Summary
The so-called Lafora's bodies were studied by histochemical techniques for mucopolysaccharides and glycoproteins.
The results do not confirm the hypothesis according to which glycogen or acid mucopolysaccharides are present in Lafora's bodies. However, the existence of the following fractions in these inclusions could be ascertained:
-
1.
A carbohydrate moiety, characterized by
-
a)
the presence of periodate reactive vic-glycols adjacent to acidic groups, carboxylic and possibly phosphates;
-
b)
its content in hexoses.
-
a)
-
2.
A proteic moiety which appears to be desintegrated and separated from the carbohydrate fraction by the action of papain. This enzyme indeed alters the alcianophily (at pH 2.5) in the core of complex Lafora's bodies. This result suggests that the enzyme might release strong acidic groups at the junction between the two above-mentioned moieties.
The question still remains whether the Lafora's bodies contain only one complex molecule with various chemical properties or two (or more) different molecules.
Résumé
Des techniques histochimiques modernes des mucopolysaccharides et des glycoproteines ont été appliquées, à l'étude des corps de Lafora, observées dans l'épilepsie-myoclonie.
Les résultats obtenus ne plaident pas en faveur de la présence dans les corps de Lafora de glycogène ou de mucopolysaccharides acides, mais permettent d'établir l'existence au sain de ces inclusions, de molécules complexes comprenant
-
1.
Une fraction sucrée caractérisée d'une part par la présence de groupes vic-glycols oxydables par l'acide periodique et situés à proximité de groupes acides carboxyles et éventuellement phosphates, d'autre part par son contenu en hexoses.
-
2.
Une fraction protéique qui se laisse apparemment digérer et cliver de la fraction sucrée par la papaïne; l'enzyme altère en effet l'alcianophilie à pH 2,5 du centre des corps complexes comme si elle libérait des groupes acides forts situés éventuellement à la jonction des deux fractions sus-décrites.
Ces observations ne permettent pas d'établir s'il existe au sein des corps de Lafora un seul corps complexe aux propriétés chimiques diverses ou deux ou plusieurs composés chimiques différents.
Similar content being viewed by others
Abbreviations
- PAS:
-
Periodic acid Schiff
- PA-paraD:
-
Periodic acid paradiamine
- PA-métaD:
-
Periodic acid métadiamine
- BA:
-
Bleu Alcian
- BT:
-
Bleu de Toluidine
- AF:
-
Aldéhyde Fuchsine
- HID:
-
High Iron Diamines
- Chr 2R:
-
Chromotrope 2R
Références
Allegranza, A., P. Canevini, andG. P. Strada: Progressive familial myoclonus epilepsy of Unverricht-Lundborg. Histochimical and ultrastructural study of the S.C. Lafora's bodies. Proc. Vth Int. Congr. Neuropath. Zürich 1965. Excerpta med. Int. Congr. Series100, 999–1003 (1965a).
—,G. P. Strada eP. Canevini: Dati istochimici in un caso di mioclonoepilessia di Unverricht-Lundborg. Riv. Pat. nerv. ment.86, 386–406 (1965b).
Brimacombe, J. S., andJ. M. Weber: Mucopolysaccharides. Chemical structure distribution and isolation. B. B. A. Library, vol. 6. Amsterdam-London-New York: Elsevier Publ. 1965.
Danielli, J. F.: Cytochemistry. A critical approach. New York: Wiley & Sons 1953.
Edgar, G. W. F.: Progressive myoclonus epilepsy as an inborn error of metabolism comparable to storage disease. Epilepsia4, 120–137 (1963a).
—: L'epilepsie myoclonique progressive comme thésaurismose. Rev. neurol.108, 932–935 (1963b).
French, J. E., andE. P. Benditt: The histochemistry of connective tissue: II. The effect of proteins on the selective staining of mucopolysaccharides by basic dyes. J. Histochem. Cytochem.1, 321–325 (1953).
Gerard, A.: Etudes histochimiques et histophysiologiques des glycoprotéines de la muqueuse gastrique chez le chien. Arch. Biol.79, 1–104 (1968).
Harriman, D. G. F., andJ. D. H. Millar: Progressive familial myoclonic epilepsy in three families. Its clinical features and biological basis. Brain78, 325–349 (1955).
Hoogwinkel, G. J. M., andG. Smits: The specificity of the periodic acid-Schiff technique studied by a quantitative test-tube method. J. Histochem. Cytochem.5, 120–126 (1957).
Janeway, R., J. R. Ravens, L. A. Pearce, D. L. Odor, andK. Suzuki: Progressive myoclonus epilepsy with Lafora inclusion bodies. I. Clinical, genetic, histopathological and biochemical aspects. Arch. Neurol. (Chic.)16, 565–583 (196).
Lafora, G. R.: Über das Vorkommen amyloider Körperchen im Innern der Ganglienzellen; zugleich ein Beitrag zum Studium der amyloiden Substanz im Nervensystem. Virchows Arch. path. Anat.205, 295–303 (1911).
Leblond, C. P., R. E. Glegg, andD. Eidinger: Presence of carbohydrates with free 1-2-glycols groups in site stained by the PAS technique. J. Histochem. Cytochem.5, 445–458 (1957).
Lev, R., andC. Bedell: On the slide paraffin walls for small volume histochemical reactions requiring incubation. Stain Technol.41, 150–151 (1966).
—, andS. Spicer: A histochemical comparison of human epithelial mucins in normal and hypersecretory states including cystic fibrosis. Amer. J. Path.46, 23–47 (1965).
Lillie, R. D.: Histopathological technic and practical histochemistry. 3rd ed. New York: McGraw Hill Co 1965.
Lison, L.: Histochimie et cytochimie animales. Paris: Gauthier Villars 1960.
Meyer, K.: Problems of histochemical identification of carbohydrate rich tissue components. J. Histochem. Cytochem.14, 605–606 (1966).
Mowry, R. W.: The special value of methods that color both acidic and vicinal hydroxyl groups in the histochemical study of mucus, with revised directions for the colloïdal iron stain, the use of alcian blue G8X and their combinations with the periodic acid-Schiff reaction. Ann. N.Y. Acad. Sci.106, 402–423 (1963).
Namba, M.: The microscopic, submicroscopic structure and histochemistry of the inclusion body seen in myoclonic epilepsy. Bull. Yamaguchi med. Sch.11, 103–141 (1964).
Nomoto, M., Y. Narahashi, andM. Murakami: A proteolytic enzyme of streptomyces Grieus: VI. Hydrolysis of protein by streptomyces Griseus protease. J. Biochem.48, 593–599 (1960).
Odor, D. L., R. Janeway, L. A. Pearce, andJ. R. Ravens: Progressive myoclonus epilepsy with Lafora inclusions bodies. II. Studies of ultrastructure. Arch. Neurol. (Chic.)16, 583–595 (1967).
Pearce, L. A., R. Janeway, J. R. Ravens, andD. L. Odor: An ultractructural and biochemical investigation of Lafora's disease. Trans. Amer. Neurol. Ass.90, 101–106 (1965).
Pearse, A. G.: Histochemistry. Theoretical and applied, 2nd ed. London: Churchill 1961.
Quintarelli, G.: Histochemical identification of salivary mucus. Ann. N. Y. Acad. Sci.106, 339–363 (1963).
Roger, J., H. Gastaut, J. Boudouresques, M. Toga, D. Dubois etH. Lob: Epilepsie myoclonic progressive avec corps de Lafora. Etude clinique et polygraphique. Contrôle anatomique ultrastructural. Rev. neurol.116, 197–212 (1967).
——,M. Toga, R. Soulayrol, H. Regis, H. Lob, C. A. Tassinari, D. Dubois, Y. Poinso etE. Mesdjian: Epilepsie myoclonie progressive avec corps de Lafora — (Etude clinique, polygraphique et anatomique d'und cas). Rev. neurol.112, 50–61 (1965).
Schnabel, R., andF. Seitelberger: Histophysical and histochemical examination of myoclonus bodies. II. Symp. Cerebral Lipidosis, Coimbra-Curia 1967.
Schwarz, G. A., andM. Yanoff: Lafora's disease: a distinct clinicopathologic form of Unverricht's syndrome. Arch. Neurol. (Chic.)12, 172–188 (1965).
Seitelberger, F.: New contribution to myoclonus body disease. Proc. Vth. Int. Congr. Neuropath. Zürich 1965. Excerpta med. Int. Congr. Series100, 153–163 (1965).
—,H. Jacob, H. J. Peiffer u.H. J. Colmant: Die Myoklonuskörperkrankheit. Eine angeborene Störung des Kohlenhydratstoffwechsels. Klinisch-pathologische Studie an fünf Fällen. Fortschr. Neurol. Psychiat.32, 305–345 (1964).
Sluga, E., u.L. Stockinger: Zur Ultrastruktur der Myoklonuskörper. Acta neuropath. (Berl.)7, 201–217 (1967).
Spicer, S. S.: Diamine methods for differentiating mucopolysaccharides histochemically. J. Histochem. Cytochem.13, 211–234 (1965).
—,R. G. Horn, andJ. J. Leppi: Histochemistry of connective tissue mucopolysaccharides. In:B. M. Wagner andD. E. Smith (Edit.): The connective tissue. Baltimore: The Williams and Wilkins Co, 1967b.
—, andM. H. Jarrels: Histochemical reaction of anaromatic diamine with acid groups and periodate engendered aldehydes in mucopolysaccharides. J. Histochem. Cytochem.9, 368–379 (1961).
—,J. T. Leppi, andP. J. Stoward: Suggestions for a histochemical terminology of carbohydrate rich tissue components. J. Histochem. Cytochem.13, 599–603 (1965).
—, andD. C. H. Sun: Carbohydrate histochemistry of gastric epithelial secretions in dog. Ann. N. Y. Acad. Sci.140, 762–783 (1967a).
—, andL. Warren: The histochemistry of sialic acid containing mucoproteins. J. Histochem. Cytochem.8, 135–137 (1960).
Toga, M., D. Dubois etJ. Hassoun: Ultrastructure des corps de Lafora. Acta neuropath. (Berl.)10, 132–142 (1968).
Van Heycop ten Ham, M. W.: Lafora's disease. Review of histochemical aspects. Arch. Neurobiol. (Madr.)28, 647–666 (1965).
—, andH. de Jager: Progressive myoclonus epilepsy with Lafora bodies. Clinical pathological features. Epilepsia4, 95–119 (1963).
Van Hoof, F., andM. Hageman-Bal: Progressive familial myoclonic epilepsy with Lafora bodies. Electron microscopic and histochemical study of a cerebral biopsy. Acta neuropath. (Berl.)7, 315–326 (1967).
Wagner, B. M., andS. H. Shapiro: Application of alcian blue as a histochemical method. Lab. Invest.6, 472–477 (1957).
Yanoff, M., andG. A. Schwarz: The retinal pathology of Lafora's of lafora's disease: a form of glycoprotein acid mucopolysaccharide dystrophy. Trans. Amer. Acad. Ophthal. Otolaryng.69, 701–708 (1965).
Yokoi, S., J. Austin, andF. Witmer: Isolation and characterization of Lafora's bodies in two cases of myoclonus epilepsy. J. Neuropath. exp. Neurol.26, 125 (1967).
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Dubois-Dalcq, M. Etude histochimique des corps de Lafora. Acta Neuropathol 12, 205–217 (1969). https://doi.org/10.1007/BF00687645
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00687645