipid storage disorders

E75.4Neuronal ceroid lipofuscinosis

Inclusion Terms

Official

Batten disease
Bielschowsky-Jansky disease
Kufs disease
Spielmeyer-Vogt disease

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E75.4 in this effective period.

Related Child Codes

Official

E75.0GM2 gangliosidosis

E75.1Other and unspecified gangliosidosis

E75.2Other sphingolipidosis

E75.3Sphingolipidosis, unspecified

E75.5Other lipid storage disorders

Includes

Official

ICD-10-CM does not list Includes notes for E75.4 in this effective period.

Excludes 1

Official

mucolipidosis, types I-III (E77.0-E77.1)
Refsum's disease (G60.1)

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E75.4 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E75.4 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E75.4 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance

Specific NCL type if known (CLN1-CLN14 or infantile, late infantile, juvenile, adult forms)

Confirmatory diagnostic evidence (genetic testing identifying CLN gene mutations, enzyme assay, or electron microscopy showing curvilinear profiles)

Current neurological status and functional assessment

Seizure frequency and anticonvulsant management

MEAT Support

HCC Buddy guidance

Specific NCL type if known (CLN1-CLN14 or infantile, late infantile, juvenile, adult forms)

Confirmatory diagnostic evidence (genetic testing identifying CLN gene mutations, enzyme assay, or electron microscopy showing curvilinear profiles)

Current neurological status and functional assessment

Seizure frequency and anticonvulsant management

Audit Caution

HCC Buddy guidance

Miscategorizing NCL as a sphingolipidosis rather than a ceroid lipofuscinosis

Failing to code the associated neurological manifestations (seizures, vision loss) as additional diagnoses

Using a nonspecific lipid storage disorder code when the provider has documented NCL or Batten disease

Not recognizing that Batten disease is a common name for juvenile NCL and should be coded to E75.4

Common Mistakes

HCC Buddy guidance

E75.29 — Other sphingolipidosis: NCL is NOT a sphingolipidosis; it involves ceroid-lipofuscin, not sphingolipids

E75.5 — Other lipid storage disorders: a broader category that should not be used when NCL is specifically diagnosed

G31.89 — Other specified degenerative diseases of nervous system: NCL is metabolic, not a primary neurodegenerative code

E75.6 — Lipid storage disorder, unspecified: too nonspecific when NCL is confirmed

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 E75.4 an HCC code?

Yes. E75.4 maps to Dementia Without Complication under the V24 model but is not retained in V28.

Code: E75.4
Description: Neuronal ceroid lipofuscinosis
HCC (V28): No CMS-HCC V28 mapping
RAF: —
Billable: Yes
Payment year: 2026

HCC Category Mapping

V24HCC 52, Dementia Without Complication

0.346

ESRDHCC 52, Dementia Without Complication

0.042

RxHCCHCC 41, Lysosomal Storage Disorders

3.081

Each model's RAF is its CMS base weight for that model's standard population, so 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 segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.

Work E75.4 in the Code Book — tabular path, V28 RAF, and MEAT checklist →

MEAT Criteria for E75.4

For E75.4 to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.

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

Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E75.4 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E75.4 is the ICD-10-CM diagnosis code for neuronal ceroid lipofuscinosis. A group of rare inherited neurological disorders characterized by progressive accumulation of lipofuscin (a fatty pigment) in nerve cells, causing progressive dementia and loss of function. E75.4 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

Under the older CMS-HCC V24 model, E75.4 maps to Dementia Without Complication (HCC 52) with a community, non-dual, aged base RAF weight of 0.346. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.

Document the age of onset and clinical presentation (infantile, late infantile, juvenile, or adult form). Because E75.4 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for E75.4 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•Document the age of onset and clinical presentation (infantile, late infantile, juvenile, or adult form)
•Link to associated symptoms such as vision loss, seizures, or cognitive decline

Clinical Significance

Neuronal ceroid lipofuscinosis (NCL) encompasses a group of inherited neurodegenerative lysosomal storage disorders characterized by progressive accumulation of autofluorescent lipopigments in neurons and other cells. These are among the most common neurodegenerative disorders of childhood, causing seizures, vision loss, cognitive decline, and premature death, requiring extensive supportive care resources.

Documentation Requirements

✓Specific NCL type if known (CLN1-CLN14 or infantile, late infantile, juvenile, adult forms)
✓Confirmatory diagnostic evidence (genetic testing identifying CLN gene mutations, enzyme assay, or electron microscopy showing curvilinear profiles)
✓Current neurological status and functional assessment
✓Seizure frequency and anticonvulsant management
✓Vision status and ophthalmologic findings
✓Documentation of progressive nature and disease trajectory

Commonly Confused Codes

•E75.29: Other sphingolipidosis: NCL is NOT a sphingolipidosis; it involves ceroid-lipofuscin, not sphingolipids
•E75.5: Other lipid storage disorders: a broader category that should not be used when NCL is specifically diagnosed
•G31.89: Other specified degenerative diseases of nervous system: NCL is metabolic, not a primary neurodegenerative code
•E75.6: Lipid storage disorder, unspecified: too nonspecific when NCL is confirmed