Pyun So Young, Jeong Jin-Ho, Bae Jong Seok
Department of Neurology, National Police Hospital, Seoul, Republic of Korea.
Busan Paik Hospital, Inje University College of Medicine, Busan, Republic of Korea.
Clin Neurol Neurosurg. 2015 Dec;139:230-3. doi: 10.1016/j.clineuro.2015.10.022. Epub 2015 Oct 19.
Recurrent Guillain-Barré syndrome (rGBS) has been described as a rare entity with distinct characteristics. However, little is known about rGBS in Asian group. The aim of this study was to identify the incidence and clinical course of rGBS, and to determine its clinical/pathophysiological implications.
The consecutive data of 117 GBS patients were retrieved from a single university-based hospital in Korea and analyzed in terms of clinical, serological, electrophysiological aspects.
A thorough review revealed that three (2.6%) of the enrolled patients had experienced more than two definite recurrent attacks of GBS. Interestingly, all three cases exhibited clinically stereotypical features, serum antiganglioside antibodies, and rapid recovery after intravenous immunoglobulin treatment. Clinical, serological, and electrophysiological features of rGBS cases were described in detail.
The stereotypic presentation of each attack in this variant suggests the importance of both host and genetic factors for the clinical manifestations. In addition, the simultaneous presence of serum antiganglioside antibodies and rapid recovery implicate reversible nerve conduction failure as the mechanism of rGBS. These features are different from typical monophasic GBS and acute onset of chronic inflammatory demyelinating polyneuropathy.
复发性吉兰 - 巴雷综合征(rGBS)被描述为一种具有独特特征的罕见疾病。然而,亚洲人群中rGBS的情况鲜为人知。本研究旨在确定rGBS的发病率和临床病程,并确定其临床/病理生理意义。
从韩国一家大学附属医院检索117例GBS患者的连续数据,并从临床、血清学、电生理方面进行分析。
全面回顾发现,纳入的患者中有3例(2.6%)经历过两次以上明确的GBS复发发作。有趣的是,所有3例均表现出临床刻板特征、血清抗神经节苷脂抗体以及静脉注射免疫球蛋白治疗后快速恢复。详细描述了rGBS病例的临床、血清学和电生理特征。
该变异型每次发作的刻板表现提示宿主和遗传因素对临床表现的重要性。此外,血清抗神经节苷脂抗体的同时存在和快速恢复表明可逆性神经传导衰竭是rGBS的机制。这些特征不同于典型的单相GBS和慢性炎症性脱髓鞘性多发性神经病的急性起病。