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患者男.54岁.双下肢无力1月余,进行性加重20天.于1992年8月1日以椎管狭窄症收入院.7年前患甲型肝炎.经治疗自觉症状好转、食欲佳,尔后一直从事重体力劳动.2个月前感到双下肢沉重、乏力,无麻痛、行走无障碍.1个月前出现两下肢软弱无力,步态不稳,运动障碍逐渐加重,两腿拖行.扶物代步,家族中无类似疾病.病后食欲如常、体质无消瘦,无发烧、咳嗽及咯血,二便如常.体检:神清,面色晦暗,颈及前胸多个蜘蛛痣,皮肤、巩膜无黄染.心、肺功能无异常.腹壁静脉无明显怒张,无压痛、肝脾未触及.腹水征阳性.脊柱生理弯曲存在,无畸形.伸屈、侧弯正常,无压痛及叩击痛.双下肢肌张力正常,肌力V级.运动自如.两下肢肌张力略减弱,肌力Ⅳ级,髋、膝、踝关节伸屈正常,两足背侧L°水肿.直腿抬高试验双侧阴性.膝腱反射两侧亢进,髌震挛及踝震挛双侧阳性,巴氏征两侧阳性.无肌肉萎缩及肌束震颤.深浅感觉均正常存在,括约肌功能无障碍.颅神经无异常.实验室检查:血、尿、粪常规均正常.总胆红质正常.SGPT18单位,TTT7.8单位,白蛋白20g/L,球蛋白37g/L.HBsAg阴性,血清钾4.0mmol/L,血清铜14μmol/L.B超:肝硬变,腹水,脾大.胸及腰椎X线摄片未见异常,椎管造影未见占位性病变.脑脊液外观无色透明,
54 years old patients with lower extremity weakness more than 1 month, progressive aggravating 20 days in August 1, 1992 with spinal canal stenosis income hospital 7 years ago with hepatitis A. After treatment symptoms improved, good appetite , Has been engaged in heavy manual labor .2 months ago, feeling both lower extremities heavy, fatigue, no numbness, walking accessibility .1 months ago appeared weakness in both lower extremities, unsteady gait, dyskinesia gradually increased, legs dragged Line. Bundle walk, there is no similar disease in the family. After the illness appetite as usual, no weight loss, no fever, cough and hemoptysis, two will be normal. Physical examination: Shen Qing, looking dull, neck and chest multiple spider nevus, skin , No yellow sclera stained heart and lung function without abnormal abdominal wall veins no obvious engorgement, no tenderness, liver and spleen untouched positive signs of ascites spine physiological bending, no deformity flexion and extension, scoliosis normal, no tenderness and Percussion pain .Lower limb muscle tension is normal, muscle strength V. Motorized .Lower limb muscle tension slightly weakened, muscle strength class Ⅳ, hip, knee, ankle flexion and extension of the normal, two dorsal dorsal L ° edema. Both sides of the elevation test negative knee tendon reflexes on both sides of the hyperextension, patellofemojiao and ankle seizures on both sides of the positive, positive on both sides of the Papanicolaou, no muscle Atrophy and fasciculation of the fascia. Sense of depth were normal, sphincter function was normal. No abnormal cranial nerves. Laboratory tests: blood, urine, normal feces were normal. Total bilirubin normal. SGTT18 units, TTT7.8 units, white Protein 20g / L, globulin 37g / L.HBsAg negative, serum potassium 4.0mmol / L, serum copper 14μmol / LB Ultra: cirrhosis, ascites, splenomegaly. Thoracic and lumbar X-ray showed no abnormalities, spinal canal Contrast no space-occupying lesions. Appearance of cerebrospinal fluid is colorless and transparent,