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Congenital Myasthenia Gravis: A Case Report With Thymectomy and Electron Microscopic Study of Resected Thymus

Congenital Myasthenia Gravis: A Case Report With Thymectomy and Electron Microscopic Study of... Abstract Thymectomy and subsequent short course of corticotropin in high doses did not change the course of persistent and unusually severe congenital myasthenia gravis in a 5-year-old boy. Grossly, the thymus appeared normal in size and consistency for the patient's age. On histopathologic examination several changes consistent with an autoimmune disorder were demonstrable, including lymphoid hyperplasia and large numbers of thymic plasma cells. Electron microscopic studies showed intralobular vascular changes and the presence of thymic myoid cells. Their possible significance is discussed. References 1. Levin PM: Congenital myasthenia in siblings . Arch Neurol Psychiat 62:745-749, 1949.Crossref 2. Teng P, Osserman K: Studies in myasthenia gravis: Neonatal and juvenile types . J Mt Sinai Hosp NY 23:711-716, 1956. 3. Rothbart HB: Myasthenia gravis in children: Its familial incidence . JAMA 108:715-717, 1937.Crossref 4. Castleman B, Norris EH: The pathology of the thymus in myasthenia gravis: Study of 38 cases . Medicine 28:29-34, 1949.Crossref 5. Van de Velde RL, Brown B: A modified Bodian protargol stain for identification of striated muscle elements . Amer J Clin Path 54:768-772, 1970. 6. Van de Velde RL, Friedman NB: Thymic myoid cells and myasthenia gravis . Amer J Path 59:347-366, 1970. 7. Van de Velde RL, Friedman NB: The thymic "myoidzellen" and myasthenia gravis . JAMA 198:287-290, 1966.Crossref 8. Keynes G: Surgery of the thymus gland: Second (and third) thoughts . Lancet 1:1197-1203, 1954.Crossref 9. Wyllie WG, Burrows NF: Myasthenia gravis in childhood , Arch Dis Child 26:457-461, 1951.Crossref 10. Sutin GJ, Hewitson RP: Myasthenia gravis in a 2-year-old treated by thymectomy . S Afr Med J 40:1002-1007, 1966. 11. Torda C, Wolff HG: Effects of adrenocorticotrophic hormone on neuromuscular function in patients with myasthenia gravis . Proc Soc Exp Biol Med 71:432-436, 1949.Crossref 12. Millikan CH, Eaton LM: Clinical evaluation of ACTH and cortisone in myasthenia gravis . Neurology 1:145-149, 1951.Crossref 13. Freydberg LD: The place of corticotropin in the treatment of myasthenia gravis . Ann Intern Med 52:806-810, 1960.Crossref 14. Von Reis G, Liljistrand A, Matell G: Results with ACTH and spironolactone in severe cases of myasthenia gravis . Acta Neurol Scand 41( (suppl 13) ):463-466, 1965.Crossref 15. Osserman KE, Genkins G: Studies in myasthenia gravis: Short-term massive corticotropin therapy . JAMA 198:699-702, 1966.Crossref 16. Castleman B, Norris EH: The pathology of the thymus in myasthenia gravis: Study of 38 cases . Medicine 28:29-36, 1949.Crossref 17. Strauss AJL, Kemp PG: Myasthenia gravis: Immunological relationship between striated muscle and thymus . Lancet 1:772-778, 1966.Crossref 18. Alpert LI, Papatestas A, Kark A, et al: A histologic reappraisal of the thymus in myasthenia gravis . Arch Path 91:55-59, 1971. 19. Mac Kay IR, De Gail P: Thymic "germinal centers" and plasma cells in systemic lupus erythematosus . Lancet 2:667-671, 1963.Crossref 20. Burnet FM: The Clonal Selection Theory of Acquired Immunity . New York, Cambridge University Press, 1959. 21. Burnet FM: The immunological significance of the thymus: An extension of the clonal selection theory of immunity . Aust Ann Med 11:79-83, 1962. 22. Marshall AHE, White RG: Experimental thymic lesions resembling those of myasthenia gravis . Lancet 1:1030-1035, 1961.Crossref 23. Clark SL Jr: The thymus in mice strain 129/J, studied with the electron microscope . Amer J Anat 112:1-5, 1963.Crossref 24. Kameya T, Watanabe Y: Electron microscopic observations on human thymus and thymoma . Acta Path Jap 15:223-226, 1965. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Congenital Myasthenia Gravis: A Case Report With Thymectomy and Electron Microscopic Study of Resected Thymus

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References (27)

Publisher
American Medical Association
Copyright
Copyright © 1971 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1971.02110040140017
Publisher site
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Abstract

Abstract Thymectomy and subsequent short course of corticotropin in high doses did not change the course of persistent and unusually severe congenital myasthenia gravis in a 5-year-old boy. Grossly, the thymus appeared normal in size and consistency for the patient's age. On histopathologic examination several changes consistent with an autoimmune disorder were demonstrable, including lymphoid hyperplasia and large numbers of thymic plasma cells. Electron microscopic studies showed intralobular vascular changes and the presence of thymic myoid cells. Their possible significance is discussed. References 1. Levin PM: Congenital myasthenia in siblings . Arch Neurol Psychiat 62:745-749, 1949.Crossref 2. Teng P, Osserman K: Studies in myasthenia gravis: Neonatal and juvenile types . J Mt Sinai Hosp NY 23:711-716, 1956. 3. Rothbart HB: Myasthenia gravis in children: Its familial incidence . JAMA 108:715-717, 1937.Crossref 4. Castleman B, Norris EH: The pathology of the thymus in myasthenia gravis: Study of 38 cases . Medicine 28:29-34, 1949.Crossref 5. Van de Velde RL, Brown B: A modified Bodian protargol stain for identification of striated muscle elements . Amer J Clin Path 54:768-772, 1970. 6. Van de Velde RL, Friedman NB: Thymic myoid cells and myasthenia gravis . Amer J Path 59:347-366, 1970. 7. Van de Velde RL, Friedman NB: The thymic "myoidzellen" and myasthenia gravis . JAMA 198:287-290, 1966.Crossref 8. Keynes G: Surgery of the thymus gland: Second (and third) thoughts . Lancet 1:1197-1203, 1954.Crossref 9. Wyllie WG, Burrows NF: Myasthenia gravis in childhood , Arch Dis Child 26:457-461, 1951.Crossref 10. Sutin GJ, Hewitson RP: Myasthenia gravis in a 2-year-old treated by thymectomy . S Afr Med J 40:1002-1007, 1966. 11. Torda C, Wolff HG: Effects of adrenocorticotrophic hormone on neuromuscular function in patients with myasthenia gravis . Proc Soc Exp Biol Med 71:432-436, 1949.Crossref 12. Millikan CH, Eaton LM: Clinical evaluation of ACTH and cortisone in myasthenia gravis . Neurology 1:145-149, 1951.Crossref 13. Freydberg LD: The place of corticotropin in the treatment of myasthenia gravis . Ann Intern Med 52:806-810, 1960.Crossref 14. Von Reis G, Liljistrand A, Matell G: Results with ACTH and spironolactone in severe cases of myasthenia gravis . Acta Neurol Scand 41( (suppl 13) ):463-466, 1965.Crossref 15. Osserman KE, Genkins G: Studies in myasthenia gravis: Short-term massive corticotropin therapy . JAMA 198:699-702, 1966.Crossref 16. Castleman B, Norris EH: The pathology of the thymus in myasthenia gravis: Study of 38 cases . Medicine 28:29-36, 1949.Crossref 17. Strauss AJL, Kemp PG: Myasthenia gravis: Immunological relationship between striated muscle and thymus . Lancet 1:772-778, 1966.Crossref 18. Alpert LI, Papatestas A, Kark A, et al: A histologic reappraisal of the thymus in myasthenia gravis . Arch Path 91:55-59, 1971. 19. Mac Kay IR, De Gail P: Thymic "germinal centers" and plasma cells in systemic lupus erythematosus . Lancet 2:667-671, 1963.Crossref 20. Burnet FM: The Clonal Selection Theory of Acquired Immunity . New York, Cambridge University Press, 1959. 21. Burnet FM: The immunological significance of the thymus: An extension of the clonal selection theory of immunity . Aust Ann Med 11:79-83, 1962. 22. Marshall AHE, White RG: Experimental thymic lesions resembling those of myasthenia gravis . Lancet 1:1030-1035, 1961.Crossref 23. Clark SL Jr: The thymus in mice strain 129/J, studied with the electron microscope . Amer J Anat 112:1-5, 1963.Crossref 24. Kameya T, Watanabe Y: Electron microscopic observations on human thymus and thymoma . Acta Path Jap 15:223-226, 1965.

Journal

American Journal of Diseases of Children – American Medical Association

Published: Oct 1, 1971

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