Skip to main content
SpringerLink
Log in

The perifascicular atrophy factor an aid in the histological diagnosis of polymyositis

  • Original Investigations
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Summary

19 biopsies of polymyositis patients were compared with 19 matched controls. The presence of smaller fibres in the periphery of the fascicles has been analyzed quantitatively using a perifascicular atrophy factor. The thinner fibres are multiplied by a factor from 1–4, considering their significance for the diagnosis of fibre atrophy. The value obtained with this method from centrally located fibres as related to the value from peripherally located ones is called the perifascicular atrophy factor. If this is less than — 300 a myopathy of the group of the polymyositis/dermatomyositis can be assumed. 47% of dermatomyositis biopsies and none of the controls were below this range.

Zusammenfassung

Unter 1430 Muskelbiopsien der Neurologischen Universitätsklinik Bern der Jahre 1962 bis 1974 fanden sich 19 geeignete Muskelbiopsie-Präparate von an Polymyositis/Dermatomyositis erkrankten Patienten. Die in Bouin oder Formalin fixierten, in Paraffin eingebetteten und mit Masson-Trichrom, Van Gieson-Elastica oder Haematoxylin-Eosin gefärbten Präparate wurden nach der Methode des «Größten kleineren Durchmessers» ausgemessen und den in gleicher Weise behandelten Vergleichsbiopsien gegenübergestellt. Die Untersuchung zeigt, daß bei geeigneter quantitativer Analyse bei allen unseren Polymyositis/Dermatomyositis-Fällen die an den Faszikelrändern gelegenen Fasern dünner sind als die im Zentrum gelegenen. Der Unterschied beträgt durchschnittlich 5,641 μ, mit einem Maximum von 19,58μ und einem Minimum von 0.82μ. In drei Fällen beträgt die Differenz der Faserdurchmesser peripher—zentral weniger als 1,7μ (=1 Teilstrich im Meßocular), so daß bei der Routineuntersuchung im Lichtmikroskop dieser Unterschied nicht beachtet werden kann. Die statistische Auswertung zeigt für die Gruppe der Polymyositis/Dermatomyositis-Fälle als Ganzes einen hochsignifikanten Unterschied in bezug auf das Kaliber zwischen peripher

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Adams, R. D.: Pathological reactions of sceletal muscle. In: Disorders of coluntary muscle. 3rd edition (ed. J. N. Walton), pp. 168–333. London: Churchill Livingstone 1974

    Google Scholar 

  2. Adams, R. D., Denny-Brown, D., Pearson, C. M.: Diseases of muscle. 1st ed. New York: Harper 1954

    Google Scholar 

  3. Adams, R. D., Denny-Brown, D., Pearson, C. M.: Diseases of muscle. 2nd ed. New York: Harper 1962

    Google Scholar 

  4. Banker, B. Q.: Dermatomyositis of Childhood. Trans. Amer. Neurol. Assoc.87, 11–15 (1962)

    CAS  Google Scholar 

  5. Banker, B. Q.: Dermatomyositis of Childhood. J. Neuropath. Exp. Neurol.34, 46–75 (1975)

    Article  PubMed  CAS  Google Scholar 

  6. Bethlem, J.: Muscle pathology. Amsterdam: North Holland Publ. Co. 1970

    Google Scholar 

  7. Bohan, A., Peter, J. B.: Polymyositis and Dermatomyositis. New Engl. J. Med.292, 344–347, 403–407 (1975)

    Article  PubMed  CAS  Google Scholar 

  8. Brooke, M. H.: The pathologic interpretation in muscle histochemistry. In: Striated muscle (eds. C. M. Pearson, F. K. Mastofi), International Academy of Pathologic Monograph, No. 12. Basel: Karger 1974

    Google Scholar 

  9. Brooke, M. H., Engel, W. K.: Muscle biopsy as a clinical diagnostic aid. In: Neurologic Diagnostic Techniques (ed. W. S. Fields), pp. 90–146. Springfied (Ill.): Thomas 1966

    Google Scholar 

  10. Brooke, M. H., Engel, W. K.: The histographic analysis of human muscle biopsies with regard to fiber types I. Neurology (Minneap.)19, 221–233 (1969)

    CAS  Google Scholar 

  11. Brooke, M. H., Engel, W. K.: The histographic analysis of human muscle biopsies with regard to fiber types II. Neurology (Minneap.)19, 378–393 (1969)

    CAS  Google Scholar 

  12. Brooke, M. H., Kaplan, H.: Muscle pathology in Rheumatoid Arthritis, Polymyalgia Rheumatica and Polymyositis. Arch. Path.94, 101–118 (1972)

    PubMed  CAS  Google Scholar 

  13. Camp, W.A., Engel, W. K.: Myopathies associated with other diseases: Clinical orthopaedics and related research. Philadelphia39, 19–38 (1965)

    CAS  Google Scholar 

  14. Cancilla, P. A., Verity, A. M.: Histochemical fibres types in clinical disorders of muscle. In: Striated muscle (eds. C. M. Pearson, F. K. Mastofi), International Academy of Pathologic Monograph, No. 12, Basel: Karger 1974

    Google Scholar 

  15. Climie, A. R. W.: Muscle biopsy: Technic and interpretation. Am J. Clin. Path.60, 753–770 (1973)

    PubMed  CAS  Google Scholar 

  16. Denny-Brown, D.: The nature of Polymyositis and related muscular diseases. Trans. Coll. Physicians Phila.28, 12–29 (1060)

    Google Scholar 

  17. Denny-Brown, D.: Degeneration of skeletal muscle. Rev. canad. Biol.21, 507–522 (1962)

    PubMed  CAS  Google Scholar 

  18. Dubowitz, V., Brooke, M. H.: Muscle biopsy. A modern approach. Major problems in Neurology, Vol. 2. Philadelphia: Saunders 1973

    Google Scholar 

  19. Engel, W. K.: The multiplicity of pathologic reactions of human sceletal muscle. In: Proceedings of the Vth int. Congress of Neuropathology, Zürich 1965 (eds. F. Lüthy, A. Bischoff) Int. Congress Series No. 100, pp. 613–624. Amsterdam: Exc. Med. Foundation 1965

    Google Scholar 

  20. Engel, W. K.: Diseases of the neuromuscular junction and muscle. In: Neurochemistry (ed. C. Adams), pp. 622–672. Amsterdam: Elsevier 1965

    Google Scholar 

  21. Engel, W. K.: Muscle biopsy — Uses and Limitations. Postgrad. Med.41, 155–160 (1967)

    PubMed  CAS  Google Scholar 

  22. Engel, W. K.: Muscle biopsies in neuromuscular diseases. Pediat. Clin. N. Amer.14, 963–995 (1967)

    PubMed  CAS  Google Scholar 

  23. Engel, W. K.: Selective and nonselective susceptibility of muscle fibre types. Arch. Neurol. (Chicago)22, 97–117 (1970)

    CAS  Google Scholar 

  24. Engel, W. K.: Classifications of neuromuscular disorders. In: Birth defects. Original article series. Vol. VII, No. 2, pp. 18–37 (1971)

    Google Scholar 

  25. Goebel, H. H.: Morphologie der Myositiden und Muskeldystrophien. Akt. Neurol.4, 259–269 (1974)

    Google Scholar 

  26. Johns, T. R., Crowly, W. J., Miller, J. Q., et al.: The syndrome of Myasthenia and Polymyositis with comments on Therapy. Ann. N.Y. Acad. Sci.183, 64–71 (1971)

    PubMed  CAS  Google Scholar 

  27. Mastaglia, F. L., Walton, J. N.: An ultrastructural study of sceletal muscle in polymyositis. J. Neurol. Sci.12, 473–504 (1971)

    Article  PubMed  CAS  Google Scholar 

  28. Mumenthaler, M.: Valeur de la biopsie pour le diagnostic des maladies neuro-musculaires. Etudes de 335 cas personnels. Rev. neurol. Paris108, 462–469 (1963)

    Google Scholar 

  29. Mumenthaler, M.: Myopathy in neuropatho. In: Proceedings of an International Congress, Milan 1969 (ed. J. N. Walton), Exc. Med. Int. Congress Series No. 199, pp. 585–598

  30. Munsat, T., Piper, D., Cancilla, P.: Inflammatory myopathy with facioscapulohumeral distribution. Neurology (Minneap.)22, 335–347 (1972)

    CAS  Google Scholar 

  31. Munsat, T., Cancilla, P.: Polymyositis without inflammation. Bull. Los Angeles neurol. Soc.39, 113–120 (1974)

    PubMed  CAS  Google Scholar 

  32. Pearson, C. M.: Actualités de pathologie neuromusculaire. In: 2ième Journée int. de Marseille, Oct. 1970 (eds. G. Serratrice, H. Roux), pp. 7–9. Expansion Scientifique Française 1970

  33. Pearson, C. M., Rose, S. A.: Myositis. In: Neuromuscular disorders (eds. R. D. Adams, L. M. Eaton, G. M. Shy), Proceedings of the Association 1958, Chapter 15, pp. 422–478. Baltimore: Williams & Wilkins 1960

    Google Scholar 

  34. Pearson, C. M., Currie, S.: Polymyositis and related disorders. In: Disorders of voluntary muscle (ed. J. N. Walton), 3rd. ed., pp. 614–652. Edinburgh and London: Churchill Livingstone 1974

    Google Scholar 

  35. Vilppula, A.: Muscular disorders in some collagen diseases. Acta med. scand. Supp. 540, 435–438 (1972)

    Google Scholar 

  36. Walton, J. N.: Polymypsitis and related disorders. Presse méd.79, 435–438 (1971)

    PubMed  CAS  Google Scholar 

  37. Walton, J. N.: Polymyositis: New Light on pathogenesis and treatment. Proc. Aust. Assoc. Neurol.9, 1–7 (1973)

    PubMed  CAS  Google Scholar 

  38. Walton, J. N.: Discussion — The aetiology of polymyositis. In: Proceedings of the 2nd Int. Congress on muscle diseases 1971, Perth, Australia (ed. B. A. Kakulas), T.C.S. No. 282, pp. 36–50. Amsterdam: Exc. Med. 1973

    Google Scholar 

  39. Walton, J. N., Adams, R. D.: Polymyositis. Edinburgh: Livingstone 1958

    Google Scholar 

  40. Whitaker, J. N., Engel, W. K.: Vascular deposits of immunoglobulin and complement in idiopathic inflammatory myopathy. New Engl. J. Med.286, 333–338 (1972)

    Article  PubMed  CAS  Google Scholar 

  41. Whitaker, J. N., Engel, W. K.: Mechanism of muscle injury in idiopathic inflammatory myopathy. New Engl. J. Med.289, 107–108 (1973)

    PubMed  CAS  Google Scholar 

  42. Referate der Tagung über Myasthenie und andere neuromuskuläre Erkrankungen. Würzburg, 20–22. 5. 1976. In: Fortschritte der Myologie. In Vorbereitung

Download references

Author information

Authors and Affiliations

  1. Department of Neurology Medical School, Inselspital, University of Berne, CH-3010, Berne, Switzerland

    Hans-Peter Baumli & Marco Mumenthaler

Authors
  1. Hans-Peter Baumli
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Marco Mumenthaler
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Hans-Peter Baumli, Mumenthaler, M. The perifascicular atrophy factor an aid in the histological diagnosis of polymyositis. J Neurol 214, 129–136 (1977). https://doi.org/10.1007/BF02430350

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02430350

Key words

Search

Navigation