E75.4

Neuronal ceroid lipofuscinosis

Billable FY2026 2025-10-01 → 2026-09-30

Exclusions 9 never code together

E75code

Excludes1

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

E70-E88block

Excludes1

androgen insensitivity syndrome (E34.5-)E34.5-
congenital adrenal hyperplasia (E25.0)E25.0
hemolytic anemias attributable to enzyme disorders (D55.-)D55.-
Marfan syndrome (Q87.4-)Q87.4-
5-alpha-reductase deficiency (E29.1)E29.1

Excludes2

Ehlers-Danlos syndromes (Q79.6-)Q79.6-

E00-E89chapter

Excludes1

transitory endocrine and metabolic disorders specific to newborn (P70-P74)P70-P74

Excludes1 — never code together. The excluded condition and this one are mutually exclusive.

Excludes2 — not included here, but the patient may have both. Code both when documented.

Instructional notes 5

E75.4code

Inclusion terms

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

E00-E89chapter

Notes

All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.E05.8 E07.0 E16-E31 E34.-

Index entries leading here 13

Idiot, idiocy (congenital) F73
- amaurotic (Bielschowsky(-Jansky)) (family) (infantile (late)) (juvenile (late)) (Vogt-Spielmeyer) E75.4
Amaurotic idiocy (infantile) (juvenile) (late) E75.4
Batten (-Mayou) disease E75.4
- retina E75.4
Disease, diseased — see also Syndrome
- retina, retinal H35.9
  - Batten's or Batten-Mayou E75.4
Bielschowsky (-Jansky) disease E75.4
Degeneration, degenerative
- brain (cortical) (progressive) G31.9
  - in
    - lipidosis
      - cerebral E75.4
Lipidosis E75.6
- cerebral (infantile) (juvenile) (late) E75.4
- cerebroretinal E75.4
Ceroid-lipofuscinosis, neuronal E75.4
Jansky-Bielschowsky amaurotic idiocy E75.4
Kufs' disease E75.4
Lipofuscinosis, neuronal (with ceroidosis) E75.4
Spielmeyer-Vogt disease E75.4
Vogt-Spielmeyer amaurotic idiocy or disease E75.4

Alongside this code 6 same parent — E75

E75.0 GM2 gangliosidosis category E75.1 Other and unspecified gangliosidosis category E75.2 Other sphingolipidosis category E75.3 Sphingolipidosis, unspecified E75.5 Other lipid storage disorders E75.6 Lipid storage disorder, unspecified

Medications 1

1 Neuronal Ceroid-Lipofuscinoses drugs

MED-RT files conditions by MeSH, which carves the world differently from ICD-10. These are name matches on this code's description, offered as candidates — not as an assertion that they are this code.

Additional references

AHA Coding Clinic® Licence required

Official coding advice for this code. Published by the American Hospital Association. Not included in this installation.

Risk adjustment

ESRD21HCC52 ESRD24HCC52 RX08HCC41

Presence is a property of the code. Untick a model to hide it everywhere.

This code

Billable: yes
Code set: FY2026
Parent: E75
Block: E70-E88
Siblings: 6

Present FY2024–FY2027.