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. 1972 Jul;11(3):321–332.

Immunological studies in patients with juvenile-onset myasthenia gravis and in their relatives

Sarah Bundey, Deborah Doniach, J F Soothill
PMCID: PMC1553664  PMID: 5042917

Abstract

Fifty-four patients who developed myasthenia before the age of 20 had an increased incidence of autoantibodies similar to that in adult-onset myasthenics, except that there was no significant increase of striated muscle antibodies. The increase in thyroid antibodies was associated with an increase of thyroid disease.

Their 150 relatives showed a significant increase in thyroid antibodies, in thyroid disease, and a non-significant increase in the five other autoantibodies studied. There was aggregation of thyroid antibodies in some families.

Two patients and one relative were deficient for immunoglobulins (one patient and one father for IgA and one patient for IgM).

These familial immunological abnormalities, which are more marked in the families of patients with juvenile-onset myasthenia than in the families of adult-onset patients, point to fundamental genetic immunopathogenesis of the disease.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bachmann R. Studies on the serum gamma-A-globulin level. 3. The frequency of A-gamma-A-globulinemia. Scand J Clin Lab Invest. 1965;17(4):316–320. doi: 10.3109/00365516509077057. [DOI] [PubMed] [Google Scholar]
  2. Blajchman M. A. Tissue antibodies in idiopathic autoimmune haemolytic anaemia. Clin Exp Immunol. 1971 May;8(5):741–748. [PMC free article] [PubMed] [Google Scholar]
  3. Bundey S. A genetic study of infantile and juvenile myasthenia gravis. J Neurol Neurosurg Psychiatry. 1972 Feb;35(1):41–51. doi: 10.1136/jnnp.35.1.41. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Bundey S., Carter C. O., Soothill J. F. Early recognition of heterozygotes for the gene for dystrophia myotonica. J Neurol Neurosurg Psychiatry. 1970 Jun;33(3):279–293. doi: 10.1136/jnnp.33.3.279. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. DONIACH D., NILSSON L. R., ROITT I. M. AUTOIMMUNE THYROIDITIS IN CHILDREN AND ADOLESCENTS. II. IMMUNOLOGICAL CORRELATIONS AND PARENT STUDY. Acta Paediatr Scand. 1965 May;54:260–274. doi: 10.1111/j.1651-2227.1965.tb06369.x. [DOI] [PubMed] [Google Scholar]
  6. Dingle P. R., Ferguson A., Horn D. B., Tubmen J., Hall R. The incidence of thyroglobulin antibodies and thyroid enlargement in a general practice in north-east England. Clin Exp Immunol. 1966 Jul;1(3):277–284. [PMC free article] [PubMed] [Google Scholar]
  7. Downes J. M., Greenwood B. M., Wray S. H. Auto-immune aspects of myasthenia gravis. Q J Med. 1966 Jan;35(137):85–105. [PubMed] [Google Scholar]
  8. FUDENBERG H., SOLOMON A. "Acquired agammaglobulinemia" with auto-immune hemolytic disease: graft-versus-host reaction? Vox Sang. 1961 Jan;6:68–79. doi: 10.1111/j.1423-0410.1961.tb03137.x. [DOI] [PubMed] [Google Scholar]
  9. Goldstein G., Hofmann W. W. Endocrine function of the thymus affecting neuromuscular transmission. Clin Exp Immunol. 1969 Feb;4(2):181–189. [PMC free article] [PubMed] [Google Scholar]
  10. Goldstein G. The thymus and neuromuscular function. A substance in thymus which causes myositis and myasthenic neuromuscular block in guineapigs. Lancet. 1968 Jul 20;2(7560):119–122. doi: 10.1016/s0140-6736(68)90414-5. [DOI] [PubMed] [Google Scholar]
  11. Hokkanen E. Myasthenia gravis. A clinical analysis of the total material from Finland with special reference to endocrinological and neurological disorders. Ann Clin Res. 1969 Sep;1(2):94–108. [PubMed] [Google Scholar]
  12. Jacob A., Clack E. R., Emery A. E. Genetic study of sample of 70 patients with myasthenia gravis. J Med Genet. 1968 Dec;5(4):257–261. doi: 10.1136/jmg.5.4.257. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. KILPATRICK R., MILNE J. S., RUSHBROOKE M., WILSON ES WILSON G. M. A survey of thyroid enlargement in two general practices in Great Britain. Br Med J. 1963 Jan 5;1(5322):29–34. doi: 10.1136/bmj.1.5322.29. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. KORNFELD P. SERUM PROTEINS IN MYASTHENIA GRAVIS. JAMA. 1964 Nov 2;190:463–463. doi: 10.1001/jama.1964.03070180061010. [DOI] [PubMed] [Google Scholar]
  15. Kalden J. R., Williamson W. G., Johnston R. J., Irvine W. J. Studies on experimental autoimmune thymitis in guinea-pigs. Clin Exp Immunol. 1969 Oct;5(4):319–340. [PMC free article] [PubMed] [Google Scholar]
  16. LOWENTHAL A., VAN SANDE M. Nouvelles déterminations des fractions protéiniques dans le sérum de patients atteints d'affections musculaires. Rev Fr Etud Clin Biol. 1956 Sep;1(7):765–771. [PubMed] [Google Scholar]
  17. MILLICHAP J. G., DODGE P. R. Diagnosis and treatment of myasthenia gravis in infancy, childhood, and adolescence: a study of 51 patients. Neurology. 1960 Nov;10:1007–1014. doi: 10.1212/wnl.10.11.1007. [DOI] [PubMed] [Google Scholar]
  18. Mackay I. R., Whittingham S., Goldstein G., Currie T. T., Hare W. S. Myasthenia gravis: clinical, serological and histological studies in relation to thymectomy. Australas Ann Med. 1968 Feb;17(1):1–11. doi: 10.1111/imj.1968.17.1.1. [DOI] [PubMed] [Google Scholar]
  19. Mancini G., Carbonara A. O., Heremans J. F. Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry. 1965 Sep;2(3):235–254. doi: 10.1016/0019-2791(65)90004-2. [DOI] [PubMed] [Google Scholar]
  20. Miller J. F., Mitchell G. F. Thymus and antigen-reactive cells. Transplant Rev. 1969;1:3–42. doi: 10.1111/j.1600-065x.1969.tb00135.x. [DOI] [PubMed] [Google Scholar]
  21. OOSTERHUIS H. J. STUDIES IN MYASTHENIA GRAVIS. 1. A CLINICAL STUDY OF 180 PATIENTS. J Neurol Sci. 1964 Nov-Dec;1(6):512–546. doi: 10.1016/0022-510x(64)90171-6. [DOI] [PubMed] [Google Scholar]
  22. OSSERMAN K. E., TENG P. Studies in myasthenia gravis: neonatal and juvenile types. J Mt Sinai Hosp N Y. 1956 Sep-Oct;23(5):711–727. [PubMed] [Google Scholar]
  23. Osserman K. E., Weiner L. B. Studies in myasthenia gravis: immunofluorescent tagging of muscle striation with antibody from serums of 256 myasthenic patients. Ann N Y Acad Sci. 1965 Jun 30;124(2):730–743. doi: 10.1111/j.1749-6632.1965.tb18998.x. [DOI] [PubMed] [Google Scholar]
  24. Rowland L. P., Osserman E. F., Scharfman W. B., Balsam R. F., Ball S. Myasthenia gravis with a myeloma-type, gamma-G (IgG) immunoglobulin abnormality. Am J Med. 1969 Apr;46(4):599–605. doi: 10.1016/0002-9343(69)90078-3. [DOI] [PubMed] [Google Scholar]
  25. SIMPSON J. A. IMMUNOLOGICAL DISTURBANCES IN MYASTHENIA GRAVIS WITH A REPORT OF HASHIMOTO'S DISEASE DEVELOPING AFTER THYMECTOMY. J Neurol Neurosurg Psychiatry. 1964 Dec;27:485–492. doi: 10.1136/jnnp.27.6.485. [DOI] [PMC free article] [PubMed] [Google Scholar]
  26. Strauss A. J., van der Geld H. W., Kemp P. G., Jr, Exum E. D., Goodman H. C. Immunological concomitants of myasthenia gravis. Ann N Y Acad Sci. 1965 Jun 30;124(2):744–766. doi: 10.1111/j.1749-6632.1965.tb18999.x. [DOI] [PubMed] [Google Scholar]
  27. Velde K T. E., Huber J., van der Slikke L. B. Primary acquired hypogammaglobulinemia, myasthenia, and thymoma. Ann Intern Med. 1966 Sep;65(3):554–559. doi: 10.7326/0003-4819-65-3-554. [DOI] [PubMed] [Google Scholar]
  28. WHITE R. G., MARSHALL A. H. The autoimmune response in myasthenia gravis. Lancet. 1962 Jul 21;2(7247):120–123. doi: 10.1016/s0140-6736(62)90005-3. [DOI] [PubMed] [Google Scholar]
  29. Walker J. G., Doniach D., Doniach I. Mitochondrial antibodies and subclinical liver disease. Q J Med. 1970 Jan;39(153):31–48. [PubMed] [Google Scholar]
  30. Weiner L. B., Osserman K. E. Studies in myasthenia gravis: demonstration of presence of immunofluorescence in serums correlated with clinical findings. Ann N Y Acad Sci. 1966 Jan 26;135(1):644–655. doi: 10.1111/j.1749-6632.1966.tb45511.x. [DOI] [PubMed] [Google Scholar]
  31. Whittingham S., Mackay I. R., Kiss Z. S. An interplay of genetic and environmental factors in familial hepatitis and myasthenia gravis. Gut. 1970 Oct;11(10):811–816. doi: 10.1136/gut.11.10.811. [DOI] [PMC free article] [PubMed] [Google Scholar]
  32. Wright R., Kerr J. H. Autoimmunity in myasthenia gravis: a family study. Clin Exp Immunol. 1967 Jan;2(1):93–101. [PMC free article] [PubMed] [Google Scholar]

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