- 著者
- Sakiko Itaya Zen Kobayashi Kokoro Ozaki Nozomu Sato Yoshiyuki Numasawa Kinya Ishikawa Takanori Yokota Hiroshi Matsuda Shuzo Shintani
- 出版者
- The Japanese Society of Internal Medicine
- 雑誌
- Internal Medicine (ISSN:09182918)
- 巻号頁・発行日
- pp.0068-17, (Released:2018-02-09)
- 参考文献数
- 14
- 被引用文献数
- 4
A 58-year-old man consulted our hospital due to a 2-year history of dysarthria and a 1-month history of blepharospasm. In addition to the ataxic dysarthria and blepharospasm, a neurological examination demonstrated slight ataxia of the trunk and lower limbs. Brain MRI demonstrated atrophy of the upper portion of the cerebellar vermis. Gene analysis established a diagnosis of spinocerebellar ataxia type 31 (SCA31). SPECT with the three-dimensional stereotaxic ROI template (3DSRT) software program demonstrated hyperperfusion in the lenticular nucleus and thalamus. Although the association between SCA31 and blepharospasm in our patient remains unclear, we considered that this combination might be more than coincidental.
- 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.