Progressive Encephalomyelitis with Rigidity and Myoclonus Resolving after Thymectomy with Subsequent Anasarca: An Autopsy Case

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

doi:10.2169/internalmedicine.1238-18

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引用する場合も本公開記事を引用してください。

Progressive Encephalomyelitis with Rigidity and Myoclonus Resolving after Thymectomy with Subsequent Anasarca: An Autopsy Case

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

著者情報

Kokoro Ozaki
Department of Neurology and Neurological Science, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Takuya Ohkubo
Department of Neurology and Neurological Science, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Tetsuo Yamada
Department of Clinical Laboratory Medicine, Graduate School of Health Care Science, Bunkyo Gakuin University, Japan
Kotaro Yoshioka
Department of Neurology and Neurological Science, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Masahiko Ichijo
Department of Neurology and Neurological Science, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Takamasa Majima
Department of Neurology and Neurological Science, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Shunsuke Kudo
Department of Neurology and Neurological Science, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Takumi Akashi
Department of Pathology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Keiji Honda
Department of Otolaryngology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Eisaku Ito
Department of Pathology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Mayumi Watanabe
Department of Pathology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Masaki Sekine
Department of Pathology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Miwako Hamagaki
Department of Pathology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Yoshinobu Eishi
Department of Pathology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Nobuo Sanjo
Department of Neurology and Neurological Science, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Satoru Ishibashi
Department of Neurology and Neurological Science, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Hidehiro Mizusawa
Department of Neurology and Neurological Science, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
The National Center Hospital, National Center of Neurology and Psychiatry, Japan
Takanori Yokota
Department of Neurology and Neurological Science, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan

キーワード: Progressive encephalomyelitis with rigidity and myoclonus, anti-glycine receptor antibody, anasarca, pleural effusion, edema, systemic fluid retention

ジャーナルオープンアクセス早期公開

論文ID: 1238-18

DOI https://doi.org/10.2169/internalmedicine.1238-18

この記事には本公開記事があります。

The final version of this article is now available: Vol. 57 (2018), No. 23 pp. 3451-3458

詳細

抄録

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.

責任著者(Corresponding author)

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