Skip to main content

‘Benign’ myopathies of childhood

  • Chapter
Biopsy Pathology of Muscle

Abstract

The term ‘benign myopathies of childhood’ is used to describe a group of disorders characterized clinically by the ‘floppy infant syndrome’. They are sometimes referred to as congenital myopathies, although in some instances they may not cause symptoms until later in childhood or even until adult life. These disorders usually have a genetic basis, although this may not be apparent. In most patients weakness is mild or moderate, and marked wasting is unusual. These disorders are usually only slowly progressive and, in some, improvement may occur with increasing maturity (Dubowitz, 1978; 1980). There are many causes of the floppy infant syndrome (Table 7.1) but many of these are best reclassified according to their underlying cause, e.g. as meta olic myopathies, leaving a group of ill-understood, relatively benign myopathies of childhood onset characterized principally by the changes found in the muscle biopsy (Table 7.2).

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  • Afifi, A. K., Smith, T. W. and Zellweger, H. (1965), Congenital non-progressive myopathy: central core and nemaline myopathy in one family. Neurology (Minneap.), 15, 371–81.

    Article  CAS  Google Scholar 

  • Bethlem, J. (1980), Myopathies, 2nd edn, Elsevier, Amsterdam.

    Google Scholar 

  • Bethlem, J., Arts, W. F. and Dingemans, K. P. (1978), Common origin of rods, cores, miniature cores and focal loss of cross-striations. Arch. Neurol., 35, 555–66.

    Article  CAS  PubMed  Google Scholar 

  • Brooke, M. H. (1973), Congenital fibre type disproportion. In Clinical Studies in Myology (ed. B. A. Kakulas ), Excerpta Medica, Amsterdam, pp. 147–59.

    Google Scholar 

  • Cavanagh, N. P. C., Lake, B. D. and McMeniman, P. (1979), Congenital fibre type disproportion myopathy: a histological diagnosis with an uncertain clinical outlook. Arch. Dis. Child., 54, 735–43.

    Article  CAS  PubMed  Google Scholar 

  • Dobkin, B. H. and Verity, M. A. (1978), Familial neuromuscular disease with Type 1 fibre hypoplasia, tubular aggregates, cardiomyopathy and myasthenic features. Neurology (NY), 28, 1135–40.

    Article  CAS  Google Scholar 

  • Donner, M. Rapola, J. and Somer, H. (1975), Congenital muscular dystrophy: a clinico-pathological and follow-up study of 15 patients. Neuropédiatrie, 6, 239–58.

    Article  CAS  Google Scholar 

  • Dubowitz, V (1978), Muscle Disorders in Childhood, W. B. Saunders, London.

    Google Scholar 

  • Dubowitz, V. (1980), The Floppy Infant Syndrome, 2nd edn, Heinemann, London.

    Google Scholar 

  • Engel, A. G., Angelini, C. and Gomez, M. R. (1972), Finger print body myopathy. Mayo Clin. Proc., 47, 377–88.

    CAS  PubMed  Google Scholar 

  • Hudgson, P., Gardner-Medwin, D., Fulthorpe, J. L. and Walton, J. N. (1967), Nemaline myopathy. Neurology (Minneap.), 17, 1125–42.

    Article  CAS  Google Scholar 

  • Lake, B. D. and Wilson, T. (1975), Zebra body myopathy: clinical, histochemical and ultrastructural studies. J. Neurol. Sci., 24, 437–46.

    Article  CAS  PubMed  Google Scholar 

  • Lundberg, A. (1979), Dissociated motor development; developmental patterns, clinical characteristics; causal factors and outcome with special reference to late-walking children. Neuropédiatrie, 10, 161–82.

    Article  CAS  Google Scholar 

  • Mastaglia, F. L. and Hudgson, P. (1981), Ultrastructural studies of diseased muscle. In Disorders of Voluntary Muscle, 4th edn (ed. J. N. Walton ), Churchill Livingstone, Edinburgh, pp. 296–356.

    Google Scholar 

  • Neville, H. E. (1978); Ultrastructural changes in diseases of human skeletal muscle. In Handbook of Clinical Neurology, Vol. 40 (eds P. J. Vinken and G. W. Bruyn ), North Holland, Amsterdam, pp. 63–124.

    Google Scholar 

  • Nienhuis, A. W., Coleman, F. R., Brown, W. J. et al. (1967), Nemaline myopathy: a histopathologic and biochemical study. Am. J. Clin. Pathol., 48, 1–13.

    CAS  PubMed  Google Scholar 

  • Paine, R. S. (1963), The future of the floppy infant, a follow up study of 133 patients Dev. Med. Child. Neurol., 5, 115–24.

    Article  CAS  PubMed  Google Scholar 

  • Schmitt, H. P. and Volk, B. (1975), The relationship between target, targetoid and targetoid-core fibres in severe neurogenic muscular atrophy. J. Neurol., 210, 167–81.

    Article  CAS  PubMed  Google Scholar 

  • Shy, G. M. and Magee, K. R. (1956), A new congenital non progressive myopathy. Brain, 79, 610–21.

    Article  CAS  PubMed  Google Scholar 

  • Spiro, A. J., Shy, G. M. and Gonatas, N. K. (1966), Myotubular myopathy. Arch. Neurol., 14, 1–14.

    Article  CAS  PubMed  Google Scholar 

  • Swash, M. and Schwartz, M. S. (1981a), Neuromuscular Diseases: A Practical Approach to Diagnosis and Management, Springer-Verlag, Berlin, Heidelberg, New York.

    Google Scholar 

  • Swash, M. and Schwartz, M. S. (1981b), Familial multicore disease with focal loss of cross striations and ophthalmoplegia. J. Neurol. Sci., 52, 1–10.

    Article  CAS  PubMed  Google Scholar 

  • van Wijngaarden, G. K., Bethlem, J., Dingemans, K. P. et al. (1977), Familial focal loss of cross-striations. J. Neurol., 216, 163–72.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

Copyright information

© 1984 M. Swash and M. S. Schwartz

About this chapter

Cite this chapter

Swash, M., Schwartz, M.S. (1984). ‘Benign’ myopathies of childhood. In: Biopsy Pathology of Muscle. Biopsy Pathology Series. Springer, Boston, MA. https://doi.org/10.1007/978-1-4899-3402-4_7

Download citation

  • DOI: https://doi.org/10.1007/978-1-4899-3402-4_7

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-0-412-24420-9

  • Online ISBN: 978-1-4899-3402-4

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics