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
AUTONOMIC NEUROSCIENCE-BASIC & CLINICAL 237 102910-102910 2022年1月
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.