Skip to main content
Log in

Pleomorphic intra-neuronal polyglucosan bodies mainly restricted to the pallidium

A case report

  • Short Original Communications
  • Published:
Acta Neuropathologica Aims and scope Submit manuscript

Summary

A rare case with pleomorphic intra-neuronal polyglucosan bodies is reported. A 45-year-old man died of gastric carcinoma with extensive visceral involvement. The autopsy showed numerous intra-neural pleomorphic PAs-positive deposits reminiscent of Bielschowsky bodies in the lateral segments of the pallidum, substantia nigra, and brain stem on both sides. Their histochemical properties were consistent with polyglucosan. The bodies were ultrastructurally composed of filamentous structures and seen in perikarya, axons, and dendrites. Occasionally, spotty electron-dense material was seen in the center of the bodies. The filaments were selectively stained with thiocarbohydrazide method after Thiéry, indicating their chief component as glucose polymers. The bodies are compared with Bielschowsky bodies, Lafora bodies, corpora amylacea, and related bodies, and their nature and significance are briefly discussed.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

References

  • Adler D, Horoupian DS, Towfighi J, Gandolfi A, Suzuki K (1982) Status marmoratus and Bielschowsky bodies. A report of two cases and review of the literature. Acta Neuropathol (Berl) 56:75–77

    Google Scholar 

  • 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 [Pathol Anat] 364:297–301

    Google Scholar 

  • Austin J, Sakai M (1977) 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 

  • Bielschowsky M (1912) Beiträge zur Histopathologie der Ganglionzellen. J Psychol Neurol (Lpz) 18:513–521

    Google Scholar 

  • Leon GA (1974) Bielschowsky bodies: Lafora-like inclusions associated with atrophy of the lateral pallidum. Acta Neuropathol (Berl) 30:183–188

    Google Scholar 

  • McMaster KR, Power JM, Henngigar GR, Wohltmann HJ (1979) Nervous system involvement in type IV glycogenosis. Arch Pathol Lab Med 103:105–111

    Google Scholar 

  • Nemetschek T, Volk B, Ule G (1975) Die sogenannte 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 

  • Palmucci L, Anzil AP, Luh S (1982) Intra-astrocytic glycogen granules and corpora amylacea stain positively for polyglucosans: A cytochemical contribution on the fine structural polymorphism of particulate polysaccarides. Acta Neuropathol (Berl) 57:99–102

    Google Scholar 

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

    Google Scholar 

  • 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 

  • Robitaille Y, Carpenter S, Karpati G, DiMauro S (1980): A distinct from 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 disease and normal aging. Brain 103:315–336

    Google Scholar 

  • Thiéry JP (1967) Mise en évidence des polysaccharides sur coupes fines en microscopie electronique. J Microsc 6:987–1018

    Google Scholar 

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

    Google Scholar 

  • Ule G, Volk B (1975) Torpide verlaufende Degeneration des äußeren Pallidungliedes mit Bielschowsky-Körperchen. Licht- und elektronenmikroskopische Befunde. J Neurol 210:191–198

    Google Scholar 

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

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Yagishita, S., Itoh, Y., Nakano, T. et al. Pleomorphic intra-neuronal polyglucosan bodies mainly restricted to the pallidium. Acta Neuropathol 62, 159–163 (1983). https://doi.org/10.1007/BF00684936

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00684936

Key words

Navigation