目的:脑桥中央髓鞘溶解症(CPM)是一种罕见的脱髓鞘疾病,可影响脑桥,并可在初始阶段导致极度残疾,如锁定综合征(LIS)。该研究的目的是描述两名CPM导致初始LIS的患者在12个月期间的演变。
方法:我们回顾性报告了这两名患者的意外临床结果,这些结果与脑部MRI记录的解剖损伤有关,与弥散张量成像和纤维束造影中皮质脊髓束重建相关。在3、6、9和12个月时系统评估以下临床参数:12个关键肌肉的肌肉测试(医学研究理事会),理解度量(框和块测试和普渡大学钉板),和日常生活行为的独立性(功能独立性度量)。
结果:两名患者在症状出现后2至3个月开始逐渐恢复,导致在12个月内几乎完全自主(FIM>110),所有接头段的电机强度大于4/5(MRC>50/60)。在脑MRI和纤维束成像上,CST似乎部分保留在桥水平。
结论:考虑到伦理问题和最初可能发生的限制护理的讨论,在12个月时几乎完全的功能恢复的可能性是重要的。这似乎是可逆的髓鞘损伤与部分保留的神经元相结合的结果。侧支通路的发展或传导阻滞的消退可以解释这种恢复。包括DTI和纤维束成像的MRI可能在运动恢复的预后中起关键作用。
OBJECTIVE: Central pontine myelinolysis (CPM) is a rare demyelinating disease that affects the pons and which can cause extreme disabilities such as locked-in syndrome (LIS) in the initial phase. The aim of the study was to describe the evolution over a 12-month period of two patients with CPM causing an initial LIS.
METHODS: We retrospectively report the unexpected clinical outcome of these two patients in relation with the anatomical damages documented by brain MRI, associated with diffusion tensor imaging and reconstruction of corticospinal tracts in tractography. The following clinical parameters systematically assessed at 3, 6, 9, and 12 months: muscle testing on 12 key muscles (Medical Research Council), prehension metrics (box and block test and purdue pegboard), and independence for acts of daily living (functional independence measure).
RESULTS: Both patients showed a progressive recovery beginning between 2 and 3 months after the onset of symptoms, leading to almost complete autonomy at 12 months (FIM > 110), with motor strength greater than 4/5 in all joint segments (MRC > 50/60). On brain MRI with tractography, CST appeared partially preserved at pons level.
CONCLUSIONS: The possibility of a near-complete functional recovery at 12 months is important to consider given the ethical issues at stake and the discussions about limiting care that may take place initially. It seems to be the consequence of reversible myelin damage combined with partially preserved neurons. Development of collateral pathways or resolution of conduction block may explain this recovery. MRI comprising DTI and tractography could play a key role in the prognosis of motor recovery.