Original Investigations

Acta Neuropathologica

, Volume 33, Issue 3, pp 191-200

First online:

Niemann-Pick disease (Crocker's type C)

A histochemical study of the distribution and of qualitative differences of the storage process
  • M. EllederAffiliated with1st Department of Pathology, Faculty of General Medicine, Charles University
  • , A. JirásekAffiliated with1st Department of Pathology, Faculty of General Medicine, Charles University
  • , F. ŠmídAffiliated with1st Department of Pathology, Faculty of General Medicine, Charles University

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Summary

A histochemical study is reported of regional differences of the lipid storage in a case of Niemann-Pick disease (NPD) type C. Besides tissues known to be affected (reticuloendothelium, hepatocytes, nervous system), storage was demonstrated in adrenal cortical spongiocytes, sweat glands, renal glomerular and tubular cells, smooth muscle, excretory tubules of some salivary glands, ependyma and in choroid plexus. In most tissues were stored sphingomyelin, cholesterol and a small amount of a glycosphingolipid. In the endothelium of cerebral and spinal vessels the main stored lipid was a glycosphingolipid. The significance of these regional differences are discussed and their study is recommended as a useful counterpart to the biochemical investigation.

Key words

Niemann-Pick disease Storage distribution Cerebral vascular glycolipid Histochemistry Electron microscopy