Rigid Spine Syndrome: Histological Examinations of Male and Female Cases

  • J. M. Mussini
  • F. Gray
  • J. J. Hauw
  • A. M. Piette
  • A. Prost
Part of the Acta Neuropathologica Supplementum book series (NEUROPATHOLOGIC, volume 7)

Summary

Histological findings in the limb-muscle of two new cases of Rigid Spine Syndrome are described. The first male case exhibited a disproportion in fiber type with predominance of the type II fibers with a slight increase of large fibers. Some of the type I fibers were slightly atrophic. No other consistent abnormalities could be seen, excepted thickness of vessel basal lamina. The second female case had a relatively distinct muscular pattern. Necrotizing phenomenon were mild without fibrosis. Type I predominance were obvious. Atrophic and hypertrophic fibers could be seen in both types populations, with a relatively higher rate in type II. These two additional cases and those of the literature showed varied non specific histology, without any histo-chemical nor ultrastructural characteristic pattern.

Key words

rigid spine syndrome histology muscle fibers myopathy 

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References

  1. 1.
    Dubowitz V (1965): “Pseudo” muscular dysstrophy. In: Research in muscular dystrophy (Proc. 3rd Symp. Research Committee of the Muscular Dystrophy Group of Great Britain). Lippincott, London, pp 57–73Google Scholar
  2. 2.
    Dubowitz V, Brooke MH (1973) Muscular Biopsy: a modern approach. In: Major problems in Neurology, vol. 2. Saunders, London, pp 368–370Google Scholar
  3. 3.
    Goebel HH, Lenard HG, Gorke W, Kunze K (1977) Fiber type disproportion in the Rigid Spine syndrome. Neuropädiatrie 8:467–477PubMedCrossRefGoogle Scholar
  4. 4.
    Prost A, Mussini J-M, Audran M, Brainvel J-V, Mathe J-F, Cottin S (1980) Syndrome de la colonne raide. Rev Rhum Mal Osteoartic 47: 49–52PubMedGoogle Scholar
  5. 5.
    Rotthauwe HW, Mortier W, Beyer H (1972) Neuer Typ einer recessiv X-Chromosomal vererbten Muskeldystrophie: scapulo-hu-mero-distale Muskeldystrophie mit frühzeitigen Kontrakturen und Herzrhythmusstörungen. Hum Genet 16:181–200CrossRefGoogle Scholar
  6. 6.
    Seay AR, Ziter FA, Petajan JH (1977) Rigid Spine syndrome. A type I myopathy. Arch Neurol 34:119–122PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 1981

Authors and Affiliations

  • J. M. Mussini
    • 1
    • 4
  • F. Gray
    • 2
  • J. J. Hauw
    • 2
  • A. M. Piette
    • 3
  • A. Prost
    • 3
  1. 1.Clinique NeurologiqueC.H.U. NantesFrance
  2. 2.Laboratoire Charles Foix-La SalpétrièreParisFrance
  3. 3.RhumatologieC.H.U. NantesFrance
  4. 4.Clinique Neurologique (Pr Feve)Hotel-Dieu C. H. U.Nantes CedexFrance

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