Summary
Four cases of S.S.L.E are reported. The electron microscopy findings show: 1. Tubulary inclusion bodies: They appear in three cases out of four, either in neuronal and glial nucleus or in axis cylinders. Their morphological features are similar to the nucleocapsides of myxoviruses and particularly measles-virus. 2. Other different types of nonspecific inclusions: nuclear bodies, cristalline-like rods and fibrillar bundles which may be considered as the result of a nuclear metabolic disorder, osmiophilic particles which may be seen as the result of a cellular intracytoplasmic hyperactivity. The tubules are found whatever the duration of the disease (3 months up to 5 years) may be. This might indicate that there is no autosterilization of the virus in the C.N.S. during the course of S.S.L.E. According to the most recent papers, such a disease might be interpreted as an infection indirectly induced by measles-virus with an unknown immunologic or metabolic mechanism.
Résumé
Les auteurs rapportent 4 observations caractéristiques de LESS: L'analyse ultra-structurale montre: 10 — des inclusions tubulaires intra-nucléaires neuronales ou gliales et intra-axonales de morphologie superposable à celle des nucléocapsides des myxo-virus, de la rougeole en particulier. 20 — des formations non spécifiques: corps nucléaires, faisceaux cristallins et fibrillaires traduisant un trouble métabolique nucléaire, particules osmiophiles liées à une hyperactivité cellulaire. Les inclusions tubulaires virales sont présentes quelque soit la durée de la maladie (3 mois à 5 ans) indiquant l'absence d'autostérilisation virale dans le S.N.C. La LESS apparaît liée au virus rougeoleux mais le mécanisme indirect immunitaire ou métabolique de cette action est inconnu.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
Bibliographie
Bernhard, W., H. L. Febvre etR. Cramer: Mise en évidence au microscope électronique d'un virus dans des cellules infectées in vitro par l'agent du polyome. C. R. Acad. Sci. (Paris)249, 483–485 (1959).
Bouteille, M., C. Fontaine, Cl. Vedrenne etJ. Delarue: Sur un cas d'encéphalite subaiguë à inclusions. Étude anatomo-clinique et ultrastructurale. Rev. neurol.118,454–458 (1965).
—,S. R. Kalifat, andJ. Delarue: Ultrastructural variations of nuclear bodies in human diseases. J. Ultrastruct. Res.19, 474–486 (1967a).
—,Cl. Vedrenne, A. M. Dupuy-Coin etJ. Delarue: Inclusions intranucléaires particulières observées au microscope electronique dans un cas d'encéphalite aiguë nécrosante. Ann. Anat. Path. (Paris)12, 403–410 (1967b).
Cheng Mei Shaw: Electron microscopic observations in subacute sclerosing panencephalitis; a supplementary report. Neurology (Minneap.)18, 144–160 (1968).
Connolly, J. H., I. V. Allen, L. J. Hurwitz, andJ. H. D. Millar: Measles-virus antibody and antigen in subacute sclerosing panencephalitis. Lancet1967 I, 542–544.
Dayan, A. D., J. V. T. Gostling, J. L. Greaves, D. W. Stevens, andM. A. Woodhouse: Evidence of a pseudomyxovirus in the brain in subacute sclerosing leucoencephalitis. Lancet1967 I, 980–981.
De The, G., M. Rivière etW. Bernhard: Examen au microscope électronique de la tumeur VX2 du lapin domestique dérivée du papillome de Shope. Bull. Ass. franç. Cancer47, 570–584 (1960).
Dorie, P., L. Martin, M. Bouteille, G. C. Guazzi etA. Manacorda: Les différentes formes des inclusions nucléaires et cytoplasmiques de la leuco-encéphalite sclérosante subaiguë en microscopie ultrastructurale. Acta neuropath. (Berl.)10, 189–201 (1968).
Freeman, J. M., R. L. Magoffin, E. H. Lennette, andR. M. Herndon: Additional evidence of the relation between subacute inclusion-body encephalitis and measles virus. Lancet1967 II, 129–131.
Gonatas, N. K.: Subacute sclerosing leuco-encephalitis: electron-microscopic and cytochemical observations on a cerebral biopsy. J. Neuropath. exp. Neurol.25, 177–201 (1966).
—,J. Martin, andI. Evangelista: The osmiophilic particles of astrocytes. Viruses, lipid droplets or products of secretion?. J. Neuropath. exp. Neurol.26, 369–376 (1967).
—, andG. M. Shy: Virus like particles in subacute sclerosing encephalitis. Nature (Lond.)208, 1338–1339 (1965).
Granboulan, N., P. Tournier, R. Wicker, andW. Bernhard: An electron microscopic study of the development of SV 40 virus. J. Cell. Biol.17, 423–441 (1963).
Herndon, R. M., andL. J. Rubinstein: Light and electron microscopy observations on the development of viral particles in the inclusions of Dawson's encephalitis (subacute sclerosing panencephalitis). Neurology (Minneap.)18, 8–20 (1968).
Krishnan, A., B. G. Uzman, andE. T. Hedley-White: Nuclear bodies: a component of cell nuclei in hamster tissues and human tumors. J. Ultrastruct. Res.19, 563–572 (1967).
Legg, N. J.: Virus antibodies in subacute sclerosing panencephalitis. Brit. med. J.1967 III, 350–352.
Lhermitte, F.: Les leuco-encéphalites. Paris: Flammarion 1950.
Lowenthal, A.: Agar Gel Electrophoresis in Neurology. Amsterdam: Elsevier Publishing Comp. 1964.
Macken, J., etF. Lhermitte: Étude clinique et anatomique de la leuco-encéphalite sclerosante subaiguë, sa place parmi les encéphalites. Bull. Acad. roy. Méd. Belg.15, 205–244, 263–321 (1958).
Mannweiller, K.: inFreeman et al. (1967).
Périer, O., etJ. J. Vanderhagen: Indications étiologiques apportées par la microscopie électronique dans certaines encéphalites humaines. Rev. neurol.115, 250–254 (1966).
——, andS. Pelc: Subacute sclerosing leuco-encephalitis. Electron microscopic findings in two cases with inclusion bodies. Acta neuropath. (Berl.)8, 362–380 (1967).
Popoff, N., andS. Stewart: The fine structure of nuclear inclusions in the brain of experimental golden hamsters. J. Ultrastruct. Res.23, 347–361 (1968).
Radermecker, J., etJ. Macken: Aspects E.E.G. et cliniques de la LESS. Rev. neurol.85, 341–370 (1951).
Tawara, J. T.: Fine structure of filaments in dog kidney cell cultures infected with measles virus. Virology25, 322–323 (1965).
Tellez-Nagel, I., andD. H. Harter: Subacute sclerosing leuco-encephalitis. I. Clinico-Pathological, electron microscopic and virological studies. J. Neuropath. exp. Neurol.25, 560–581 (1966a).
——: Report structure similar to the tubular filaments described here in nuclear and cytoplasmic inclusions of two cases of SSLE. Science154, 899–901 (1966b).
——,A. B. Johnson, andD. Carver: Virus like particles in subacute sclerosing encephalitis; electron-microscopic, virological and histochemical studies. J. Neuropath. exp. Neurol.26, 121–123 (1967).
Ulrich, J., andM. Kidd: Subacute inclusion body encephalitis. A histological and electron microscopical study. Acta neuropath. (Berl.)6, 359–370 (1966).
Van Bogaert, L.: Une leuco-encephalite sclérosante subaiguë. J. Neurol. Neurosurg. Psychiat.8, 101–120 (1945).
Zeman, W.: LESS et virus de la rougeole. Presse méd.76, 907–908 (1968b).
Zeman, W.: Communication personnelle (1969).
—, andO. Kolar: Reflections on the etiology and pathogenesis of subacute sclerosing panencephalitis. Neurology (Minneap.)18, 1–7 (1968a).
Zu Rhein, G. M., andS. M. Chou: Subacute sclerosing panencephalitis: ultrastructural study of a brain biopsy. Neurology (Minneap.)18, 146–160 (1968).
Author information
Authors and Affiliations
Additional information
Laboratoire d'Anatomie Pathologique de la Faculté de Médecine et Unité de Recherches Neurobiologiques de l'Inserm (Pr. Gastaut)
Rights and permissions
About this article
Cite this article
Toga, M., Dubois, D., Berard, M. et al. Étude ultrastructurale de quatre cas de leuco-encéphalite sclérosante subaiguë. Acta Neuropathol 14, 1–13 (1969). https://doi.org/10.1007/BF00687698
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00687698