E75.4
BillableNeuronal ceroid lipofuscinosis
Neuronal ceroid lipofuscinosis
Coding Notes
Inclusion Terms
Alternative clinical terms for this condition
- Batten disease
- Bielschowsky-Jansky disease
- Kufs disease
- Spielmeyer-Vogt disease
Excludes 1
Codes that cannot be used together with this code (mutual exclusion)
- •transitory endocrine and metabolic disorders specific to newbornP70-P74
- •androgen insensitivity syndromeE34.5
- •congenital adrenal hyperplasiaE25.0
- •hemolytic anemias attributable to enzyme disordersD55
- •Marfan syndromeQ87.4
- •5-alpha-reductase deficiencyE29.1
- •mucolipidosis, types I-IIIE77.0-E77.1
- •Refsum's diseaseG60.1
Related Codes(6)
ICD-11 Equivalents(1)
ICD-11 Equivalents
View full mappingCorresponding ICD-11 codes from the WHO crosswalk mapping
Also Known As / Clinical Terms(348)
SNOMED CT
- Cerebral degeneration in childhood2584003
- Amaurotic idiocy early juvenile type14637005
- Amaurotic idiocy late infantile type14637005
- Amaurotic idiocy, early juvenile type14637005
- Amaurotic idiocy, late infantile type14637005
- Bielschowsky-Jansky disease14637005
- Bielschowsky-Jansky type neuronal ceroid lipofuscinosis14637005
- Dollinger-Bielschowsky syndrome14637005
- Dollinger-Bielschowsky type neuronal ceroid lipofuscinosis14637005
- Late infantile neuronal ceroid lipofuscinosis14637005
- Late-infantile neuronal ceroid lipofuscinosis14637005
- Cerebral lipidosis16517004
- Cerebromacular degeneration42012007
- Cerebromacular dystrophy42012007
- Neuronal ceroid lipofuscinosis42012007
- Pigmentary retinal lipoid neuronal heredodegeneration42012007
- Hagberg-Santavouri type neuronal ceroid lipofuscinosis58258004
- Hagberg-Santavuori disease58258004
- Haltia-Santavouri type neuronal ceroid lipofuscinosis58258004
- Infantile neuronal ceroid lipofuscinosis58258004
- Neuronal ceroid lipofuscinosis infantile Finnish type58258004
- Neuronal ceroid lipofuscinosis, infantile Finnish type58258004
- Polyunsaturated acid lipidosis58258004
- Polyunsaturated fatty acid lipidosis58258004
- Santavuori disease58258004
- Amaurotic idiocy juvenile type61663001
- Amaurotic idiocy, juvenile type61663001
- Batten-Mayou disease61663001
- Batten-Mayou syndrome61663001
- Batten-Spielmeyer-Vogt disease61663001
- Cerebral lipidosis myoclonic variant61663001
- Cerebral lipidosis, myoclonic variant61663001
- Juvenile neuronal ceroid lipofuscinosis61663001
- Spielmeyer-Vogt disease61663001
- Spielmeyer-Vogt type neuronal ceroid lipofuscinosis61663001
- Adult neuronal ceroid lipofuscinosis62009002
- Adult-type amaurotic idiocy62009002
- Amaurotic idiocy adult type62009002
- Amaurotic idiocy late familial62009002
- Kufs type neuronal ceroid lipofuscinosis62009002
- Kufs' disease62009002
- Late familial amaurotic idiocy62009002
- Dementia associated with cerebral lipidosis698624003
- Neuronal ceroid lipofuscinosis 8703526007
- Northern epilepsy703526007
- Progressive epilepsy with mental retardation703526007
- Progressive epilepsy-intellectual disability syndrome Finnish type703526007
- Cathepsin D deficient neuronal ceroid lipofuscinosis720830009
- Congenital neuronal ceroid lipofuscinosis720830009
- Neuronal ceroid lipofuscinosis 10720830009
- Neuronal ceroid lipofuscinosis due to cathepsin D deficiency720830009
- Neuronal ceroid lipofuscinosis due to deficiency of cathepsin D720830009
- Acquired ataxia722968003
- ATPase cation transporting 13A2 related juvenile neuronal ceroid lipofuscinosis789657008
- CLN12 disease789657008
- Juvenile parkinsonism, neuronal ceroid lipofuscinosis789657008
- Myoclonic disorder due to neuronal ceroid lipofuscinosis1263506002
- CLN6 transmembrane ER protein-related neuronal ceroid lipofuscinosis type 6A1296784002
- CLN6-related neuronal ceroid lipofuscinosis type 6A1296784002
- Neuronal ceroid lipofuscinosis type 6A1296784002
- vLINCL - variant late infantile neuronal ceroid lipofuscinosis1296784002
UMLS
- Adult Neuronal Ceroid LipofuscinosisC0022797
- Adult neuronal ceroid lipofuscinosisC0022797
- Adult neuronal ceroid lipofuscinosis (disorder)C0022797
- Adult-type amaurotic idiocyC0022797
- Amaurotic Idiocy, Adult TypeC0022797
- Amaurotic idiocy adult typeC0022797
- Amaurotic idiocy late familialC0022797
- Ceroid Lipofuscinosis, Neuronal 4C0022797
- Disease, Kuf'sC0022797
- KUFS DISC0022797
- Kuf DiseaseC0022797
- Kuf's DiseaseC0022797
- Kufs DiseaseC0022797
- Kufs Type Neuronal Ceroid LipofuscinosisC0022797
- Kufs diseaseC0022797
- Kufs type neuronal ceroid lipofuscinosisC0022797
- Kufs' diseaseC0022797
- Late familial amaurotic idiocyC0022797
- Neuronal Ceroid Lipofuscinosis, AdultC0022797
- Neuronal Ceroid Lipofuscinosis, Adult TypeC0022797
- kuf diseaseC0022797
- kuf's diseaseC0022797
- kufs diseaseC0022797
- kufs' diseaseC0022797
- Amaurotic idiocy early juvenile typeC0022340
- Amaurotic idiocy late infantile typeC0022340
- Amaurotic idiocy, early juvenile typeC0022340
- Amaurotic idiocy, late infantile typeC0022340
- Bielschowsky-Jansky diseaseC0022340
- Bielschowsky-Jansky type neuronal ceroid lipofuscinosisC0022340
- Dollinger-Bielschowsky syndromeC0022340
- Dollinger-Bielschowsky type neuronal ceroid lipofuscinosisC0022340
- JANSKY BIELSCHOWSKY DISC0022340
- Jansky Bielschowsky DiseaseC0022340
- Jansky-Bielschowsky DiseaseC0022340
- Jansky-Bielschowsky diseaseC0022340
- LINCLC0022340
- Late infantile neuronal ceroid lipofuscinosisC0022340
- Late-Infantile Neuronal Ceroid LipofuscinosisC0022340
- Late-infantile Batten diseaseC0022340
- Late-infantile neuronal ceroid lipofuscinosisC0022340
- Late-infantile neuronal ceroid lipofuscinosis (disorder)C0022340
- Neuronal Ceroid Lipofuscinosis, Late InfantileC0022340
- Neuronal Ceroid Lipofuscinosis, Late-InfantileC0022340
- Neuronal ceroid lipofuscinosis, late-infantileC0022340
- bielschowsky disease janskyC0022340
- jansky-bielschowsky diseaseC0022340
- Amaurotic idiocy juvenile typeC0751383
- Amaurotic idiocy, juvenile typeC0751383
- BATTEN DISC0751383
- BATTEN DISEASEC0751383
- BATTEN MAYOU DISC0751383
- BATTEN SPIELMEYER VOGT DISC0751383
- Batten DiseaseC0751383
- Batten Disease, JuvenileC0751383
- Batten Diseases, JuvenileC0751383
- Batten Mayou DiseaseC0751383
- Batten Spielmeyer Vogt DiseaseC0751383
- Batten diseaseC0751383
- Batten's diseaseC0751383
- Batten's syndromeC0751383
- Batten-Mayou DiseaseC0751383
- Batten-Mayou diseaseC0751383
- Batten-Mayou syndromeC0751383
- Batten-Spielmeyer-Vogt DiseaseC0751383
- Batten-Spielmeyer-Vogt diseaseC0751383
- CEROID LIPOFUSCINOSIS, NEURONAL, 3C0751383
- CLN3C0751383
- CLN3 Related Neuronal Ceroid LipofuscinosisC0751383
- CLN3 diseaseC0751383
- CLN3-Related Neuronal Ceroid-LipofuscinosesC0751383
- CLN3-Related Neuronal Ceroid-LipofuscinosisC0751383
- CLN3-related neuronal ceroid-lipofuscinosisC0751383
- Cerebral lipidosis myoclonic variantC0751383
- Cerebral lipidosis, myoclonic variantC0751383
- Cerebroretinal Degeneration, JuvenileC0751383
- Cerebroretinal Degenerations, JuvenileC0751383
- Ceroid Lipofuscinosis, Neuronal 3, JuvenileC0751383
- Ceroid Lipofuscinosis, Neuronal, 3C0751383
- Ceroid-Lipofuscinosis, CLN3-Related NeuronalC0751383
- Disease, Juvenile BattenC0751383
- Disease, Spielmeyer-SjogrenC0751383
- Disease, Vogt SpielmeyerC0751383
- Disease, Vogt-SpielmeyerC0751383
- JNCLC0751383
- Juvenile Batten DiseaseC0751383
- Juvenile Batten DiseasesC0751383
- Juvenile Batten diseaseC0751383
- Juvenile Cerebroretinal DegenerationC0751383
- Juvenile Cerebroretinal DegenerationsC0751383
- Juvenile Neuronal Ceroid LipofuscinosisC0751383
- Juvenile cerebroretinal degenerationC0751383
- Juvenile neuronal ceroid lipofuscinosisC0751383
- Juvenile neuronal ceroid lipofuscinosis (disorder)C0751383
- NEURONAL CEROID LIPOFUSCINOSIS, JUVENILEC0751383
- Neuronal Ceroid Lipofuscinosis Juvenile TypeC0751383
- Neuronal Ceroid Lipofuscinosis Type 3C0751383
- Neuronal Ceroid Lipofuscinosis, JuvenileC0751383
- Neuronal Ceroid-Lipofuscinoses, CLN3-RelatedC0751383
- Neuronal Ceroid-Lipofuscinosis, CLN3-RelatedC0751383
- SPIELMEYER VOGT DISC0751383
- SPIELMEYER-SJOGREN DISEASEC0751383
- Spielmeyer Disease, VogtC0751383
- Spielmeyer Sjogren DiseaseC0751383
- Spielmeyer Vogt DiseaseC0751383
- Spielmeyer-Sjogren DiseaseC0751383
- Spielmeyer-Vogt DiseaseC0751383
- Spielmeyer-Vogt diseaseC0751383
- Spielmeyer-Vogt type neuronal ceroid lipofuscinosisC0751383
- VOGT-SPIELMEYER DISEASEC0751383
- Vogt Spielmeyer DiseaseC0751383
- Vogt Spielmeyer diseaseC0751383
- Vogt-Spielmeyer DiseaseC0751383
- Vogt-Spielmeyer diseaseC0751383
- batten diseaseC0751383
- batten's diseaseC0751383
- batten's syndromeC0751383
- battens diseaseC0751383
- spielmeyer-vogt diseaseC0751383
- Cerebromacular degenerationC0027877
- Cerebromacular dystrophyC0027877
- Ceroid Lipofuscinosis, NeuronalC0027877
- Ceroid Storage DiseaseC0027877
- Ceroid Storage DiseasesC0027877
- Ceroid-Lipofuscinosis, NeuronalC0027877
- Disease, Ceroid StorageC0027877
- Disease, Lipofuscin StorageC0027877
- Lipofuscin Storage DiseaseC0027877
- Lipofuscin Storage DiseasesC0027877
- Lipofuscinosis, Neuronal CeroidC0027877
- Neuronal Ceroid LipofuscinosesC0027877
- Neuronal Ceroid LipofuscinosisC0027877
- Neuronal Ceroid-LipofuscinosesC0027877
- Neuronal Ceroid-LipofuscinosisC0027877
- Neuronal ceroid lipofuscinosisC0027877
- Neuronal ceroid lipofuscinosis (disorder)C0027877
- Neuronal ceroid lipofuscinosis NOSC0027877
- Pigmentary retinal lipoid neuronal heredodegenerationC0027877
- Storage Disease, CeroidC0027877
- Storage Disease, LipofuscinC0027877
- ceroid lipofuscinoses neuronalC0027877
- ceroid lipofuscinosisC0027877
- hereditary ceroid lipofuscinosisC0027877
- neuronal ceroid lipofuscinosesC0027877
- neuronal ceroid lipofuscinosisC0027877
- neuronal ceroid-lipofuscinosisC0027877
Clinical Terms
- BATTEN DIS
- Disease, Kuf's
- Neuronal Ceroid Lipofuscinosis, Juvenile
- Spielmeyer Sjogren Disease
- Hagberg-Santavouri type neuronal ceroid lipofuscinosis
- Batten-Spielmeyer-Vogt Disease
- Progressive epilepsy-intellectual disability syndrome Finnish type
- Bielschowsky-Jansky type neuronal ceroid lipofuscinosis
- Hagberg-Santavuori disease
- Kufs' disease
- Neuronal Ceroid Lipofuscinosis, Adult
- CLN6 transmembrane ER protein-related neuronal ceroid lipofuscinosis type 6A
- Juvenile Batten disease
- Cerebroretinal Degeneration, Juvenile
- Cerebroretinal Degenerations, Juvenile
- Ceroid Lipofuscinosis, Neuronal
- Cerebral lipidosis myoclonic variant
- Neuronal ceroid lipofuscinosis 10
- Northern epilepsy
- Neuronal Ceroid-Lipofuscinosis, CLN3-Related
- Kufs disease
- CLN6-related neuronal ceroid lipofuscinosis type 6A
- Lipofuscinosis, Neuronal Ceroid
- Neuronal Ceroid Lipofuscinosis, Adult Type
- Batten Mayou Disease
- Neuronal Ceroid Lipofuscinosis Juvenile Type
- Neuronal Ceroid Lipofuscinoses
- Disease, Lipofuscin Storage
- Cerebral lipidosis, myoclonic variant
- batten's syndrome
- JNCL
- spielmeyer-vogt disease
- Late familial amaurotic idiocy
- Late infantile neuronal ceroid lipofuscinosis
- Batten Disease
- bielschowsky disease jansky
- Neuronal ceroid lipofuscinosis due to cathepsin D deficiency
- Neuronal Ceroid Lipofuscinosis, Late Infantile
- Lipofuscin Storage Disease
- Kuf's Disease
- battens disease
- Late-Infantile Neuronal Ceroid Lipofuscinosis
- neuronal ceroid-lipofuscinosis
- Disease, Vogt-Spielmeyer
- Kufs Type Neuronal Ceroid Lipofuscinosis
- Amaurotic idiocy juvenile type
- Spielmeyer-Vogt type neuronal ceroid lipofuscinosis
- Ceroid-Lipofuscinosis, Neuronal
- Batten-Mayou Disease
- Disease, Ceroid Storage
- CLN3-Related Neuronal Ceroid-Lipofuscinoses
- Ceroid Lipofuscinosis, Neuronal, 3
- Disease, Juvenile Batten
- Adult Neuronal Ceroid Lipofuscinosis
- Neuronal ceroid lipofuscinosis due to deficiency of cathepsin D
- Batten Spielmeyer Vogt Disease
- Juvenile neuronal ceroid lipofuscinosis (disorder)
- Progressive epilepsy with mental retardation
- Haltia-Santavouri type neuronal ceroid lipofuscinosis
- Storage Disease, Lipofuscin
- kuf disease
- Polyunsaturated acid lipidosis
- BATTEN MAYOU DIS
- Juvenile cerebroretinal degeneration
- Batten-Mayou syndrome
- Juvenile Neuronal Ceroid Lipofuscinosis
- LINCL
- Disease, Spielmeyer-Sjogren
- SPIELMEYER VOGT DIS
- Batten's disease
- Ceroid Lipofuscinosis, Neuronal 3, Juvenile
- Amaurotic idiocy early juvenile type
- Ceroid-Lipofuscinosis, CLN3-Related Neuronal
- Batten Disease, Juvenile
- Neuronal ceroid lipofuscinosis, infantile Finnish type
- Cerebromacular degeneration
- Dollinger-Bielschowsky type neuronal ceroid lipofuscinosis
- CLN3-related neuronal ceroid-lipofuscinosis
- JANSKY BIELSCHOWSKY DIS
- Cathepsin D deficient neuronal ceroid lipofuscinosis
- Late-infantile Batten disease
- Jansky Bielschowsky Disease
- Juvenile Cerebroretinal Degenerations
- BATTEN SPIELMEYER VOGT DIS
- Amaurotic idiocy adult type
- Neuronal ceroid lipofuscinosis 8
- Vogt Spielmeyer disease
- Amaurotic Idiocy, Adult Type
- Spielmeyer Vogt Disease
- Congenital neuronal ceroid lipofuscinosis
- Vogt-Spielmeyer Disease
- Juvenile Batten Diseases
- CLN12 disease
- SPIELMEYER-SJOGREN DISEASE
- Acquired ataxia
- jansky-bielschowsky disease
- Santavuori disease
- Amaurotic idiocy, late infantile type
- ceroid lipofuscinosis
- CLN3
- Lipofuscin Storage Diseases
- Spielmeyer Disease, Vogt
- Amaurotic idiocy late infantile type
- CLN3 Related Neuronal Ceroid Lipofuscinosis
- Storage Disease, Ceroid
- hereditary ceroid lipofuscinosis
- Neuronal ceroid lipofuscinosis infantile Finnish type
- Neuronal ceroid lipofuscinosis NOS
- Myoclonic disorder due to neuronal ceroid lipofuscinosis
- Dollinger-Bielschowsky syndrome
- Ceroid Storage Diseases
- Ceroid Lipofuscinosis, Neuronal 4
- Disease, Vogt Spielmeyer
- Ceroid Storage Disease
- Batten Diseases, Juvenile
- Neuronal ceroid lipofuscinosis, late-infantile
- Neuronal ceroid lipofuscinosis (disorder)
- Amaurotic idiocy late familial
- Neuronal Ceroid-Lipofuscinoses, CLN3-Related
- Bielschowsky-Jansky disease
- Amaurotic idiocy, early juvenile type
- Amaurotic idiocy, juvenile type
- Neuronal ceroid lipofuscinosis type 6A
- Late-infantile neuronal ceroid lipofuscinosis (disorder)
- Juvenile parkinsonism, neuronal ceroid lipofuscinosis
- Adult-type amaurotic idiocy
- ceroid lipofuscinoses neuronal
- ATPase cation transporting 13A2 related juvenile neuronal ceroid lipofuscinosis
- Neuronal Ceroid Lipofuscinosis Type 3
- Dementia associated with cerebral lipidosis
- vLINCL - variant late infantile neuronal ceroid lipofuscinosis
- CLN3 disease
- Cerebral lipidosis
- Pigmentary retinal lipoid neuronal heredodegeneration
- Polyunsaturated fatty acid lipidosis
- Neuronal Ceroid-Lipofuscinoses
- Adult neuronal ceroid lipofuscinosis (disorder)
- Cerebral degeneration in childhood
- KUFS DIS
- Cerebromacular dystrophy
- Infantile neuronal ceroid lipofuscinosis
Frequently Asked Questions
What is the ICD-10 code for neuronal ceroid lipofuscinosis?
The ICD-10-CM code for neuronal ceroid lipofuscinosis is E75.4. The full clinical description is "Neuronal ceroid lipofuscinosis". E75.4 is a billable/specific code that can be used on insurance claims and medical billing.
What does ICD-10 code E75.4 mean?
ICD-10-CM code E75.4 represents “Neuronal ceroid lipofuscinosis”. It is classified under Chapter 4: Endocrine, Nutritional and Metabolic Diseases and is a billable/specific code that can be used on a claim.
Is E75.4 a billable code?
Yes, E75.4 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.
What chapter is E75.4 in?
E75.4 is in Chapter 4: Endocrine, Nutritional and Metabolic Diseases (codes E00-E89).
What codes cannot be used with E75.4?
E75.4 has Excludes1 notes indicating codes that cannot be used together with it, including: transitory endocrine and metabolic disorders specific to newborn (P70-P74); androgen insensitivity syndrome (E34.5-); congenital adrenal hyperplasia (E25.0); and 5 more.
What SNOMED CT codes does E75.4 map to?
E75.4 maps to 14 SNOMED CT concepts: 789657008, 722968003, 62009002, 14637005, 61663001, and 9 more. SNOMED CT is a clinical terminology used in electronic health records.
What are the UMLS CUIs for E75.4?
E75.4 is linked to 4 UMLS Concept Unique Identifiers: C0022797, C0022340, C0751383, C0027877. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.
How does E75.4 relate to ICF functioning codes?
ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like neuronal ceroid lipofuscinosis affect a person's functioning — body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.
What is the ICD-11 equivalent of E75.4?
E75.4 maps to the ICD-11 code: 5C56.1 (Neuronal ceroid lipofuscinosis).
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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.