Division of Neurology, Department of Medicine, Faculty of Medicine, University of Malaya, 50603, Kuala Lumpur, Malaysia.
Muscle Nerve. 2014 Apr;49(4):558-63. doi: 10.1002/mus.23973. Epub 2014 Jan 27.
Differing patterns of neurophysiological abnormalities have been reported in patients with Fisher syndrome. Fisher syndrome is rare, and few series have incorporated prospective serial studies to define the natural history of nerve conduction studies in Guillain-Barré syndrome.
In an ongoing prospective study of Guillain-Barré syndrome patients, patients who presented with Fisher syndrome and its spectrum of illness were assessed through serial neurological examinations, nerve conduction studies, and serological testing of IgG against gangliosides and ganglioside complexes.
Of the 36 Guillain-Barré syndrome patients identified within 2 years, 17 had features of Fisher syndrome. Serial nerve conduction studies detected significant abnormalities in sensory nerve action potential amplitude in 94% of patients associated with 2 patterns of recovery-non-demyelinating reversible distal conduction failure and axonal regeneration. Similar changes were seen in motor nerves of 5 patients.
Patients with the Fisher syndrome spectrum of illness have significant sensory involvement, which may only be evident with serial neurophysiological studies.
在 Fisher 综合征患者中,已经报道了不同模式的神经生理学异常。Fisher 综合征较为罕见,少数系列研究纳入了前瞻性连续研究,以明确 Guillain-Barré 综合征中神经传导研究的自然病程。
在一项正在进行的 Guillain-Barré 综合征患者前瞻性研究中,对出现 Fisher 综合征及其一系列疾病的患者进行了连续的神经系统检查、神经传导研究以及针对神经节苷脂和神经节苷脂复合物的 IgG 的血清学检测。
在 2 年内确定的 36 例 Guillain-Barré 综合征患者中,有 17 例具有 Fisher 综合征的特征。连续的神经传导研究在 94%的患者中发现了感觉神经动作电位幅度的显著异常,这些患者存在两种恢复模式,即非脱髓鞘性可逆性远端传导失败和轴突再生。5 例运动神经也出现了类似的变化。
Fisher 综合征谱疾病患者有明显的感觉受累,这可能只有通过连续的神经生理学研究才能发现。