Department of Neurology, Fujita Health University School of Medicine, Toyoake, Aichi, Japan.
Department of Neurology, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan.
Auton Neurosci. 2022 Jan;237:102910. doi: 10.1016/j.autneu.2021.102910. Epub 2021 Nov 16.
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.
进行性脑脊髓炎伴僵硬和肌阵挛（PERM）是僵硬人综合征谱的一种严重形式。我们报告了一例 59 岁男性，他因进行性脑脊髓炎导致复视、球麻痹、严重自主神经功能紊乱，随后出现僵硬和肌阵挛发作。实验室检查显示轻度白细胞增多，血浆去甲肾上腺素、肾上腺素和精氨酸加压素水平显著升高。血清和脑脊液中均发现甘氨酸受体抗体。尽管甲泼尼龙、免疫球蛋白和血浆置换反应不佳，但α-阻滞剂稳定了自主神经功能紊乱。自主神经功能紊乱推测是由于抗体介导的去抑制性交感神经亢进所致；然而，本例提示免疫治疗同时使用α-阻滞剂可能改善自主神经功能紊乱。
