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Polyglucosan bodies in intramuscular motor nerves

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Summary

The presence of polyglucosan bodies was studied in intramuscular motor nerves of 292 muscle biopsies. These biopsies were classified into five diagnostic categories and investigated for the presence of polyglucosan bodies in relation to age and sex. Their presence was nonspecific in patients over 20 years, the only correlation being with ageing. Under 20 years, their presence pointed to the diagnosis of Lafora's disease. In cases in which both a muscle biopsy and a sural nerve biopsy were performed, the former appeared to contain these polyglucosans more frequently.

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References

  1. Anzil AP, Herrlinger H, Blinzinger K, Kronski D (1974) Intraneuritic corpora amylacea: demonstration in orbital cortex of elderly subjects by means of early postmortem brain sampling and electron microscopy. Virchows Arch [A] 364:297–301

    Google Scholar 

  2. Asbury AK, Johnson PC (1978) Pathology of peripheral nerve. Saunders, Philadelphia, pp 256–257

    Google Scholar 

  3. Austin J, Sakai M (1976) Disorders of glycogen and related macromolecules in the nervous system. In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology, vol 27. North-Holland, Amsterdam, pp 169–219

    Google Scholar 

  4. Averback P, Langevin H (1978) Corpora amylacea of the lumbar spinal cord and peripheral nervous system. Arch Neurol 35:95–96

    Google Scholar 

  5. Baumann RJ, Kocoshis SA, Wilson D (1983) Lafora disease: liver histopathology in presymptomatic children. Ann Neurol 14:86–89

    Google Scholar 

  6. Berard-Badier M, Pelissier JF, Gambarelli D, De Barsy T, Roger J, Toga M (1980) The retina in Lafora disease: light and electron microscopy. Albrecht Von Graefes Arch Klin Exp Ophthalmol 212:285–294

    Google Scholar 

  7. Bielschowsky M (1912) Beiträge zur Histopathologie der Ganglienzelle. J Psychol Neurol (Leipzig) 18:513–521

    Google Scholar 

  8. Busard HLSM, Renier WO, Gabreëls FJM, Jaspar HHJ, Van Haelst UJG, Slooff JL (1986) Lafora's disease: comparison of inclusion bodies in skin and in brain. Arch Neurol 43:296–299

    Google Scholar 

  9. Busard HLSM, Renier WO, Gabreëls FJM, Jaspar HHJ, Slooff JL, Janssen AJM, Van Haelst UJG (1987) Lafora disease: a quantitative morphological and biochemical study of the cerebral cortex. Clin Neuropathol 6:1–6

    Google Scholar 

  10. Busard HLSM, Gabreëls-Festen AAWM, Renier WO, Gabreëls FJM, Stadhouders AM (1987) Axilla skin biopsy: a reliable test for the diagnosis of Lafora's disease. Ann Neurol 21:599–601

    Google Scholar 

  11. Carpenter S, Karpati G (1976) Intra-axonal polyglucosan bodies: an unusual lesion of peripheral nerves. Neurology 26:369 [Abstr]

    Google Scholar 

  12. Carpenter S, Karpati G (1981) Sweat gland duct cells in Lafora disease: diagnosis by skin biopsy. Neurology 31:1564–1568

    Google Scholar 

  13. Carpenter S, Karpati G, Andermann F, Jacob JC, Andermann E (1974) Lafora's disease: peroxisomal storage in skeletal muscle. Neurology 24:531–538

    Google Scholar 

  14. Coërs C, Woolf AL (1959) The innervation of muscle: a biopsy study. Blackwell, Oxford, pp 7–11

    Google Scholar 

  15. Coërs C, Telerman-Toppet N, Gérard J-M (1973) Terminal innervation ratio in neuromuscular disease. I. Methods and controls. Arch Neurol 29:210–214

    Google Scholar 

  16. Coleman DL, Gambetti P, Di Mauro S, Blume RE (1974) Muscle in Lafora disease. Arch Neurol 31:396–406

    Google Scholar 

  17. Fukuhara N (1977) Intra-axonal corpora amylacea in the peripheral nerve seen in a healthy woman. J Neurol Sci 34:423–426

    Google Scholar 

  18. Harriman DGF, Millar JHD (1955) Progressive familial myoclonic epilepsy in three families: its clinical features and pathological basis. Brain 78:325–349

    Google Scholar 

  19. Harriman DGF, Taverner D, Woolf AL (1970) Ekbom's syndrome and burning paraesthesiae: a biopsy study by vital staining and electron microscopy of the intramuscular innervation with a note on age changes in motor nerve endings in distal muscles. Brain 93:393–406

    Google Scholar 

  20. Johnson PC (1976) Anterior horn cell eosinophilic cytoplasmic inclusion bodies in sporadic motor neuron disease of adults. J Neuropathol Exp Neurol 35:368 [abstr]

    Google Scholar 

  21. Kobayashi M, Iijima M, Hatano M, Akai K, Yoshikura N (1975) An autopsy case of progressive supranuclear palsy with Lafora-like intraneuronal inclusions. Adv Neurol Sci 19:384–395

    Google Scholar 

  22. Kraus-Ruppert R, Ostertag B, Häfner H (1970) A study of the late form (type Lundborg) of progressive myoclonic epilepsy. J Neurol Sci 11:1–15

    Google Scholar 

  23. Lafora GR (1911) Über das Vorkommen amyloider Körperchen im Innern der Ganglienzellen: zugleich ein Beitrag zum Studium der amyloiden Substanz im Nervensystem. Virchows Arch [A] 205:295–303

    Google Scholar 

  24. Mancardi GL, Schenone A, Tabaton M, Tassinari T, Mainardi P (1985) Polyglucosan bodies in the sural nerve of a diabetic patient with polyneuropathy. Acta Neuropathol (Berl) 66:83–86

    Google Scholar 

  25. McMaster KR, Powers JM, Hennigar GR Jr, Wohltmann HJ, Farr GH (1979) Nervous involvement in type IV glycogenosis. Arch Pathol Lab Med 103:105–111

    Google Scholar 

  26. Nemetschek Th, Volk B, Ule G (1975) Die sogenannten Filamente in Lafora-Körperchen, Corpora amylacea und Bielschowsky-Körperchen: fascioläre Substrukturen in intraplasmatischen Speicherungsprodukten bei cellulären Dysmetabolien. Acta Neuropathol (Berl) 33:79–84

    Google Scholar 

  27. Neville HE, Brooke MH, Austin JH (1974) Studies in myoclonus epilepsy (Lafora body form). IV. Skeletal muscle abnormalities. Arch Neurol 30:466–474

    Google Scholar 

  28. Nishimura RN, Ishak KG, Reddick R, Porter R, James S, Barranger JA (1980) Lafora disease: diagnosis by liver biopsy. Ann Neurol 8:409–415

    Google Scholar 

  29. Okamoto K, Llena JF, Hirano A (1982) A type of adult polyglucosan body disease. Acta Neuropathol (Berl) 58:73–77

    Google Scholar 

  30. Orthner H, Becker PE, Müller D (1973) Recessiv erbliche amyotrophische Lateralsklerose mit “Lafora-Körpern”. Arch Psychiatr Nervenkr 217:387–412

    Google Scholar 

  31. Peress NS, DiMauro S, Roxburgh VA (1979) Adult polysaccharidosis: clinico-pathological, ultrastructural, and biochemical features. Arch Neurol 36:840–845

    Google Scholar 

  32. Petito CK, Hart MN, Porro RS, Earle KM (1973) Ultrastructural studies of olivopontocerebellar atrophy. J Neuropathol Exp Neurol 32:503–522

    Google Scholar 

  33. Powell HC, Ward HW, Garrett RS, Orloff MJ, Lampert PW (1979) Glycogen accumulation in the nerves and kidney of chronically diabetic rats: a quantitative electron microscopic study. J Neuropathol Exp Neurol 38:114–127

    Google Scholar 

  34. Probst A, Sandoz P, Vanoni C, Baumann JU (1980) Intraneuronal polyglucosan storage restricted to the lateral pallidum (Bielschowsky bodies): a Golgi, light and electron microscopic study. Acta Neuropathol (Berl) 51:119–126

    Google Scholar 

  35. Robitaille Y, Carpenter S, Karpati G, DiMauro S (1980) A distinct form of adult polyglucosan body disease with massive involvement of central and peripheral neuronal processes and astrocytes: a report of four cases and a review of the occurrence of polyglucosan bodies in other conditions such as Lafora's disease and normal ageing. Brain 103:315–336

    Google Scholar 

  36. Roger J, Pelissier JF, Bureau M, Dravet C, Revol M, Tinuper P (1983) Le diagnostic précoce de la maladie de Lafora. Rev Neurol (Paris) 139:115–124

    Google Scholar 

  37. Sakai M, Austin J, Witmer F, Trueb L (1969) Studies of corpora amylacea. I. Isolation and preliminary characterization by chemical and histochemical techniques. Arch Neurol 21:526–544

    Google Scholar 

  38. Sakai M, Austin J, Witmer F, Trueb L (1970) Studies in myoclonus epilepsy (Lafora body form). II. Polyglucosans in the systemic deposits of myoclonus epilepsy and in corpora amylacea. Neurology 20:160–176

    Google Scholar 

  39. Schwarz GA, Yanoff M (1965) Lafora's disease: distinct clinico-pathological form of Unverricht's syndrome. Arch Neurol 12:172–188

    Google Scholar 

  40. Seitelberger F, Jacob H, Peiffer J, Colmant HJ (1964) Die Myoklonuskörperkrankheit. Eine angeborene Störung des Kohlenhydratstoffwechsels. Klinisch-pathologische Studie an fünf Fällen. Fortschr Neurol Psychiatr 32:305–345

    Google Scholar 

  41. Stam FC, Wigboldus JM, Bots GThAM (1980) Presenile dementia: a form of Lafora disease. J Am Geriatr Soc 28:237–240

    Google Scholar 

  42. Suzuki, K, David E, Kutschman B (1971) Presenile dementia with “Lafora-like” intraneuronal inclusions. Arch Neurol 25:69–80

    Google Scholar 

  43. Takahashi K, Agari M, Nakamura H (1975) Intra-axonal corpora amylacea in ventral and lateral horns of the spinal cord. Acta Neuropathol (Berl) 31:151–158

    Google Scholar 

  44. Vanderhaeghen JJ, Manil J, Franken L, Cappel R (1967) Deux observations de spasme de torsion accompagné de choréoathétose avec nombreux corps de Lafora dans la partie externe du globus pallidus. Acta Neuropathol (Berl) 9:45–52

    Google Scholar 

  45. Vos AJM, Joosten EMG, Gabreëls-Festen AAWM (1983) Adult polyglucosan body disease: clinical and nerve biopsy findings in two cases. Ann Neurol 13:440–444

    Google Scholar 

  46. Yagishita S, Itoh Y, Nakano T (1977) Corpora amylacea in the peripheral nerve axons. Acta Neuropathol (Berl) 37:73–76

    Google Scholar 

  47. Yokoi S, Aihara Y, Maeda S (1975) The myocardium in Lafora disease. Acta Neuropathol (Berl) 33:343–349

    Google Scholar 

  48. Yoshimura T (1977) Beiträge zu den klinisch-und histopathologischen Untersuchungen über die Fälle der Laforaähnlichen Einschlußkörperchen. Folia Psychiatr Neurol Jpn 31:89–102

    Google Scholar 

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Authors and Affiliations

  1. Institute of Neurology, University Hospital Nijmegen, P.O. Box 9101, NL-6500 HB, Nijmegen, The Netherlands

    R. A. J. A. M. Bernsen, H. L. S. M. Busard, H. J. Ter Laak, F. J. M. Gabreëls, W. O. Renier & E. M. G. Joosten

  2. Department of Statistical Consultation, University Hospital Nijmegen, P.O. Box 9101, NL-6500 HB, Nijmegen, The Netherlands

    A. G. M. Theeuwes

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  1. R. A. J. A. M. Bernsen
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  2. H. L. S. M. Busard
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  3. H. J. Ter Laak
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  4. F. J. M. Gabreëls
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  5. W. O. Renier
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  6. E. M. G. Joosten
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  7. A. G. M. Theeuwes
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Bernsen, R.A.J.A.M., Busard, H.L.S.M., Ter Laak, H.J. et al. Polyglucosan bodies in intramuscular motor nerves. Acta Neuropathol 77, 629–633 (1989). https://doi.org/10.1007/BF00687891

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  • DOI: https://doi.org/10.1007/BF00687891

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