ICD10BillableValid for clinical use

ICD-10 Code E75.4: Neuronal ceroid lipofuscinosis

E75.4 is a billable ICD-10 diagnosis code used to classify Neuronal ceroid lipofuscinosis in medical records and claims. You may see this code in hospital records, discharge summaries, insurance claims, encounter documentation, referrals, or other healthcare billing and coding records. ICD-10 codes are diagnosis classification codes used in healthcare records, reporting, coding workflows, and billing support. This code sits within the broader ICD-10 area for Endocrine, nutritional and metabolic diseases (E00-E89).

Reviewed by HealthAssure Clinical TeamUpdated 26 May 2026

What is this code?

ICD-10 codes are diagnosis classification codes used in healthcare records, reporting, coding workflows, and billing support. This code sits within the broader ICD-10 area for Endocrine, nutritional and metabolic diseases (E00-E89).

When is it used?

  • May be used when a clinician documents neuronal ceroid lipofuscinosis in a patient's medical record.
  • May appear in hospital records, claims, referrals, and clinical documentation.
  • This code can be used as a clinically usable diagnosis entry in standardized coding workflows.

What it does not mean

  • A code alone does not explain severity, treatment plan, or outcome.
  • A medical code should not be treated as a substitute for a doctor's diagnosis or advice.

Code hierarchy

chapter
4Endocrine, nutritional and metabolic diseases (E00-E89)
block
E70-E88Metabolic disorders
category
E75Disorders of sphingolipid metabolism and other lipid storage disorders
currentE75

Official coding notes

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

Where you may see this code

You may see this code in hospital records, discharge summaries, insurance claims, encounter documentation, referrals, or other healthcare billing and coding records.

Related specialists

EndocrinologistGeneral Physician

Coverage-related procedures and services

CPT 70460high
COMPUTED TOMOGRAPHY, HEAD OR BRAIN; WITH CONTRAST MATERIAL(S)
Source: CMS coverage guidance
CPT 70450high
COMPUTED TOMOGRAPHY, HEAD OR BRAIN; WITHOUT CONTRAST MATERIAL
Source: CMS coverage guidance
CPT 70470high
COMPUTED TOMOGRAPHY, HEAD OR BRAIN; WITHOUT CONTRAST MATERIAL, FOLLOWED BY CONTRAST MATERIAL(S) AND FURTHER SECTIONS
Source: CMS coverage guidance
CPT 96105high
ASSESSMENT OF APHASIA (INCLUDES ASSESSMENT OF EXPRESSIVE AND RECEPTIVE SPEECH AND LANGUAGE FUNCTION, LANGUAGE COMPREHENSION, SPEECH PRODUCTION ABILITY, READING, SPELLING, WRITING, EG, BY BOSTON DIAGNOSTIC APHASIA EXAMINATION) WITH INTERPRETATION AND REPORT, PER HOUR
Source: CMS coverage guidance
CPT 92651high
AUDITORY EVOKED POTENTIALS; FOR HEARING STATUS DETERMINATION, BROADBAND STIMULI, WITH INTERPRETATION AND REPORT
Source: CMS coverage guidance
CPT 92652high
AUDITORY EVOKED POTENTIALS; FOR THRESHOLD ESTIMATION AT MULTIPLE FREQUENCIES, WITH INTERPRETATION AND REPORT
Source: CMS coverage guidance

Coding guidelines

Compatibility

Legacy and official ICD code match exactly.
Legacy codes
E75.4

Common synonyms

Neuronal ceroid lipofuscinosis

Frequently asked questions

Code details

CodeE75.4
SystemICD10
Display nameNeuronal ceroid lipofuscinosis
ChapterEndocrine, nutritional and metabolic diseases (E00-E89)
BlockMetabolic disorders

Flags

BillableYes
Valid clinical useYes

Source

SourceICD-10
Version2026-annual
Releaseannual
Year2026

Index terms

Matched terms
Amaurotic idiocy(infantile) (juvenile) (late)Batten(-Mayou)diseaseBatten(-Mayou)diseaseBielschowsky(-Jansky)diseaseCeroid-lipofuscinosis, neuronalDegeneration, degenerativeDisease, diseasedIdiot, idiocy(congenital)Jansky-Bielschowsky amaurotic idiocyKufs' diseaseLipidosisLipidosis

About this content

This page is prepared by HealthAssure's clinical team using official coding standards from ICD-10. AI tools assist with drafting explanations, which are then reviewed and verified by healthcare professionals for accuracy. This content is for informational purposes and does not replace professional medical advice. Meet our team.

ICD-10 Code E75.4 for Neuronal ceroid lipofuscinosis | HealthAssure