- 著者
- Kokoro Ozaki Takuya Ohkubo Tetsuo Yamada Kotaro Yoshioka Masahiko Ichijo Takamasa Majima Shunsuke Kudo Takumi Akashi Keiji Honda Eisaku Ito Mayumi Watanabe Masaki Sekine Miwako Hamagaki Yoshinobu Eishi Nobuo Sanjo Satoru Ishibashi Hidehiro Mizusawa Takanori Yokota
- 出版者
- The Japanese Society of Internal Medicine
- 雑誌
- Internal Medicine (ISSN:09182918)
- 巻号頁・発行日
- vol.57, no.23, pp.3451-3458, 2018-12-01 (Released:2018-12-01)
- 参考文献数
- 29
- 被引用文献数
- 1 8
Progressive encephalomyelitis with rigidity and myoclonus (PERM) is an autoimmune disorder involving the brainstem and spinal cord and is sometimes associated with thymoma. We encountered a 75-year-old woman with typical PERM features, glycine receptor antibody, and thymoma. Her neurologic symptoms improved after thymectomy, but she unexpectedly developed anasarca with massive pleural effusions and hypoalbuminemia and finally succumbed to death. The autopsy showed edema and mononuclear infiltration in the pleura but no neuropathological findings typical of PERM. Effective treatment of PERM can reverse the neuropathological signs of encephalomyelitis. The autoimmune nature of anasarca is possible but not proven.