Mao Zhifeng, Hu Xueqiang
Neurol Res. 2014 Dec;36(12):1106-13. doi: 10.1179/1743132814Y.0000000400. Epub 2014 Jun 10.
Some patients with Guillain-Barré syndrome (GBS) also have acquired demyelination of the central nervous system (CNS) (i.e., acquired demyelinating syndrome, ADS). Often, the overlap of GBS and ADS is overlooked. Therefore, we evaluated case reports of GBS/ADS overlap syndrome.
We mainly performed website-based research based on articles in cases presented with GBS/ADS overlap syndrome. A total of 66 cases were included. Clinical and prognosis data were analyzed.
A total of 85% of patients with simultaneous or consecutive occurrence of GBS and ADS were identified within 4 weeks of the initial diagnosis. Transverse myelitis (TM) (32%) was the most common ADS found in GBS/ADS. Patients with Miller Fisher syndrome (MFS)/ADS overlap syndrome had greater female predominance, mean age, frequency of onset at the same time period, or within a short period, and percentage of sole involvement of the subtentorial region. The outcome was favorable based on the functional status in 74% of patients. The sensory level (OR = 0.182, 95% CI = 0.055-0.598; P = 0.005) was the best predictor of a poor outcome, while visual deficit (OR = 4.667, 95% CI = 1.187-18.352; P = 0.027) predicted a favorable outcome.
The ADS in GBS are diverse, CNS demyelinating may occur at any time, but early in the GBS course (and vice versa). MFS/ADS overlap syndromes is more common. The prognosis is generally good, but patients with sensory level deficit are likely to have a poor prognosis. The features of MFS/other CIS may better reflect involvement of the brainstem in MFS itself, rather than ADS in autoimmune peripheral neuropathies.
一些吉兰 - 巴雷综合征(GBS)患者也存在中枢神经系统(CNS)获得性脱髓鞘(即获得性脱髓鞘综合征,ADS)。GBS和ADS的重叠情况常常被忽视。因此,我们对GBS/ADS重叠综合征的病例报告进行了评估。
我们主要基于GBS/ADS重叠综合征病例的文章进行基于网络的研究。共纳入66例病例。对临床和预后数据进行了分析。
8岁5%同时或相继发生GBS和ADS的患者在初始诊断后4周内被确诊。横贯性脊髓炎(TM)(32%)是GBS/ADS中最常见的ADS类型。伴有米勒费雪综合征(MFS)/ADS重叠综合征的患者女性占比更高,平均年龄更大,同时期或短期内发病频率更高,以及幕下区域单独受累的比例更高。根据功能状态,74%的患者预后良好。感觉平面(OR = 0.182,95%CI = 0.055 - 0.598;P = 0.005)是预后不良的最佳预测指标,而视觉缺陷(OR = 4.667,95%CI = 1.187 - 18.352;P = 0.027)预测预后良好。
GBS中的ADS多种多样,CNS脱髓鞘可能在任何时间发生,但多在GBS病程早期(反之亦然)。MFS/ADS重叠综合征更为常见。总体预后良好,但有感觉平面缺陷的患者预后可能较差。MFS/其他临床孤立综合征的特征可能更好地反映MFS本身脑干的受累情况,而非自身免疫性周围神经病中的ADS。