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

  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},
  volume={183 3},
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.
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
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.


Electron microscopic and histochemical identification of lipofuscin in the human inner ear.
Normal inner ears of 14 individuals ranging from 2 days to 91 years of age were studied with electron microscopic and histochemical techniques for the distribution of lipofuscin and lysosomal enzymes, which revealed large membrane-limited inclusions in the apical cytoplasm of all epithelial cells lining the endolymphatic space. Expand
A histochemical and ultrastructural study of stored material in neuronal ceroid lipofuscinosis
  • M. Elleder
  • Biology, Medicine
  • Virchows Archiv. B, Cell pathology
  • 1978
SummaryA histochemical and ultrastructural study of five cases of neuronal ceroid lipofuscinosis (NCL) revealed the existence of two related lipopigments differing in some tinctorial properties andExpand
Cytosome morphology and distribution of generalized ceroidlipofuscinosis in a twenty-eight month old boy with normal myeloperoxidase activity.
The patient's age, cytosome morphology and distribution and results of peroxidase assay add special interest to this case of generalized ceroidlipofuscinosis, however, none of these features warrants creation of a distinct subtype within this group of disorders. Expand
Ultrastructural studies on the retinal pigment epithelium in the neuronal ceroid-lipofuscinoses
Canine NCL is an established spontaneous model for juvenile human NCL but only statement 1 not statement 2 as mentioned above, is correct for diseased English setters. Expand
Influence of N-acetylhomocysteine thiolactone on cultured retinal cells in canine neuronal lipofuscinosis. An ultrastructural study.
An SH- group donor drug, N-acetylhomocysteine thiolactone (0.2 mg/ml), was added to retinal cultures from an adult dog affected with ceroid lipofuscinosis (NCL), revealing extensive fading out or vacuolation and randomly arranged lamellar or 'fingerprint' membranes. Expand
Modified staining technique for surface preparation of the organ of Corti
The method described proves to be very useful for histological examination of human temporal bones as well and makes possible an immediate evaluation of the hair cells of the human cochlea and direct correlation of the inner ear pathology with audiometric data. Expand
Tay-Sachs diesease in its relation to otolaryngology.
  • L. Boies
  • Medicine
  • Archives of otolaryngology
  • 1963