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A Case of Myasthenia Gravis Combined with Mediastinal
Leiomyosarcoma and Stevens-Johnson Syndrome
Dong-Kuck Lee, Young-Mi Kweon
Department of Neurology, School of Medicine, Catholic University of Daegu, Korea We report a case of 36-year-old woman with myasthenia gravis (MG) combined with mediastinal leiomyosarcoma (LMS) and Stevens-Johnson syndrome (SJS). She was admitted to ICU with the symptoms of acute onset headache,diplopia, ptosis, dysphagia, general weakness, and respiratory difficulty for several days. Physical examination revealedtachypnea, decreased breath sounds and dullness to percussion in right lower chest. Neurologic examination showedptosis, diplopia, decreased gag reflexes, and generalized proximal weakness. Laboratory studies revealed increasedserum acetylcholine receptor antibodies and positive Tensilon test. Chest CT showed a huge mass in the right middlemediastium but no evidence of thymic enlargement. Mediastinal LMS was diagnosed by ultrasound-guided needle biop-sy. The myasthenic symptoms were fluctuated in spite og intravenous immunoglobulin, plasmapheresis, and corticos-teroid. During therapy, SJS developed. She died 4 months after the onset of the myasthenic symptoms despite thechemotherapy for LMS.
Key Words: Myasthenia Gravis, Mediastinal Leiomyosarcoma, Stevens-Johnson Syndrome
Address for correspondence
Dong Kuck Lee, M.D.
Department of Neurology, School of Medicine
Catholic University of Daegu
3056-6 Daemyung 4 Dong, Namgu, Daegu, 705-718, Korea
Tel: +82-53-650-4267 Fax: +82-53-654-9786
E-mail : [email protected]
Copyright 2004 by the Korean Society for Clinical Neurophysiology Figure 1. Chest CT shows a huge, well-enhanced mediastinal
Figure 2. Skin biopsy shows dermo-epidermal pustules (
tumor containing multiple nodular calcifications and abundant containing vacuolar degenerated cells, necrotic keratinocytes at ), and perivascular infiltration of lymphocytes in J Korean Society for Clinical Neurophysiology / Volume 6 / May, 2004 Figure 3. Histopathologic study shows bundles of malignant
smooth muscle fibers containing spindle-shaped nuclei (H&E,
100). Immunohistochemical stainings were positive for vimentin and SMA (alpha smooth muscle actin).
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