Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, United Kingdom.
Mov Disord. 2012 Sep 15;27(11):1360-3. doi: 10.1002/mds.25072. Epub 2012 Jun 14.
The majority of patients with anti-N-methyl-D-aspartate-receptor encephalitis (NMDAE) present a characteristic movement disorder, which consists of complex bilateral stereotyped movements of the arms, with perioral and eye movements, and less frequently involvement of the legs. We have observed striking similarities in the characteristics of the abnormal movements observed in NMDAE and those described in Status Dissociatus, which is characterized by a complete breakdown of state-determining boundaries (wakefulness, REM and NREM sleep) and can result from pathophysiologically diverse disorders (e.g. fatal familial insomnia, delirium tremens, Morvan's syndrome). Here, we suggest that the state of paradoxical responsiveness in which NMDAE patients present these stereotyped movements may be that of Status Dissociatus and discuss the clinical similarities and pathophysiological explanations that support such a suggestion. This hypothesis explains why patients that seem to be unconscious have a movement disorder that is not epileptic and may have management implications, since many patients with NMDAE-related movement disorder are treated with anticonvulsants that may not be indicated.
大多数抗 N-甲基-D-天冬氨酸受体脑炎（NMDAE）患者表现出一种特征性的运动障碍，其由手臂的复杂双侧刻板运动组成，伴有口周和眼部运动，腿部受累较少见。我们观察到 NMDAE 中观察到的异常运动的特征与分离状态（Status Dissociatus）中描述的特征非常相似，后者的特征是状态确定边界（觉醒、REM 和 NREM 睡眠）完全崩溃，并且可能由病理生理学上不同的疾病引起（例如致命性家族性失眠症、震颤谵妄、莫尔万综合征）。在这里，我们建议 NMDAE 患者出现这些刻板运动的矛盾反应状态可能是分离状态，并讨论支持这一假设的临床相似性和病理生理学解释。这一假说解释了为什么似乎无意识的患者会出现非癫痫性运动障碍，并且可能具有管理意义，因为许多 NMDAE 相关运动障碍患者接受的抗惊厥药物可能并不适用。
