Abstract:
The locked-in syndrome (LiS) is defined as the loss of most voluntary muscle movements with preserved cognitive abilities due to a ventral pontine lesion. However, some patients may also have severe impairment of consciousness [locked-in plus syndrome (LiPS)]. Here we aimed to explore structural differences between LiS and LiPS patients of vascular aetiology, focusing on lesion patterns and locations to better delineate the clinical spectrum of LiS and LiPS. In this retrospective case series study, we report nine patients (two women), ages 29-74 years (median 50) with LiS and LiPS who were diagnosed between 2007 and 2021. Clinical parameters, MRI findings including the lesioned structures, and a shape feature calculation are presented for every patient. The lesioned structures were determined by a senior neuroradiologist. Two of nine patients had fully retained consciousness (LiS) and seven showed various degrees of impaired consciousness (LiPS). Lesions of LiS patients are round and confined to the pons, whereas lesions of LiPS patients are more elongated and reach neighbouring areas such as the mesencephalon, thalamus or ascending reticular activating system. Lesions involving the mesencephalon and the thalamus are strong indicators of LiPS, whereas for lesions restricted to the pons, the dorsal extension and the associated damage to the ascending reticular activating system are crucial to differentiate LiS from LiPS. Recognizing LiPS using clinical and radiological findings is important as these patients may need different therapies and care and, most importantly, should not be mistaken as unresponsive wakefulness syndrome.
摘要:
锁定综合征(LiS)被定义为由于脑桥腹侧病变而导致的认知能力得到保留的大多数自愿肌肉运动的丧失。然而,一些患者还可能有严重的意识障碍[锁定+综合征(LiPS)].在这里,我们旨在探讨血管病因的LiS和LiPS患者之间的结构差异,重点关注病变模式和位置,以更好地描绘LiS和LiPS的临床谱。在这项回顾性病例系列研究中,我们报告了9名患者(2名女性),年龄29-74岁(中位数50),在2007年至2021年间诊断为LiS和LiPS。临床参数,MRI检查结果包括病变结构,并为每个患者提供形状特征计算。病变结构由高级神经放射学家确定。9例患者中有2例完全保留了意识(LiS),7例表现出不同程度的意识障碍(LiPS)。LiS患者的病变是圆形的,局限于脑桥,而LiPS患者的病变更细长并到达邻近区域,例如中脑,丘脑或上行网状激活系统。累及中脑和丘脑的病变是LiPS的有力指标,而对于局限于脑桥的病变,背侧延伸和对上行网状激活系统的相关损害对于区分LiS和LiPS至关重要。认识到使用临床和放射学发现的LiPS是重要的,因为这些患者可能需要不同的治疗和护理,最重要的是,不应该被误认为是反应迟钝的觉醒综合症。