[A case of non-thymomatous refractory anti-AChR, Kv1.4 and titin antibodies positive generalized myasthenia gravis successfully treated by extended thymectomy].

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  • Additional Information
    • Source:
      Publisher: Nihon Shinkei Gakkai Country of Publication: Japan NLM ID: 0417466 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1882-0654 (Electronic) Linking ISSN: 0009918X NLM ISO Abbreviation: Rinsho Shinkeigaku Subsets: MEDLINE
    • Publication Information:
      Publication: April 1963-: Tōkyō : Nihon Shinkei Gakkai
      Original Publication: Tōkyō : Nihon Rinshō Shinkei Gakkai, 1960-
    • Subject Terms:
      Myasthenia Gravis*/therapy ; Thymoma* ; Thymus Neoplasms*; Humans ; Female ; Aged ; Connectin ; Thymectomy
    • Abstract:
      A 74-year-old Japanese woman, who had been previously diagnosed as ocular myasthenia gravis (MG), presented to our hospital complaining of dropped head and increased fatiguability while eating. The edrophonium test was positive and decremental response was recorded on repetitive nerve stimulation. Her clinical presentation was compatible with generalized MG, and anti-AChR, Kv1.4 and titin antibodies turned out positive. Contrast enhanced CT scan showed no tumorous lesion such as thymoma. We initiated her treatment with a minimum dose of oral prednisolone. However, her condition got worse even after intravenous immune globulin and experienced myasthenic crisis twice, the former of which led to cardiopulmonary arrest. As she did not respond to traditional treatments, we determined to perform extended thymectomy. The histopathology showed atrophic change but her condition rapidly improved in several days after the operation, and soon she was weaned off the ventilator. Shortly thereafter her symptoms disappeared, followed by the titers of the antibodies above found all markedly decreased. It remains unclear how the atrophic thymus acted on the pathogenesis of refractory generalized MG.
    • Contributed Indexing:
      Keywords: anti-striational antibodies; myasthenia gravis; thymoma
    • Accession Number:
      0 (Connectin)
    • Publication Date:
      Date Created: 20230201 Date Completed: 20230228 Latest Revision: 20230228
    • Publication Date:
      20230228
    • Accession Number:
      10.5692/clinicalneurol.cn-001813
    • Accession Number:
      36725010