Morton and Gloria Shulman Movement Disorders Centre and the Edmond J. Safra Program in Parkinson's Disease, Toronto Western Hospital, UHN, Division of Neurology, University of Toronto, Toronto, Ontario, Canada.
Krembil Research Institute, Toronto, Ontario, Canada.
Mov Disord. 2017 Dec;32(12):1667-1676. doi: 10.1002/mds.27207. Epub 2017 Nov 16.
Status dystonicus is a movement disorder emergency that has been a source of controversy in terms of terminology, phenomenology, and management since it was first described in 1982. Here we argue that the current use of the term status dystonicus falls well short of the precision needed for either clinical or academic use. We performed a critical review on this topic, describing possible pathophysiological mechanisms and areas of uncertainties. This review also addresses the problems derived by the extreme clinical heterogeneity of this condition, as the lack of an objective criterion useful for the definition, or the fact that status dystonicus may present not only in the context of a known dystonic syndrome. We propose a new possible definition that includes not only dystonia but also other hyperkinetic movements in the wide range of movement disorders that can be seen during an episode. The new definition keeps the term status dystonicus and highlights the fact that this is a medical emergency based on the impairment of bulbar and/or respiratory function requiring hospital admission as the principal feature. Furthermore, the new definition should not consider as necessary unspecific features as patient's condition at baseline, the distribution of dystonia, occurrence of systemic symptoms such as fever or laboratory findings. We hope that this proposal will stimulate the debate on this subject among our peers, further developing a clinical and pathophysiological understanding of status dystonicus. © 2017 International Parkinson and Movement Disorder Society.
僵住状态是一种运动障碍急症,自 1982 年首次描述以来,其术语、表现和管理一直存在争议。在这里,我们认为当前僵住状态的使用远远不能满足临床或学术使用的精确性要求。我们对这个主题进行了批判性审查,描述了可能的病理生理机制和不确定领域。本综述还讨论了这种情况极端临床异质性带来的问题,因为缺乏有用的定义客观标准,或者僵住状态不仅可能出现在已知的肌张力障碍综合征中。我们提出了一个新的可能定义,该定义不仅包括肌张力障碍,还包括在广泛的运动障碍中出现的其他运动障碍。新定义保留了僵住状态这个术语,并强调了这是一种基于延髓和/或呼吸功能障碍的医疗急症,需要住院治疗作为主要特征。此外,新定义不应将患者基线时的情况、肌张力障碍的分布、全身性症状(如发热或实验室发现)等非特异性特征视为必要条件。我们希望这项提议能激发同行们对此问题的讨论,进一步加深对僵住状态的临床和病理生理学的理解。