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Ceroid Lipofuscinosis; Miscellaneous Lipidoses

  • Reinhard L. Friede

Abstract

This disease, like Tay-Sachs disease, was initially recognized by its fundoscopic manifestations (Batten, 1903). In the first report on its cerebral lesions Spielmeyer (1905) noticed the pigmentary nature of some of the material deposited in the neurons and thought that the disease differed from Tay-Sachs disease in its later onset and longer duration. Vogt (1909), on the other hand, was more impressed with the similarities in the symptomatology and in the neuronal changes of his cases with Tay-Sachs disease and thought that both were fundamentally similar. His interpretation was to prevail for half a century, and neuronal ceroid lipofuscinosis was classified as late infantile, juvenile or adult forms of amaurotic idiocy. The evolution of the unitary concept of amaurotic idiocies and their eventual separation into different disease entities are discussed in the first paragraphs of Chapter 36.

Keywords

Metachromatic Leukodystrophy Batten Disease Acta Neuropath Wolman Disease Curvilinear Body 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

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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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