Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a subtype of stiff-person syndrome (formerly stiff-man syndrome). It is rare and disabling, and characterised by brainstem symptoms, muscle stiffness, breathing issues and autonomic dysfunction. We describe a 65-year-old man who presented with odynophagia together with tongue and neck swelling, followed by multiple cranial nerve palsies culminating in bilateral vocal cord paralysis with acute stridor. He subsequently developed progressive generalised hypertonia and painful limb spasms. Serum antiglycine receptor antibody was strongly positive, but antiglutamic acid decarboxylase and other antibodies relating to stiff-person syndrome were negative. We diagnosed PERM and gave intravenous corticosteroids and immunoglobulins without benefit; however, following plasma exchange he has made a sustained improvement.

中文翻译：

---

进行性脑脊髓炎伴强直和肌阵挛 (PERM)

伴有强直和肌阵挛的进行性脑脊髓炎（PERM）是僵人综合征（以前的僵人综合征）的一种亚型。它罕见且致残，其特点是脑干症状、肌肉僵硬、呼吸问题和自主神经功能障碍。我们描述了一名 65 岁男性，他出现吞咽痛并伴有舌头和颈部肿胀，随后出现多发性颅神经麻痹，最终导致双侧声带麻痹并伴有急性喘鸣。随后，他出现进行性全身性高张力和疼痛性肢体痉挛。血清抗甘氨酸受体抗体呈强阳性，但抗谷氨酸脱羧酶等与僵人综合征相关的抗体呈阴性。我们诊断出PERM并给予静脉注射皮质类固醇和免疫球蛋白，但没有获益；然而，