Schnetzer Laura, McCoy Mark, Bergmann Jürgen, Kunz Alexander, Leis Stefan, Trinka Eugen
Department of Neurology, Neurological Intensive Care and Neurorehabilitation, Christian Doppler Medical Centre, Paracelsus Medical University, Ignaz-Harrer-Straße 79, A-5020 Salzburg, Austria.
MRI Research Unit, Neuroscience Institute, Christian Doppler Medical Centre, Paracelsus Medical University, Salzburg, Austria.
Ther Adv Neurol Disord. 2023 Mar 29;16:17562864231160873. doi: 10.1177/17562864231160873. eCollection 2023.
The locked-in syndrome (LiS) is characterized by quadriplegia with preserved vertical eye and eyelid movements and retained cognitive abilities. Subcategorization, aetiologies and the anatomical foundation of LiS are discussed. The damage of different structures in the pons, mesencephalon and thalamus are attributed to symptoms of classical, complete and incomplete LiS and the locked-in plus syndrome, which is characterized by additional impairments of consciousness, making the clinical distinction to other chronic disorders of consciousness at times difficult. Other differential diagnoses are cognitive motor dissociation (CMD) and akinetic mutism. Treatment options are reviewed and an early, interdisciplinary and aggressive approach, including the provision of psychological support and coping strategies is favoured. The establishment of communication is a main goal of rehabilitation. Finally, the quality of life of LiS patients and ethical implications are considered. While patients with LiS report a high quality of life and well-being, medical professionals and caregivers have largely pessimistic perceptions. The negative view on life with LiS must be overthought and the autonomy and dignity of LiS patients prioritized. Knowledge has to be disseminated, diagnostics accelerated and technical support system development promoted. More well-designed research but also more awareness of the needs of LiS patients and their perception as individual persons is needed to enable a life with LiS that is worth living.
闭锁综合征（LiS）的特征是四肢瘫痪，但保留垂直眼球运动和眼睑运动以及认知能力。本文讨论了闭锁综合征的亚分类、病因及解剖学基础。脑桥、中脑和丘脑不同结构的损伤可归因于典型、完全和不完全闭锁综合征以及闭锁加综合征的症状，后者的特征是意识出现额外障碍，这使得有时难以与其他慢性意识障碍进行临床区分。其他鉴别诊断包括认知运动分离（CMD）和运动不能性缄默症。本文回顾了治疗方案，并支持早期、多学科和积极的治疗方法，包括提供心理支持和应对策略。建立沟通是康复的主要目标。最后，本文考虑了闭锁综合征患者的生活质量和伦理问题。虽然闭锁综合征患者报告了较高的生活质量和幸福感，但医学专业人员和护理人员大多持悲观看法。必须重新审视对闭锁综合征患者生活的负面看法，并优先考虑其自主性和尊严。必须传播知识、加快诊断并促进技术支持系统的开发。需要开展更多设计良好的研究，同时提高对闭锁综合征患者需求及其作为个体的认知的认识，以使闭锁综合征患者能够过上有价值的生活。
