Murakami Takenobu, Yoshihara Akioh, Kikuchi Saeko, Yasuda Megumi, Hoshi Akihiko, Ugawa Yoshikazu
Department of Neurology, Fukushima Medical University.
Rinsho Shinkeigaku. 2013;53(4):299-303. doi: 10.5692/clinicalneurol.53.299.
A 69-year-old woman complained of diplopia and truncal titubation after upper respiratory infection. She presented with mydriasis and external opthalmoplegia of bilateral eyes, ataxia, hyporeflexia and cervical-brachial muscle weakness. The protein abnormally increased (49 mg/dl) in the cerebrospinal fluid, and the serum anti-GQ1b and anti-GT1a IgG antibodies were positive. The blood sodium level was 128 mmol/l indicating hyponatremia. She had low plasma osmolarity (251 mOsm/kg), high urine osmolarity (357 mOsm/kg) and high urine sodium level (129 mmol/l), while the blood level of antidiuretic hormone was not able to be measured. She was diagnosed to have Fisher syndrome (FS), pharyngeal-cervical-brachial variant of Guillain-Barré syndrome (PCB) and syndrome of inappropriate secretion of antidiuretic hormone (SIADH). The hyponatremia improved with hyperosmotic saline infusion and restriction of water intake. Intravenous immunoglobulin therapy (IVIg) was effective only for ataxia, but the other symptoms mostly remained unchanged for a month. The serum anti-GQ1b IgG antibody was still positive even after one month. We performed high-dose intravenous steroid-pulse therapy. Then the mydriasis, external opthalmoplegia and cervical-brachial muscle weakness were immediately improved. This was a rare case of FS and PCB complicated with SIADH. IVIg, not steroid therapy, is generally chosen for FS since FS is considered as a variant of Guillain-Barré syndrome and steroid is not effective for Guillain-Barré syndrome as was proven by double-blind study. We suppose that the combined therapy of IVIg and steroid would be effective in patients with complicated symptoms and multiple antibodies.
一名69岁女性在患上呼吸道感染后出现复视和躯干震颤。她表现为双侧瞳孔散大及眼球外展麻痹、共济失调、反射减退和颈臂肌无力。脑脊液中蛋白质异常升高(49mg/dl),血清抗GQ1b和抗GT1a IgG抗体呈阳性。血钠水平为128mmol/l,提示低钠血症。她血浆渗透压降低(251mOsm/kg),尿渗透压升高(357mOsm/kg),尿钠水平升高(129mmol/l),而抗利尿激素的血水平无法测得。她被诊断为费舍尔综合征(FS)、吉兰 - 巴雷综合征咽颈臂变异型(PCB)和抗利尿激素分泌不当综合征(SIADH)。通过输注高渗盐水和限制水摄入,低钠血症得到改善。静脉注射免疫球蛋白治疗(IVIg)仅对共济失调有效,但其他症状在一个月内大多保持不变。即使一个月后血清抗GQ1b IgG抗体仍为阳性。我们进行了大剂量静脉类固醇冲击治疗。随后瞳孔散大、眼球外展麻痹和颈臂肌无力立即得到改善。这是一例罕见的FS和PCB合并SIADH的病例。由于FS被认为是吉兰 - 巴雷综合征的一种变异型,且双盲研究已证明类固醇对吉兰 - 巴雷综合征无效,所以FS一般选择IVIg而非类固醇治疗。我们推测IVIg和类固醇联合治疗对有复杂症状和多种抗体的患者可能有效。