Diseases

Neuronal Ceroid-Lipofuscinoses

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.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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.

MeSH Record

Classification

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MeSH Record

Synonyms

77 entry terms
  • Ceroid Storage Disease
  • Ceroid-Lipofuscinosis, Neuronal
  • Lipofuscin Storage Disease
  • Lipofuscinosis, Neuronal Ceroid
  • Neuronal Ceroid Lipofuscinosis
  • Neuronal Ceroid-Lipofuscinosis
  • Ceroid Lipofuscinosis, Neuronal
  • Ceroid Storage Diseases
  • Disease, Ceroid Storage
  • Disease, Lipofuscin Storage
  • Lipofuscin Storage Diseases
  • Neuronal Ceroid Lipofuscinoses
  • Storage Disease, Ceroid
  • Storage Disease, Lipofuscin
  • 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
  • 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
  • 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
  • Santavuori-Haltia Disease
  • Spielmeyer-Sjogren Disease
  • Spielmeyer-Vogt Disease
  • Vogt Spielmeyer Disease
  • Vogt-Spielmeyer Disease
  • Batten Disease, Juvenile
  • Batten Diseases, Juvenile
  • Batten Mayou Disease
  • Batten Spielmeyer Vogt Disease
  • CLN3 Related Neuronal Ceroid Lipofuscinosis
  • CLN3-Related Neuronal Ceroid-Lipofuscinoses
  • CLN4As
  • Cerebroretinal Degeneration, Juvenile
  • Cerebroretinal Degenerations, Juvenile
  • Ceroid-Lipofuscinosis, CLN3-Related Neuronal
  • Disease, Juvenile Batten
  • Disease, Kuf's
  • Disease, Spielmeyer-Sjogren
  • Disease, Vogt Spielmeyer
  • Disease, Vogt-Spielmeyer
  • Jansky Bielschowsky Disease
  • Juvenile Batten Diseases
  • Juvenile Cerebroretinal Degenerations
  • Kuf Disease
  • Neuronal Ceroid-Lipofuscinoses, CLN3-Related
  • Neuronal Ceroid-Lipofuscinosis, CLN3-Related
  • Santavuori Haltia Disease
  • Spielmeyer Disease, Vogt
  • Spielmeyer Sjogren Disease
  • Spielmeyer Vogt Disease

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

History Note

2007 (1983)

MeSH Record

Previous Indexing

  • Ceroid (1975-1982)
  • Lipofuscin (1975-1982)
  • Lipoidosis (1966-1982)
  • Pigments (1966-1982)

MeSH Hierarchy

Tree Numbers

MeSH Record

NLM Classification

QU 265.5.L5

AMA Style

References

  1. National Library of Medicine. Neuronal Ceroid-Lipofuscinoses. Medical Subject Headings (MeSH). 2026. Unique ID D009472. http://id.nlm.nih.gov/mesh/2026/D009472
  2. Neuronal Ceroid-Lipofuscinoses. In: Wikipedia. https://en.wikipedia.org/wiki/Neuronal_ceroid_lipofuscinosis
  3. Neuronal Ceroid-Lipofuscinoses. In: Wikidata. https://www.wikidata.org/wiki/Q4358039