眼肌麻庳、運動失調和深部肌腱反射消失症候群：病變位置在周邊或是中樞神經系統？

本文提出3個Fisher氏症候群的病例，他們的臨床特徵是眼冗麻痺、運動失調和深部肌腱反射消失。此外，這3個病例的眼球向下轉動不受影響，病例2和3有Bell氏現象，而病例2的顱神經2、7、9、10和12以及病例3的7、9及10皆受侵犯。在這3個病例中，病例1的神經傳導正常，聽覺誘發電位和體感覺誘發電位檢查顯示橋腦及內側蹄係路徑異常。病例2兩側正中及右側尺骨運動神經傳導速度延長，兩側H反射消失，而聽覺誘發電位顯示橋腦異常。病例3的左側尺骨運動神經傳導速度減慢、兩側H反射潛時延長而眨眼反射檢查顯示橋腦延髓異常。雖然此3病例神經電學檢查結果無法圓滿解釋其臨床症狀，然而暗示著周邊和中樞神經系統同時受侵犯的可能。

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並列摘要

Three cases of Fisher’s syndrome presenting with ophthalmoplegia, ataxia and areflexia were described. Clinical analysis was made in these 3 patients. There werer downward gaze sparing in all cases, Bell’s phenomenon and other cranial nerves dysfunction in case 2 and 3. On the basis of electrophysiological findings, case 1 had normal nerve conduction velocity with abnormal auditory evoked potentials and somatosensory evoked potentials. Case 2 had prolonged bilateral median and right ulnar nerve motor conduction velocity, absence of bilateral H reflexes and abnormal auditory evoked potentials. The remaining case had prolonged left ulnar nerve motor conduction velocity, prolonged bilateral H reflexes and abnormal blink reflexes. The electrophysiological data could not explain all of the clinical finding of these three patients, but they provided the evidence of combined lesions of the peripheral and central nervous system.

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