Van Diest D, Van Goethem J W M, Vercruyssen A, Jadoul C, Cras P
Department of Neurology, AZ Maria Middelares Hospital, St. Niklaas, Belgium.
Clin Neurol Neurosurg. 2007 Jan;109(1):58-62. doi: 10.1016/j.clineuro.2006.01.004. Epub 2006 Feb 17.
We report 62-year-old female patient with coincident posterior reversible encephalopathy syndrome (PRES) and Guillain-Barré syndrome (GBS). The first presentation of PRES was a generalised tonic-clonic seizure. A risk factor for PRES was acute arterial hypertension. The diagnosis of PRES was established by MRI (magnetic resonance imaging) and hypertension was treated with labetalol 800mg daily followed by regression of symptoms of PRES. Two days after the seizure the first motor signs of GBS presented with a weakness in both upper arms. The diagnosis of GBS was finally established 6 days after the seizure by clinical evolution, lumbar puncture and electrophysiological findings. After treatment of GBS with intravenous immunoglobulins (IVIg), antihypertensive therapy could be phased out and finally stopped. The patient was discharged after 25 days without any medication. At that time she was completely recovered from PRES and recovering well from GBS. The acute arterial hypertension, the provoking factor of PRES, was probably caused by an autonomic dysfunction in the context of GBS before motor signs of GBS were present but we speculate also that there are other GBS related factors playing a role in PRES. This hypothesis is based on the relatively high coincidence of these two rare syndromes which appears from a review of the literature. One other possible mechanism can be the influence of cytokines, produced in the context of a GBS, on the permeability of blood brain barrier.
我们报告了一名62岁的女性患者,其同时患有后部可逆性脑病综合征(PRES)和吉兰-巴雷综合征(GBS)。PRES的首次表现为全身性强直阵挛发作。PRES的一个危险因素是急性动脉高血压。PRES的诊断通过磁共振成像(MRI)确定,高血压采用拉贝洛尔每日800mg治疗,随后PRES症状消退。癫痫发作两天后,GBS的首个运动体征出现,表现为双侧上臂无力。癫痫发作6天后,通过临床病情演变、腰椎穿刺和电生理检查结果最终确诊为GBS。在用静脉注射免疫球蛋白(IVIg)治疗GBS后,抗高血压治疗可逐步减少并最终停止。患者在25天后出院,无需任何药物治疗。那时她已从PRES完全康复,GBS也恢复良好。PRES的诱发因素急性动脉高血压,可能是在GBS出现运动体征之前,由GBS背景下的自主神经功能障碍引起的,但我们也推测还有其他与GBS相关的因素在PRES中起作用。这一假设基于对文献回顾中这两种罕见综合征相对较高的并发率。另一种可能的机制是GBS背景下产生的细胞因子对血脑屏障通透性的影响。