54岁西洋流行音乐天后席琳狄翁(Celine Dion)在社群平台上透露,自己罹患发病率百万分之一的“僵硬人症候群”( Stiff person syndrome),被迫取消欧洲演唱行程。Celine Dion has been diagnosed with a neurological disorder that ultimately leaves sufferers as human statues as it progressively locks the body into rigid positions, leaving people unable to walk or talk. This is so sad pic.twitter.com/6oYqaQVS3k
— virgo’s grooviest. (@virgosgrooviest) December 8, 2022
根据台大医院神经部杨智超医师的介绍,僵硬人症候群( Stiff person syndrome)是种罕见的中枢神经系统疾病,主要症状为躯干及近端肢体僵硬、紧缩,尤好发于腹部与下背部的肌肉。
僵硬人症候群多在成年后发病,六至八成的病人,血中及脑脊髓液中会出现抗麸胺酸脱羧基(酶)的抗体(Anti-glutamic acid decarboxylase antibody),这种酵素是合成中枢神经一种抑制性传导物质GABA所需的,若遭受到抗体的破坏,可能导致中枢神经抑制功能的不足,使得肌肉产生过强的活性,继而变得僵硬。