Light and electron microscopic appearance of the inner ear in juvenile ceroid lipofuscinosis (CL).

@article{Elleder1988LightAE,
  title={Light and electron microscopic appearance of the inner ear in juvenile ceroid lipofuscinosis (CL).},
  author={M. Elleder and L. Voldřich and L. {\'U}lehlov{\'a} and S. Dimitt and D. Armstrong},
  journal={Pathology, research and practice},
  year={1988},
  volume={183 3},
  pages={
          301-7
        }
}
Inner ear cells were studied by histology, histochemistry and electron microscopy in one case of juvenile form of ceroid lipofuscinosis (Batten's disease). It was found that despite the clinically normal range of auditory acuity (nonaudiometric evaluation) there was a storage process of moderate degree with intralysosomal deposition of a lipopigment of variable ultrastructure with two patterns predominating, curvilinear and fingerprint. Storage was demonstrable in slightly variable degree in… Expand
Spectral properties and mechanisms that underlie autofluorescent accumulations in Batten disease.
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It is concluded that cellular disturbances outside the lysosome in addition to compromised function of this organelle can result in accumulation of lysOSomal AFSM in NCLs and possibly as a result of cellular aging. Expand
Neurology of the neuronal ceroid-lipofuscinoses: late infantile and juvenile types.
  • R. Boustany
  • Biology, Medicine
  • American journal of medical genetics
  • 1992
My experience with more than 80 cases of the late infantile and juvenile forms of the neuronal ceroid-lipofuscinoses over the last 5 years has led to the following realizations. The 2 variants areExpand
Subcellular, Cellular and Organ Pathology of Fabry Disease
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All these structural changes are presented as a basis of the known functional sequels responsible for the altered biology of the storage lysosomal system and of storage affected cells and which, ultimately, lead to cell death, and organ destruction and failure. Expand
Bibliography on ceroid-lipofuscinoses, II.

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