Pathogenesis of multiple system atrophy.
Pathogenesis of multiple system atrophy.
复制标题
多系统萎缩的发病机制。
DOI：
10.1007/s00415-014-7271-5
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发表时间：
2013
影响因子：
0.4
通讯作者：
Takeda A.
中科院分区：
文献类型：
--
作者：
Hasegawa T;Kikuchi A;Takeda A.
多灶性获得性脱髓鞘感觉和运动神经病（MADSAM）[1]定义为慢性炎性脱髓鞘性多发性神经病（CIDP）的不对称表现。MADSAM的神经传导研究（NCS）显示特征性脱髓鞘改变，如传导阻滞和时间离散。我们报告一例MADSAM只有F波缺席三个月后，临床发病的疾病和部分运动传导阻滞，在左前臂首次发病后七个月。一名63岁男性于2012年1月因三个月前右腿进行性肌无力、两个月前左手和右脚感觉迟钝以及一个月前双臂无力而入住我院。入院时的神经系统检查显示四肢不对称无力，左手和双脚有全身性反射消失和浅表感觉障碍。左内侧、右股和左腓深神经在临床上受到影响，尺神经、桡神经和坐骨神经也受到双侧影响。血细胞计数和生化包括自身抗体正常。脑脊液检查显示蛋白浓度升高（92 mg/dl），无白细胞增多（2/μl）。NCS显示在腕和腋窝之间的正中神经（图1 a）、腕和手肘上方之间的尺神经（图1 B）或踝和膝之间的腓神经和胫神经中没有脱髓鞘，例如暂时分散或传导阻滞，但是在双侧正中神经和左侧尺神经和胫神经中没有F波。双侧刺激Erb点未诱发复合肌肉动作电位（CMAP）。全脊柱MRI未见明显异常。2012年5月，左腿无力和感觉迟钝逐渐加重，患者无法独立行走。NCS显示前臂左侧正中神经（图1c）和尺神经（图1d）部分运动传导阻滞，双侧正中神经和左侧尺神经和胫神经无F波。根据欧洲神经学会联合会和周围神经学会（EFNS/PNS）标准，患者被诊断为MADSAM [1]。静脉注射免疫球蛋白（IVIG）治疗（400 mg/kg/天，持续5天）3次无效，但在静脉注射皮质类固醇脉冲治疗（甲泼尼龙1 g/天，持续3天）后，随后口服皮质类固醇（泼尼松龙60 mg/天），其症状显著改善（图2）。2012年7月，左侧正中神经部分出现F波。2013年3月，患者接受口服泼尼松龙7 mg/天治疗，可自行拄拐杖行走。在腋窝或膝盖以下的远端肢体中，高蛋白出血、F波缺失以及缺乏异常脱髓鞘发现提示脱髓鞘可能是
Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM)[1] is defined as an asymmetrical presentation of chronic inflammatory demyelinating polyneuropathy (CIDP). Nerve conduction studies (NCS) in MADSAM show characteristic demyelinating changes such as conduction block and temporal dispersion. We report a case of MADSAM with only F-waves absent three months after clinical onset of the disease and partial motor conduction blocks in the left forearm for the first time seven months after onset. A 63-year-old male was admitted to our hospital in January 2012 because of progressive muscle weakness of the right leg from three months before, dysesthesia of the left hand and the right foot from two months before, and bilateral weakness in the arms from one month before. Neurological examination on admission revealed asymmetrical weakness in all four limbs, with generalized areflexia and superficial sensory disturbance in the left hand and both feet. The left medial, right femoral, and left deep peroneal nerves were affected clinically, and bilateral effects were also observed in the ulnar, radial, and sciatic nerves. Blood cell counts and biochemistry including autoantibodies were normal. Cerebrospinal fluid examination showed an elevated protein concentration (92 mg/dl) without pleocytosis (2/μl). NCS showed no demyelination such as temporal dispersion or conduction block in the median nerve between the wrist and axilla (Fig. 1 a), the ulnar nerve between the wrist and above the elbow (Fig. 1 b), or the peroneal and tibial nerves between the ankle and knee, but F-waves were absent in the bilateral median and the left ulnar and tibial nerves. No compound muscle action potential (CMAP) was evoked with bilateral stimulation at Erb’s point. Whole spine MRI showed no remarkable abnormal findings. In May 2012, the weakness and dysesthesia in the left leg had increased gradually and the patient could not walk unassisted. NCS revealed partial motor conduction blocks in the left median (Fig. 1 c) and ulnar (Fig. 1 d) nerves in the forearm in addition to absent F-waves in the bilateral median and the left ulnar and tibial nerves. The patient was diagnosed with MADSAM based on the European Federation of Neurological Societies and the Peripheral Nerve Society (EFNS/PNS) criteria [1]. Intravenous immunoglobulin (IVIG) treatment (400 mg/kg/day for five days) administered three times was ineffective, but his symptoms improved dramatically after intravenous corticosteroid pulse therapy (methylprednisolone 1 g/day for three days) followed by oral corticosteroids (prednisolone 60 mg/day)(Fig. 2). In July 2012, F-waves appeared partially in the left median nerve. In March 2013, the patient was treated with oral prednisolone 7 mg/day and could walk with a cane by himself. Hyperproteinorrhachia, the absent F-waves, and the lack of abnormal demyelinating findings in the distal limbs below the axilla or knee suggest that demyelination might