E75.4 Neuronal ceroid lipofuscinosis

The ICD-10-CM code for Neuronal ceroid lipofuscinosis is E75.4 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Chapter

4: Endocrine, nutritional and metabolic diseases (E00-E89)

Section

E70-E88: Metabolic disorders (E70-E88)

Category E75

31 codes (26 billable)

FY2026 Status

Stable since FY2024

Also Known As

ICD-10-CM Alphabetic Index entries that lead to E75.4:

• Batten (-Mayou) disease
• Lipofuscinosis, neuronal (with ceroidosis)
• Bielschowsky (-Jansky) disease
• Jansky-Bielschowsky amaurotic idiocy
• Amaurotic idiocy (infantile) (juvenile) (late)
• Kufs' disease
• Spielmeyer-Vogt disease
• Ceroid-lipofuscinosis, neuronal

Inclusion Terms

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

U.S. Hospital Utilization

• An estimated 340 U.S. inpatient stays in 2023 included E75.4 among the documented diagnoses.

Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.

Official Coding Guidelines

Diabetes mellitus (TOC entry)

a. Diabetes mellitus ................................................................................................................... 39

Obesity (TOC entry)

b. Obesity .................................................................................................................................. 42

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

• CMS — ICD-10-CM Official Code Set and Guidelines, FY2026
• CDC/NCHS — ICD-10-CM Files and Browser Tool
• AHRQ — HCUP National Inpatient Sample, Frequencies by Diagnosis Code (2016–2023)