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

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Article number102910
JournalAutonomic Neuroscience: Basic and Clinical
Volume237
DOIs
Publication statusPublished - 01-2022

All Science Journal Classification (ASJC) codes

  • Endocrine and Autonomic Systems
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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