MeSH Browser

MeSH Heading: Neuronal Ceroid-Lipofuscinoses
Tree Number(s): C10.574.500.550; C16.320.400.600; C16.320.565.398.641.509; C18.452.584.563.641.509; C18.452.648.398.641.509
Unique ID: D009472
RDF Unique Identifier: http://id.nlm.nih.gov/mesh/D009472
Scope Note: A group of severe neurodegenerative diseases characterized by intracellular accumulation of autofluorescent wax-like lipid materials (CEROID; LIPOFUSCIN) in neurons. There are several subtypes based on mutations of the various genes, time of disease onset, and severity of the neurological defects such as progressive DEMENTIA; SEIZURES; and visual failure.
Entry Term(s): Adult Neuronal Ceroid Lipofuscinosis; Amaurotic Idiocy, Adult Type; Batten Disease; Batten-Mayou Disease; Batten-Spielmeyer-Vogt Disease; CLN3-Related Neuronal Ceroid-Lipofuscinosis; CLN4A; CLN4B; Ceroid Lipofuscinosis, Neuronal 3, Juvenile; Ceroid Lipofuscinosis, Neuronal 4; Ceroid Lipofuscinosis, Neuronal, 3; Ceroid Lipofuscinosis, Neuronal, 4A, Autosomal Recessive; Ceroid Lipofuscinosis, Neuronal, 4B, Autosomal Dominant; Ceroid Lipofuscinosis, Neuronal, Parry Type; Ceroid Storage Disease; Ceroid-Lipofuscinosis, Neuronal; Infantile Neuronal Ceroid Lipofuscinosis; Jansky-Bielschowsky Disease; Juvenile Batten Disease; Juvenile Cerebroretinal Degeneration; Juvenile Neuronal Ceroid Lipofuscinosis; Kuf's Disease; Kufs Disease; Kufs Disease Autosomal Recessive; Kufs Disease, Autosomal Dominant; Kufs Disease, Autosomal Recessive; Kufs Type Neuronal Ceroid Lipofuscinosis; Late-Infantile Neuronal Ceroid Lipofuscinosis; Lipofuscin Storage Disease; Lipofuscinosis, Neuronal Ceroid; Neuronal Ceroid Lipofuscinosis; Neuronal Ceroid Lipofuscinosis Juvenile Type; Neuronal Ceroid Lipofuscinosis, Adult; Neuronal Ceroid Lipofuscinosis, Adult Type; Neuronal Ceroid Lipofuscinosis, Infantile; Neuronal Ceroid Lipofuscinosis, Juvenile; Neuronal Ceroid Lipofuscinosis, Late Infantile; Neuronal Ceroid Lipofuscinosis, Late-Infantile; Neuronal Ceroid-Lipofuscinosis; Santavuori-Haltia Disease; Spielmeyer-Sjogren Disease; Spielmeyer-Vogt Disease; Vogt Spielmeyer Disease; Vogt-Spielmeyer Disease
NLM Classification #: QU 265.5.L5
Previous Indexing: Ceroid (1975-1982); Lipofuscin (1975-1982); Lipoidosis (1966-1982); Pigments (1966-1982)
Public MeSH Note: 2007; see NEURONAL CEROID-LIPOFUSCINOSIS 1983-2006
History Note: 2007 (1983)
Date Established: 1983/01/01
Date of Entry: 1982/04/28
Revision Date: 2021/06/30

Allowable Qualifiers: blood (BL)

cerebrospinal fluid (CF)

chemically induced (CI)

classification (CL)

complications (CO)

diagnosis (DI)

diagnostic imaging (DG)

diet therapy (DH)

drug therapy (DT)

economics (EC)

embryology (EM)

enzymology (EN)

epidemiology (EP)

ethnology (EH)

etiology (ET)

genetics (GE)

history (HI)

immunology (IM)

metabolism (ME)

microbiology (MI)

mortality (MO)

nursing (NU)

parasitology (PS)

pathology (PA)

physiopathology (PP)

prevention & control (PC)

psychology (PX)

radiotherapy (RT)

rehabilitation (RH)

surgery (SU)

therapy (TH)

urine (UR)

veterinary (VE)

virology (VI)

Nervous System Diseases [C10]
- Neurodegenerative Diseases [C10.574]
  - Heredodegenerative Disorders, Nervous System [C10.574.500]

Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Genetic Diseases, Inborn [C16.320]
  - Heredodegenerative Disorders, Nervous System [C16.320.400]

Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Genetic Diseases, Inborn [C16.320]
  - Metabolism, Inborn Errors [C16.320.565]
    - Lipid Metabolism, Inborn Errors [C16.320.565.398]
      - Lipidoses [C16.320.565.398.641]
        
        Cholesterol Ester Storage Disease [C16.320.565.398.641.201]
        
        Neuronal Ceroid-Lipofuscinoses [C16.320.565.398.641.509]
        
        Sjogren-Larsson Syndrome [C16.320.565.398.641.723]
        
        Sphingolipidoses [C16.320.565.398.641.803]

Nutritional and Metabolic Diseases [C18]
- Metabolic Diseases [C18.452]
  - Lipid Metabolism Disorders [C18.452.584]
    - Lipid Metabolism, Inborn Errors [C18.452.584.563]
      - Lipidoses [C18.452.584.563.641]
        
        Cholesterol Ester Storage Disease [C18.452.584.563.641.201]
        
        Neuronal Ceroid-Lipofuscinoses [C18.452.584.563.641.509]
        
        Sjogren-Larsson Syndrome [C18.452.584.563.641.723]
        
        Sphingolipidoses [C18.452.584.563.641.803]

Nutritional and Metabolic Diseases [C18]
- Metabolic Diseases [C18.452]
  - Metabolism, Inborn Errors [C18.452.648]
    - Lipid Metabolism, Inborn Errors [C18.452.648.398]
      - Lipidoses [C18.452.648.398.641]
        
        Cholesterol Ester Storage Disease [C18.452.648.398.641.201]
        
        Neuronal Ceroid-Lipofuscinoses [C18.452.648.398.641.509]
        
        Sjogren-Larsson Syndrome [C18.452.648.398.641.723]
        
        Sphingolipidoses [C18.452.648.398.641.803]

Neuronal Ceroid-Lipofuscinoses Preferred

Concept UI: M0014734
Scope Note: A group of severe neurodegenerative diseases characterized by intracellular accumulation of autofluorescent wax-like lipid materials (CEROID; LIPOFUSCIN) in neurons. There are several subtypes based on mutations of the various genes, time of disease onset, and severity of the neurological defects such as progressive DEMENTIA; SEIZURES; and visual failure.
Terms: Neuronal Ceroid-Lipofuscinoses Preferred Term
Term UI T647813
Date08/03/2005
LexicalTag NON
ThesaurusID NLM (2007); Ceroid-Lipofuscinosis, Neuronal
Term UI T028233
Date04/28/1982
LexicalTag NON
ThesaurusID UNK (19XX); Lipofuscinosis, Neuronal Ceroid
Term UI T028234
Date07/31/1996
LexicalTag NON
ThesaurusID NLM (1998); Neuronal Ceroid Lipofuscinosis
Term UI T858835
Date04/21/2014
LexicalTag NON
ThesaurusID NLM (2015); Ceroid Storage Disease
Term UI T769368
Date04/14/2010
LexicalTag NON
ThesaurusID; Lipofuscin Storage Disease
Term UI T769369
Date04/14/2010
LexicalTag NON
ThesaurusID; Neuronal Ceroid-Lipofuscinosis
Term UI T028235
Date01/01/1999
LexicalTag NON
ThesaurusID NLM (1983)

Late-Infantile Neuronal Ceroid Lipofuscinosis Narrower

Adult Neuronal Ceroid Lipofuscinosis Narrower

Juvenile Neuronal Ceroid Lipofuscinosis Narrower

Infantile Neuronal Ceroid Lipofuscinosis Narrower

Ceroid Lipofuscinosis, Neuronal, 4B, Autosomal Dominant Narrower

Ceroid Lipofuscinosis, Neuronal, 4A, Autosomal Recessive Narrower

Neuronal Ceroid-Lipofuscinoses MeSH Descriptor Data 2024