Severe dysautonomia in glycine receptor antibody-positive progressive encephalomyelitis with rigidity and myoclonus (PERM): A case report

Yasuaki Mizutani, Shunsuke Adachi, Shoko Nakano, Kazutaka Hayashi, Atsuhiro Higashi, Kouichi Kikuchi, Toshiki Maeda, Kenichiro Murate, Sayuri Shima, Takahiro Iizuka, Akihiro Ueda, Mizuki Ito, Hirohisa Watanabe

研究成果: ジャーナルへの寄稿学術論文査読

2 被引用数 (Scopus)

抄録

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a severe form of stiff-person spectrum disorder. We report a 59-year-old man who presented with progressive encephalomyelitis causing diplopia, bulbar palsy, severe dysautonomia, followed by stiffness and myoclonic cluster. Laboratory tests showed mild pleocytosis, with markedly elevated plasma levels of norepinephrine, epinephrine, and arginine vasopressin. Glycine-receptor antibodies were identified in both serum and CSF. Despite a poor response to methylprednisolone, immunoglobulins, and plasma exchange, α-blocker stabilized dysautonomia. Dysautonomia is presumed to be due to antibody-mediated disinhibited sympathetic hyperactivity; however, this case suggests that concomitant use of α-blocker with immunotherapy may ameliorate dysautonomia.

本文言語英語
論文番号102910
ジャーナルAutonomic Neuroscience: Basic and Clinical
237
DOI
出版ステータス出版済み - 01-2022

All Science Journal Classification (ASJC) codes

  • 内分泌系および自律システム
  • 臨床神経学
  • 細胞および分子神経科学

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