Shinoda Koji, Murai Hiroyuki, Shibata Ken-Ichi, Samejima Shoko, Kaneto Shuji, Takashima Nobuyoshi, Tanaka Kimihiro
Department of Neurology, Iizuka Hospital.
Rinsho Shinkeigaku. 2012;52(1):30-3. doi: 10.5692/clinicalneurol.52.30.
A 29-year-old female developed diplopia, nasal voice and gait disturbance after an upper respiratory infection. On admission, she presented with bilateral external ophthalmoplegia, slight bilateral facial nerve palsy, dysarthria, dysphagia, cervical and brachial muscle weakness, ataxia and areflexia. She had serum anti-GT1a, anti-GQ1b and anti-galactocerebroside IgG antibodies. She was diagnosed with an overlap case of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. Intravenous immunoglobulin therapy was effective for the ophthalmoplegia and ataxia, but did not improve the bilateral facial nerve palsy and brachial muscle weakness. The facial nerve palsy clearly worsened despite improvement in other symptoms, and therefore high-dose intravenous methylprednisolone therapy was added. The distinct response to treatment may be caused by different activity, production, clearance and reactivity to intravenous immunoglobulin of the autoantibodies. The present case suggests that treatment response and patterns of recovery differ according to the causative anti-ganglioside antibodies.
一名29岁女性在上呼吸道感染后出现复视、鼻音和步态障碍。入院时,她表现为双侧眼球外肌麻痹、轻度双侧面神经麻痹、构音障碍、吞咽困难、颈部和臂部肌肉无力、共济失调和反射消失。她血清中存在抗GT1a、抗GQ1b和抗半乳糖脑苷脂IgG抗体。她被诊断为Fisher综合征和格林-巴利综合征咽颈臂型的重叠病例。静脉注射免疫球蛋白治疗对眼球外肌麻痹和共济失调有效,但未改善双侧面神经麻痹和臂部肌肉无力。尽管其他症状有所改善,但面神经麻痹明显加重,因此加用了大剂量静脉注射甲泼尼龙治疗。治疗反应不同可能是由于自身抗体对静脉注射免疫球蛋白的活性、产生、清除和反应性不同所致。本病例表明,根据致病性抗神经节苷脂抗体的不同,治疗反应和恢复模式也有所不同。