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1、Case Report,病例：53岁女性伴下肢麻木无力 2017年06月发表于新英格兰杂志 资料来源于麻省总医院,53岁女性，有类风湿关节炎病史。 首发症状为左足外侧感觉减退。 病史4个月。,病例介绍,During the following 2 days The hypoesthesia spread to involve the toes and midfoot; chronic low-back pain, which radiated to the buttocks, had increased in intensity, but there was no pain in the le
2、gs, fever, weight loss, or bowel or bladder changes. Examination: Perception of light touch and pinprick was absent on the left toes and left forefoot; the remainder of the examination was normal. Lab: ESR, VB12, glycated hemoglobin, thyrotropin were normal.,Presentation of Case,病例介绍,首次症状出现后2天： 症状：感
3、觉减退逐渐发展到左足趾及左脚掌，同时存在慢性背痛，并放射到臀部，且程度加强，无双下肢疼痛，无发热，无体重减轻，无大小便异常。 查体：左足趾及左脚掌浅感觉及针刺觉缺失，余查体未见明显异常。 检查化验：血沉、维生素B12、糖化血红蛋白、促甲状腺素未见异常。,During the next 5 days hypoesthesia progressed to involve the proximal portion of the left foot, as well as the ankle and the lateral lower leg, but no pain or bowel or blad
4、der incontinence. Examination: cranial-nerve examinations were normal. Strength was 4+ out of 5 during flexion and extension of the toes on the left foot, dorsiflexion and plantar flexion of the left ankle, eversion and inversion of the left foot, and flexion of the left knee. Perception of vibratio
5、n was markedly diminished at the left hallux. Perception of light touch and pinprick was decreased on the left foot and the left lateral leg. Deep-tendon reflexes of the arms and legs were normal. The plantar reflex was flexor on the right side and absent on the left side.,Presentation of Case,病例介绍,
6、此后的5天： 症状：感觉减退逐渐进展至整个左脚、左脚踝、左小腿外侧，但无肢体疼痛及大小便失禁。 查体： 颅神经查体未见异常。 左足趾屈伸运动、左踝关节趾屈和背屈、左足内外翻、左膝关节屈曲肌力检查4+级。 左足趾震动觉明显减退。 左足、左小腿触觉、针刺觉减退。 四肢腱反射正常。 左侧足跖反射消失，右侧正常。,7 days later (3 months before this admission) The sensory and motor responses were normal and symmetric. F-wave minimal latencies in the tibial ne
7、rves were normal. H-reflex was absent at the left soleus and normal at the right soleus. Needle electromyography of the left leg showed diminished recruitment of long-duration, polyphasic motor-unit potentials in proximal and distal muscles of the L5 myotome. Results of electromyography of the right
8、 leg were normal.,Nerve-conduction studies,神经传导速度,7天后（本次入院前3个月）： 感觉、运动神经传导速度正常、对称。 胫神经F波潜伏期正常。 左侧胫神经H反射消失，右侧正常。 针肌电图显示左下肢长时程募集相及腰5神经支配肌肉的多相运动电位减低，右下肢未见异常。,Imaging,L3-S1,T1-weighted, show clumped, thickened, and enhancing cauda equina nerve roots in the lumbar spinal canal,影像,腰椎MRI+强化： 平扫显示腰椎间盘突出、黄韧带
9、增厚、小关节病，可见L4-L5水平椎间孔、L5-S1水平椎间孔狭窄。 箭头处所示L3-S1水平神经根增粗，强化。背根神经节及周围神经，对称未见强化。,CSF analysis,Oligoclonal banding was not. Cytologic studies showed increased lymphocytes that were polymorphous and occasionally reactive and appeared to be benign. Immunophenotypic evaluation did not reveal evidence of a mon
10、oclonal B-cell or unusual T-cell population.,脑脊液分析,寡克隆带(-)。 淋巴细胞增多，呈多形性表现，考虑反应增多是良性表现。 脑脊液免疫表型未见单克隆B细胞或T细胞数量异常。 葡萄糖轻度减低为41mg/dl（正常值 50-75mg/dl），蛋白为207mg/dl（正常值 5-55mg/dl）。 巨细胞及EB病毒检测阴性。,CIDP,Provisional diagnosis,Intravenous immune globulin was administered 4 weeks and 1 week before the patient was
11、admitted to this hospital.,初步诊断,慢性炎症性脱髓鞘性多发性神经根神经病 分别于本次入院前4周、1周给予丙种球蛋白治疗,Despite the administration of intravenous immune globulin, symptoms in the left leg did not improve, and hypoesthesia and weakness developed in the right leg and progressed centripetally. The patient had several falls at home
12、and began to use a cane, then a walker, and ultimately a wheelchair. The patient reported episodes of bowel and bladder incontinence that she attributed to leg weakness interfering with her ability to walk to the bathroom quickly.,Admission,入院情况,在接受丙种球蛋白治疗后，患者左下肢症状未见改善，且无力及感觉减退进展到右下肢，并向心性发展。 患者曾在家多次
13、跌倒，并开始使用拐杖，后改用助力器，最终患者因病情只能借助轮椅活动。 患者此次就诊于急诊，表诉存在大小便失禁，但患者认为是因为下肢无力症状而妨碍她去往卫生间。,She reported no fevers, weight loss, or night sweats. Rheumatoid arthritis had been diagnosed 18 months before this admission, and therapy with methotrexate and prednisone had been initiated. Because of persistent diseas
14、e activity, therapy with etanercept was initiated 7 months before this admission, after an interferon- release assay for Mycobacterium tuberculosis had been negative.,Admission,入院情况,患者无发热、体重减轻、盗汗。 患者于本次入院前18个月确诊类风湿关节炎，并接受甲氨蝶呤、强的松治疗，因疾病仍处于活动期，在进行结核分枝杆菌干扰素释放实验阴性后，加用伊那西普治疗。,CT of the chest: there were
15、subcentimeter pulmonary nodules in the bilateral lower lobes that had remained unchanged over a period of 4 years. Results of routine screening mammography performed 1 month before this admission were normal.,Admission,入院情况,肺CT显示双肺底可见小结节，4年内肺CT比较未见明显改变。 入院前1个月乳腺X线检查未见明显异常。,Medication: omeprazole, me
16、thotrexate, prednisone, folic acid, gabapentin, and duloxetine. The patient had recently stopped taking etanercept, because she thought her progressive symptoms could be related to that medication. Medical history: gastroesophageal reflux disease, uterine fibroids, and -thalassemia minor. Personal history: She was a current smoker, and did not drink alcohol or use illicit drugs. Family history: Her sister had multiple sclerosis; there was no family history of cancer.,
