Diseases

Globoid Cell Leukodystrophy

An autosomal recessive metabolic disorder caused by a deficiency of GALACTOSYLCERAMIDASE leading to intralysosomal accumulation of galactolipids such as GALACTOSYLCERAMIDES and PSYCHOSINE. It is characterized by demyelination associated with large multinucleated globoid cells, predominantly involving the white matter of the central nervous system. The loss of MYELIN disrupts normal conduction of nerve impulses.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

An autosomal recessive metabolic disorder caused by a deficiency of GALACTOSYLCERAMIDASE leading to intralysosomal accumulation of galactolipids such as GALACTOSYLCERAMIDES and PSYCHOSINE. It is characterized by demyelination associated with large multinucleated globoid cells, predominantly involving the white matter of the central nervous system. The loss of MYELIN disrupts normal conduction of nerve impulses.

MeSH Record

Classification

Broader headings

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MeSH Record

See Also

MeSH Record

Synonyms

67 entry terms
  • Diffuse Globoid Body Sclerosis
  • GALC Deficiency
  • Galactocerebrosidase Deficiency
  • Galactosylceramidase Deficiency Disease
  • Galactosylceramide Lipidosis
  • Galactosylceramide beta-Galactosidase Deficiency
  • Galactosylceramide-beta-Galactosidase Deficiency Disease
  • Galactosylcerebrosidase Deficiency
  • Galactosylsphingosine Lipidosis
  • Globoid Body Sclerosis, Diffuse
  • Globoid Cell Leukoencephalopathy
  • Globoid Leukodystrophy
  • Krabbe Disease
  • Krabbe Leukodystrophy
  • Krabbe's Disease
  • Krabbe's Leukodystrophy
  • Leukodystrophy, Globoid Cell
  • Psychosine Lipidosis
  • Cell Leukodystrophies, Globoid
  • Cell Leukodystrophy, Globoid
  • Cell Leukoencephalopathies, Globoid
  • Cell Leukoencephalopathy, Globoid
  • Deficiencies, GALC
  • Deficiencies, Galactocerebrosidase
  • Deficiencies, Galactosylceramide beta-Galactosidase
  • Deficiency Disease, Galactosylceramidase
  • Deficiency Disease, Galactosylceramide-beta-Galactosidase
  • Deficiency Diseases, Galactosylceramidase
  • Deficiency Diseases, Galactosylceramide-beta-Galactosidase
  • Deficiency, GALC
  • Deficiency, Galactocerebrosidase
  • Deficiency, Galactosylceramide beta-Galactosidase
  • Disease, Galactosylceramidase Deficiency
  • Disease, Galactosylceramide-beta-Galactosidase Deficiency
  • Diseases, Galactosylceramidase Deficiency
  • Diseases, Galactosylceramide-beta-Galactosidase Deficiency
  • GALC Deficiencies
  • Galactocerebrosidase Deficiencies
  • Galactosylceramidase Deficiency Diseases
  • Galactosylceramide beta Galactosidase Deficiency
  • Galactosylceramide beta Galactosidase Deficiency Disease
  • Galactosylceramide beta-Galactosidase Deficiencies
  • Galactosylceramide-beta-Galactosidase Deficiency Diseases
  • Globoid Cell Leukodystrophies
  • Globoid Cell Leukoencephalopathies
  • Globoid Leukodystrophies
  • Krabbes Disease
  • Krabbes Leukodystrophy
  • Leukodystrophies, Globoid
  • Leukodystrophies, Globoid Cell
  • Leukodystrophy, Globoid
  • Leukodystrophy, Krabbe
  • Leukodystrophy, Krabbe's
  • Leukoencephalopathies, Globoid Cell
  • Leukoencephalopathy, Globoid Cell
  • beta-Galactosidase Deficiencies, Galactosylceramide
  • beta-Galactosidase Deficiency, Galactosylceramide
  • Classic Globoid Cell Leukodystrophy
  • Early-Onset Globoid Cell Leukodystrophy
  • Infantile Globoid Cell Leukodystrophy
  • Late-Onset Globoid Cell Leukodystrophy
  • Leukodystrophy, Globoid Cell, Classic
  • Leukodystrophy, Globoid Cell, Early-Onset
  • Leukodystrophy, Globoid Cell, Infantile
  • Leukodystrophy, Globoid Cell, Late-Onset
  • Early Onset Globoid Cell Leukodystrophy
  • Late Onset Globoid Cell Leukodystrophy

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

1974(1963)

MeSH Hierarchy

Tree Numbers

  1. C10w.228w.140w.163.100.362w.500w
  2. C10w.228w.140w.163.100.435.825w.590w
  3. C10w.228w.140w.695w.625w.500w
  4. C10w.314w.400w.500w
  5. C16w.320w.565w.189.362w.500w
  6. C16w.320w.565w.189.435.825w.590w
  7. C16w.320w.565w.398w.641w.803w.585w
  8. C16w.320w.565w.595w.554.825w.590w
  9. C18.452w.132.100.362w.500w
  10. C18.452w.132.100.435.825w.590w
  11. C18.452w.584w.563w.641w.803w.585w
  12. C18.452w.648w.189.362w.500w
  13. C18.452w.648w.189.435.825w.590w
  14. C18.452w.648w.398w.641w.803w.585w
  15. C18.452w.648w.595w.554.825w.590w

AMA Style

References

  1. National Library of Medicine. Globoid Cell Leukodystrophy. Medical Subject Headings (MeSH). 2026. Unique ID D007965. http://id.nlm.nih.gov/mesh/2026/D007965
  2. Globoid Cell Leukodystrophy. In: Wikipedia. https://en.wikipedia.org/wiki/Krabbe_disease
  3. Globoid Cell Leukodystrophy. In: Wikidata. https://www.wikidata.org/wiki/Q511372