ICD10BillableValid for clinical use
ICD-10 Code E75.4: Neuronal ceroid lipofuscinosis
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
Related codes
Sibling codes
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
Mapped diagnoses and classifications
SNOMED_CT 789657008high
ATPase cation transporting 13A2 related juvenile neuronal ceroid lipofuscinosis
Source: SNOMED International ICD-10 map
SNOMED_CT 62009002high
Adult neuronal ceroid lipofuscinosis
Source: SNOMED International ICD-10 map
SNOMED_CT 16517004high
Cerebral lipidosis
Source: SNOMED International ICD-10 map
SNOMED_CT 720830009high
Congenital neuronal ceroid lipofuscinosis type 10
Source: SNOMED International ICD-10 map
SNOMED_CT 720831008high
Deficiency of cathepsin D
Source: SNOMED International ICD-10 map
SNOMED_CT 58258004high
Infantile neuronal ceroid lipofuscinosis
Source: SNOMED International ICD-10 map
Coding guidelines
Compatibility
Legacy and official ICD code match exactly.
Legacy codes
E75.4
Common synonyms
Neuronal ceroid lipofuscinosis
Frequently asked questions
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.