Dididze M N
Department of Neurology, Tbilisi State Medical University, Georgia.
Georgian Med News. 2009 Jan(166):48-51.
Acute Inflammatory Demyelinating Polyneuropathy--Guillain-Barre syndrome (GBS) affects spinal roots, peripheral and cranial nerves. Various clinical variants of GBS have been described. Isolated cranial nerve involvement without prominent signs of GBS is considered as rare variant of this disease. The aim of the study was to identify clinical characteristics of various forms of GBS particularly in rare variants of the disease. 57 patients with GBS were evaluated based on clinical and electrophysiological data. The following forms of GBS were revealed: 27 had acute inflammatory demyelinating polyradiculoneuropathy, 9--acute motor axonal neuropathy, 12--acute motor and sensory axonal neuropathy, 5--Fisher syndrome and 3--facial diplegia (rare clinical variant). 50 patients were graded 3 or more according to Hughes functional grading scale. Seasonal preponderance was found in spring (March-May) and autumn (September-November). 23 patients received IVIG and 34 were treated by plasma exchange within two weeks after onset. Follow up study revealed: 46 recovered satisfactory, 8 were persistently disabled, 3 died during admission to hospital. Guillain-Barre syndrome showed seasonal distribution and high frequency of axonal forms. Intravenous immunoglobulin therapy was more effective than plasma exchange. Poor outcomes were likely due to severe condition (required mechanical ventilation) and axonal forms. It is crucial to timely identify rare variants of GBS which recover with appropriate treatment.
急性炎性脱髓鞘性多发性神经病——吉兰-巴雷综合征(GBS)会影响脊神经根、周围神经和颅神经。GBS的各种临床变体已被描述。孤立的颅神经受累且无GBS明显体征被认为是该疾病的罕见变体。本研究的目的是确定各种形式GBS的临床特征,尤其是该疾病的罕见变体。基于临床和电生理数据对57例GBS患者进行了评估。发现了以下几种GBS形式:27例为急性炎性脱髓鞘性多发性神经根神经病,9例为急性运动轴索性神经病,12例为急性运动和感觉轴索性神经病,5例为费舍尔综合征,3例为双侧面瘫(罕见临床变体)。根据休斯功能分级量表,50例患者分级为3级或更高。在春季(3月至5月)和秋季(9月至11月)发现有季节性优势。23例患者在发病后两周内接受了静脉注射免疫球蛋白治疗,34例接受了血浆置换治疗。随访研究显示:46例恢复良好,8例持续残疾,3例在住院期间死亡。吉兰-巴雷综合征呈季节性分布且轴索形式发生率高。静脉注射免疫球蛋白治疗比血浆置换更有效。预后不良可能是由于病情严重(需要机械通气)和轴索形式。及时识别GBS的罕见变体并进行适当治疗使其恢复至关重要。