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Acta Neuropathologica

, Volume 18, Issue 2, pp 160–172 | Cite as

Generalisierte zentrale Neurofibromatosen und ihre singulären formes frustes

  • R. Iizuka
  • H. Jacob
Originalarbeiten
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Generalized central neurofibromatosis and its unifocale formes frustes

Summary

The case of a 14-month-old boy afflicted with central neurofibrobromatosis is presented which included the following dysgenetic disorders: glioma of the optic nerve; diffuse cellular and fibrillar gliosis with deficient myelinization; subpial, subendymal and perivascular fibrillar gliosis with hypermyelinization; diffuse cellular and fibrillar gliosis in the cortex mainly of the limbic system and the paleocerebellum; ectopic subarachnoid glia; paraendymal spongioblast nodules and a single blastomatous glial nest. The diffuse dysgenetic gliosis did not appear to be blastomatous, and thus it cannot be compared with diffuse glioblastomatosis. The question of special dysgenetic disturbances of myelinization—with respect both to retarded and to paradox myelinization—is discussed in relation to findings made in recent electron microscopic studies and animal experiments. Bielschowsky's term: glial dysgenesia with blastomatosis, (Gliadysgenesien mit blastomatösem Einschlag) applies to tuberous sclerosis and central neurofibromatosis as well as to a very large group of singular, unifocal lesions for which only relatively rarely genetic connections with the two principal groups, i.e. Reckling-hausen's and Bourneville's disease, can be found. Epileptogenic, tumor-like, “paratumoral”, dysgenetic uncus gliosis and dysgenetic aqueduct stenosis for example, should be included here.

Key-Words

Neurofibromatosis Central Dysgenesia Glial Glioblastosis Diffuse Dysmyelination Glioma Opticum 

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Copyright information

© Springer-Verlag 1971

Authors and Affiliations

  • R. Iizuka
    • 1
  • H. Jacob
    • 1
  1. 1.Universitätsnervenklinik Marburg (Lahn)Marburg

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