van der Meché F G, Meulstee J, Vermeulen M, Kievit A
Department of Neurology, University Hospital Dijkzigt, Rotterdam, The Netherlands.
Brain. 1988 Apr;111 ( Pt 2):405-16. doi: 10.1093/brain/111.2.405.
Within the limits of the Guillain-Barré syndrome, the pattern of clinical deficit is variable. Motor deficit may begin proximally or distally in the extremities and sensory deficit is sometimes, but not always present. Longitudinal studies were performed in 13 patients, starting during the progressive phase. The severity of the clinical deficit was related to the amplitude of the compound muscle action potentials (CMAPs) and sensory nerve action potentials (SNAPs). Two patterns were detected, but could only be reliably distinguished during the progressive phase of the disease. (1) A length-dependent reduction of the CMAP, i.e., a progressive decrease of the CMAP on moving the stimulating electrode to more proximal stimulation sites. This could often be attributed to conduction block. In this pattern (Group A), the sensory potentials were spared with only 1 exception. These patients had motor involvement without sensory deficit and the myotatic reflexes could be preserved up to MRC grade 3 paresis. (2) A simple reduction of the CMAP, i.e., the amplitude decreased during clinical deterioration, but during a single investigation remained similar for stimulation at all levels of the nerve. In this pattern (Group B), motor and sensory fibres were similarly involved. These patients showed both motor and sensory deficit and early myotatic areflexia. The second pattern might be explained by an immunological mechanism with a predilection for those regions of the peripheral nervous system where the blood-nerve barrier is naturally deficient. In the first pattern the discrepancy between the involvement of motor and sensory fibres raises new questions, the solution of which might be of importance for the understanding of the Guillain-Barré syndrome.
在吉兰 - 巴雷综合征的范围内,临床缺损模式是可变的。运动缺损可始于四肢的近端或远端,感觉缺损有时存在,但并非总是存在。对13例患者进行了纵向研究,从疾病进展期开始。临床缺损的严重程度与复合肌肉动作电位(CMAP)和感觉神经动作电位(SNAP)的波幅有关。检测到两种模式,但仅在疾病进展期才能可靠区分。(1)CMAP的长度依赖性降低,即随着刺激电极向更靠近近端的刺激部位移动,CMAP逐渐降低。这通常可归因于传导阻滞。在这种模式(A组)中,感觉电位不受影响,仅1例例外。这些患者有运动受累但无感觉缺损,肌伸张反射可保留至MRC 3级轻瘫。(2)CMAP的单纯降低,即在临床恶化期间波幅降低,但在单次检查中,神经各水平刺激时波幅保持相似。在这种模式(B组)中,运动和感觉纤维同样受累。这些患者表现出运动和感觉缺损以及早期肌伸张反射消失。第二种模式可能由一种免疫机制解释,这种机制对周围神经系统中血 - 神经屏障天然缺乏的区域有偏好。在第一种模式中,运动和感觉纤维受累情况的差异提出了新问题,解决这些问题可能对理解吉兰 - 巴雷综合征具有重要意义。