Kubo J, Hozumi A, Mihara B, Gomi S, Hirata K
Department of Neurology, Mihara Memorial Hospital, Instituete of Brain and Vessels, 366 Ohtamachi, Isesaki, Gunma 372-0006, Japan.
No To Shinkei. 2001 Jul;53(7):649-51.
A 69-year-old woman was admitted to our hospital because of slowly progressive weakness in the neck, shoulders, proximal arms, oropharyngeal muscles. From a viewpoint of clinical course and signs, it was suspected that the patient was suffered from motor neuron disease. However, serial electrophysiological studies showed the existence of local demyelination of the motor nerves. The immunoadsorption was then performed and marked recovery of symptoms was obtained. In this case, we could not detect any established anti-ganglioside antibodies which was related to pharyngeal-cervical-brachial variant of Guillain-Barré syndrome(PCB) or Guillain-Barré syndrome (GBS). It is suggested that unknown anti-ganglioside antibody may play an important role in cases of PCB. Despite of atypical slowliness of clinical progression and negative results of immunological studies, this patient is considered to be suffered from PCB, because of the results of electrophysiological studies and effectiveness of immunoadsorption therapy. Accordingly it may be important to take the possibility of PCB into diagnostic consideration, whenever the patient shows slowly progressive weakness in proximal arms, oropharyngeal muscles.
一名69岁女性因颈部、肩部、近端手臂及口咽肌无力呈缓慢进行性加重而入住我院。从临床病程和体征来看，怀疑该患者患有运动神经元病。然而，系列电生理研究显示存在运动神经局部脱髓鞘。随后进行了免疫吸附治疗，症状明显改善。在该病例中，我们未检测到任何与吉兰-巴雷综合征咽颈臂变异型（PCB）或吉兰-巴雷综合征（GBS）相关的已确定的抗神经节苷脂抗体。提示未知抗神经节苷脂抗体可能在PCB病例中起重要作用。尽管临床进展非典型缓慢且免疫学研究结果为阴性，但鉴于电生理研究结果及免疫吸附治疗的有效性，该患者仍被认为患有PCB。因此，每当患者出现近端手臂、口咽肌无力且呈缓慢进行性加重时，考虑PCB的可能性对于诊断可能很重要。
