Globoid Cell Leukodystrophy

  • Reinhard L. Friede


The credit for the recognition of globoid cell leukodystrophy is traditionally given to Krabbe (1916), who reported 2 sets of siblings and one sporadic case, 3 of the 5 with histologic data. Short descriptions of the cerebral lesions were published earlier by Bullard and Southard (1906) and by Beneke (1908). Krabbe considered his cases representative of a new form of diffuse sclerosis and cited Beneke’s case as the only other similar observation. He emphasized that his cases differed from those described by Pelizaeus and Merzbacher by an early onset and relentless progress, from Tay-Sachs’ disease by the absence of nerve cell changes, and from Schilder’s encephalitis periaxialis diffusa by the early onset and familial occurrence. Krabbe described and illustrated the globoid cells and referred to them at one point as epithelial, but he did not emphasize them as a diagnostic feature. The pathognomonic significance of the globoid cells was recognized by Collier and Greenfield (1924) who stressed that the morphology of globoid cells and the absence of cytoplasmic osmophilia are quite unlike the features of the fatty macrophages of Wallerian degeneration; they introduced the term “globoid cell” which later became pathognomic and synonymous with Krabbe’s disease.


White Matter Schwann Cell Adrenal Insufficiency Myelin Sheath Wallerian Degeneration 
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Copyright information

© Springer-Verlag Wien 1975

Authors and Affiliations

  • Reinhard L. Friede
    • 1
    • 2
  1. 1.Case Western Reserve UniversityClevelandUSA
  2. 2.University of ZurichSwitzerland

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