2026 ICD-10-CM Diagnosis Code E75.4Neuronal ceroid lipofuscinosis

ICD-10-CM Codes›E00–E89›E70-E88›E75

ICD-10-CM E75.4
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E75.4 is a billable ICD-10-CM diagnosis code for neuronal ceroid lipofuscinosis. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). 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
E75.4
Billable Status
Yes — Valid for Submission
Code Describes
Neuronal ceroid lipofuscinosis
Short Description
Neuronal ceroid lipofuscinosis
Same as the full description in the CMS dataset.
Parent Code
E75
Disorders of sphingolipid metabolism and other lipid storage disorders

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE75Disorders of sphingolipid metabolism and other lipid storage disorders
This CodeE75.4Neuronal ceroid lipofuscinosis

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Acquired ataxia
  • Adult neuronal ceroid lipofuscinosis
  • ATPase cation transporting 13A2 related juvenile neuronal ceroid lipofuscinosis
  • Cerebral degeneration in childhood
  • Cerebral lipidosis
  • Congenital neuronal ceroid lipofuscinosis
  • Dementia associated with cerebral lipidosis
  • Infantile neuronal ceroid lipofuscinosis
  • Juvenile neuronal ceroid lipofuscinosis
  • Late-infantile neuronal ceroid lipofuscinosis
  • Myoclonic disorder due to neuronal ceroid lipofuscinosis
  • Neuronal ceroid lipofuscinosis
  • Neuronal ceroid lipofuscinosis 8
  • Neuronal ceroid lipofuscinosis type 6A

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

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

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.

    • Amaurotic idiocy(infantile) (juvenile) (late)
    • Batten(-Mayou) disease
    • Batten(-Mayou) disease
      • retina
    • Bielschowsky(-Jansky) disease
    • Ceroid-lipofuscinosis, neuronal
    • Degeneration, degenerative
      • brain (cortical) (progressive)
        • in
          • lipidosis
            • cerebral
    • Disease, diseased
      • retina, retinal
        • Batten's or Batten-Mayou
    • Idiot, idiocy(congenital)
      • amaurotic (Bielschowsky (-Jansky)) (family) (infantile (late)) (juvenile (late)) (Vogt-Spielmeyer)
    • Jansky-Bielschowsky amaurotic idiocy
    • Kufs' disease
    • Lipidosis
      • cerebral (infantile) (juvenile) (late)
    • Lipidosis
      • cerebroretinal
    • Lipofuscinosis, neuronal(with ceroidosis)
    • Spielmeyer-Vogt disease
    • Vogt-Spielmeyer amaurotic idiocy or 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

Patient EducationClinical

Genetic Brain Disorders

A genetic brain disorder is caused by a variation or a mutation in a gene. A variation is a different form of a gene. A mutation is a change in a gene. Genetic brain disorders affect the development and function of the brain.

Read the full article at MedlinePlus

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

Convert E75.4 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 E75.4Overview

Is E75.4 a billable code?

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

What is the ICD-9 equivalent of E75.4?

Under the General Equivalence Mappings, neuronal ceroid lipofuscinosis 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.