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[伴有严重低血压的费希尔综合征]

[Fisher's syndrome with profound hypotension].

作者信息

Arai M, Yuki N

机构信息

Department of Neurology, Seirei Mikatabara General Hospital.

出版信息

Rinsho Shinkeigaku. 1996 Jun;36(6):797-9.

PMID:8937206
Abstract

A case of Fisher's syndrome was described in which profound hypotension was encountered. A 70-year-old previously healthy female developed diplopia and unsteady gait following an upper respiratory infection. On admission, her blood pressure was 140/60 mmHg. Adduction of the left eye and elevation of the right eye were limited. Other cranial nerve functions and muscle strength were intact. She had gait ataxia with negative Romberg sign. Tendon reflexes were hypoactive. The cerebrospinal fluid, electrocardiography, and cranial MR imaging were unremarkable. Her serum had a high titer of IgG anti-body. After admission she developed bilateral internuclear ophthalmoplegia and severe gait ataxia. During double filtration plasmapheresis, her systolic pressure fell down to 60 mmHg. To maintain her systolic pressure between 90 and 120-mmHg supine, infusion of lactated Ringer solution (3,000-3,500 ml/day), dopamine hydrochloride (10-12 micrograms/kg/min), and norepinephrine (50-90 ng/kg/min) was needed for 3 weeks. There was no evidence of denervation supersensitivity. Echocardiography demonstrated normal cardiac output, suggesting that decreased vascular resistance was responsible for her hypotension. In Guillain-Barré syndrome, cardiovascular dysautonomia is frequently encountered and is occasionally related to sudden death. In contrast, Fisher's syndrome with cardiovascular dysautonomia has been rarely reported. Cardiovascular functions should be monitored in every patient with Fisher's syndrome.

摘要

本文描述了一例伴有严重低血压的费希尔综合征病例。一名70岁既往健康的女性在一次上呼吸道感染后出现复视和步态不稳。入院时,她的血压为140/60 mmHg。左眼内收和右眼上抬受限。其他颅神经功能和肌力均正常。她有步态共济失调,罗姆伯格征阴性。腱反射减弱。脑脊液、心电图和头颅磁共振成像均无异常。她的血清中有高滴度的IgG抗体。入院后她出现双侧核间性眼肌麻痹和严重的步态共济失调。在双重滤过血浆置换过程中,她的收缩压降至60 mmHg。为使她仰卧时收缩压维持在90至120 mmHg之间,需要连续3周输注乳酸林格液(3000 - 3500 ml/天)、盐酸多巴胺(10 - 12微克/千克/分钟)和去甲肾上腺素(50 - 90纳克/千克/分钟)。没有去神经超敏反应的证据。超声心动图显示心输出量正常,提示血管阻力降低是导致她低血压的原因。在吉兰 - 巴雷综合征中,心血管自主神经功能障碍很常见,偶尔与猝死有关。相比之下,伴有心血管自主神经功能障碍的费希尔综合征很少有报道。每例费希尔综合征患者都应监测心血管功能。

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