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患者女性32岁,因复视,走路不稳13天于1986年8月12日入院,不伴发热、头痛、呕吐。病前2周有上感史,病后第6天症状加重。入院查体:意识清楚、言语尚流利,血压15/10kPa,双上睑下垂,双眼球略呈内收位,双眼外展不能,向内、向上、向下运动力弱。瞳孔等大同圆约3mm,对光反射存在。眼底正常,无眼震,双侧睫毛征(十)。面部感觉正常,双侧鼓腮力量略弱。听力及舌咽迷走和舌下神经检查正常。四肢无感觉障碍,肌力正常,无肌萎缩及不自主运动。走路不隐,Romberg’s 征(十)、双侧指鼻及跟膝胫试验(十)。四肢腱反射和病理反射未引出,脑膜刺激征(一),胸腹部及四肢脊柱检查正常。辅助检查:CSF 压力1.42kPa、蛋白0.72/g/L、自细胞3×10~6/L。糖和氯化物正常。肌电图正常,MCV 略减慢,SCV 正常。头 CT 扫描和心电图正常。血尿常规、血沉、抗 O、血糖、血钾,血钠、血肌酸和肌酐、血 BUN 和 CO_2—CP、肝功均正常。临床诊断:Fisher 综合征。应用地塞米松、胞二磷胆碱、三磷酸腺苷及维生素 B 族等药物,治疗41天走路正常、复视消失,此后出院。间隔2个月复查仍有腱反射减弱。本患具有以下几个特点:①双侧眼外肌及其它颅神经运动障碍;②小脑性共济失调;③四肢腱反射减弱或消失;④脑脊液中蛋白细胞分离现象;⑤起病急预后较好。⑥以上符合 Fisher 综合征诊断标准,但需同脑干的炎症和血管疾病、后颅窝肿瘤、多发性硬化、Wernick 脑病、Kearne-Sayre’s 综合征,重症肌无力,肉毒中毒等病鉴别。目前关于 Fisher 综合征的病因、发病机理等方面还不十分清楚,认为其是 GBS 综合征的变异型。由于本患周围神经没有做病理检查,能否把神经传导速度变化做为诊断周围神经脱髓鞘改变的依据值得探讨。(1996—05—26收稿)
Female 32 years old, due to diplopia, walking instability for 13 days in August 12, 1986 admission, without fever, headache, vomiting. There is a sense of illness 2 weeks before illness, symptoms after the first 6 days of illness aggravate. Admission examination: Consciousness, the language is still fluent, blood pressure 15 / 10kPa, double ptosis, eyes slightly retracted, eyes can not be outreach, inward, upward, downward movement weak. Pupils and other Datong round about 3mm, there is light reflection. Fundus normal, no nystagmus, bilateral eyelashes sign (X). The face feels normal, with a slightly weaker strength on both sides of the drum. Hearing and glossopharyngeal vagal and sublingual nerve tests were normal. Limbs without sensory disturbances, muscle strength, muscle atrophy and involuntary movements. Walking is not hidden, Romberg’s sign (ten), bilateral nasolabial and knee tibia test (ten). Extremities tendon reflex and pathological reflex did not lead to meningeal irritation (a), chest and abdomen and limbs spine normal. Auxiliary examination: CSF pressure 1.42kPa, protein 0.72 / g / L, since the cells 3 × 10 ~ 6 / L. Sugar and chloride are normal. EMG normal, MCV slightly slowed, SCV normal. Head CT scan and ECG normal. Hematuria, ESR, anti-O, blood glucose, serum potassium, serum sodium, creatine and creatinine, blood BUN and CO_2-CP, liver function were normal. Clinical diagnosis: Fisher syndrome. Dexamethasone, citicoline, adenosine triphosphate and vitamin B family and other drugs, treatment of 41 days walk normal diplopia disappear, then discharged. Retinal reflex was still diminished after 2 months of review. The patient has the following characteristics: ① bilateral extraocular muscles and other cranial nerve dyskinesia; ② cerebellar ataxia; ③ limb tendon reflexes weakened or disappeared; ④ cerebrospinal fluid protein cells isolated; ⑤ onset acute prognosis than it is good. ⑥ more consistent with the Fisher syndrome diagnostic criteria, but with the brain stem inflammation and vascular disease, posterior fossa tumors, multiple sclerosis, Wernick encephalopathy, Kearne-Sayre’s syndrome, myasthenia gravis, botulism and other diseases identified. The current cause of Fisher syndrome, pathogenesis and other aspects is not yet very clear, that it is a variant of GBS syndrome. As the peripheral nerves did not do pathological examination, whether the nerve conduction velocity changes as a basis for diagnosis of peripheral nerve demyelination is worth exploring. (1996-05-26 Received)