Sauron B, Bouche P, Cathala H P, Chain F, Castaigne P
Neurology. 1984 Jul;34(7):953-6. doi: 10.1212/wnl.34.7.953.
Clinical and electrophysiologic data recorded in patients with Fisher syndrome characterized by ophthalmoplegia, ataxia, and areflexia are presented. Cases with limb weakness or pleocytosis in the CSF were excluded, according to Fisher. Ten patients were selected. All had hand and foot numbness. A large amount of protein without cell reaction was found during the third week of illness in serial CSF examinations. EMGs showed very slight limb involvement without spontaneous activity, and in most cases, facial muscles exhibited a denervation pattern. Distal motor nerve conduction velocity on limbs and F wave latencies were normal, whereas the sensory nerve action potentials were altered in all but one case when tested (seven out of eight cases). By means of blink reflex study performed in four patients, no significant pattern of brainstem dysfunction was discovered. The authors discuss the preeminent role of peripheral nerve lesions with regard to the ataxia and ophthalmoplegia.
本文呈现了以眼肌麻痹、共济失调和无反射为特征的费舍尔综合征患者的临床和电生理数据。根据费舍尔的标准,排除了伴有肢体无力或脑脊液中细胞增多的病例。共选取了10例患者。所有患者均有手足麻木症状。在连续的脑脊液检查中,发病第三周发现大量蛋白而无细胞反应。肌电图显示肢体受累非常轻微,无自发活动,且在大多数情况下,面部肌肉呈现失神经模式。肢体远端运动神经传导速度和F波潜伏期正常,而在测试时,除1例(8例中的7例)外,所有患者的感觉神经动作电位均有改变。通过对4例患者进行眨眼反射研究,未发现明显的脑干功能障碍模式。作者讨论了周围神经病变在共济失调和眼肌麻痹方面的突出作用。