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多灶性运动神经病中的感觉丧失:一项临床和电生理研究。

Sensory loss in multifocal motor neuropathy: a clinical and electrophysiological study.

作者信息

Lambrecq Virginie, Krim Elsa, Rouanet-Larrivière Marie, Lagueny Alain

机构信息

Neurology Department, CHU Bordeaux, Hôpital Haut-Lévêque, Avenue de Magellan, 33604 Pessac, France.

出版信息

Muscle Nerve. 2009 Feb;39(2):131-6. doi: 10.1002/mus.21163.

Abstract

Some patients fulfilling the criteria for the diagnosis of multifocal motor neuropathy with conduction block (MMN-CB) at the onset of disease may subsequently develop a sensory loss associated with electrophysiological sensory abnormalities. The latter could represent an overlap between MMN-CB and multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy. The objective was to specify the features of MMN-CB with sensory loss (MMN-CB-Se). Five patients in a series of 11 consecutive patients who fulfilled the criteria of the American Association of Neuromuscular and Electrodiagnostic Medicine for MMN-CB at the first examination and were treated periodically with intravenous immunoglobulin (IVIg) developed sensory loss in the course of the disease. In these five patients we compared the clinical, laboratory, and electrophysiological features found after the development of sensory loss with those at the first examination. The mean time to appearance of objective sensory signs was 7.2 years. In three of the five patients the sensory loss was preceded by intermittent paresthesias in the same nerve territories as the motor involvement. The most frequent electrophysiological abnormality was amplitude reduction of sensory nerve action potentials. There were no bilateral or symmetrical clinical and electrophysiological sensory abnormalities. Anti-GM1 IgM antibodies were positive in four patients. MMN-CB-Se could be an overlap between MMN-CB and MADSAM. It shares the distribution of the sensory disorders encountered in MADSAM, but it is closer to MMN-CB on clinical and therapeutic levels. Study of more patients would be useful to classify this subgroup more accurately.

摘要

一些在疾病发作时符合多灶性运动神经病伴传导阻滞(MMN-CB)诊断标准的患者,随后可能会出现与电生理感觉异常相关的感觉丧失。后者可能代表MMN-CB与多灶性获得性脱髓鞘感觉和运动(MADSAM)神经病之间的重叠。目的是明确伴有感觉丧失的MMN-CB(MMN-CB-Se)的特征。在连续11例首次检查符合美国神经肌肉和电诊断医学协会MMN-CB标准并定期接受静脉注射免疫球蛋白(IVIg)治疗的患者中,有5例在疾病过程中出现了感觉丧失。在这5例患者中,我们比较了感觉丧失出现后发现的临床、实验室和电生理特征与首次检查时的特征。客观感觉体征出现的平均时间为7.2年。在这5例患者中的3例中,感觉丧失之前在与运动受累相同的神经区域出现间歇性感觉异常。最常见的电生理异常是感觉神经动作电位波幅降低。不存在双侧或对称的临床和电生理感觉异常。4例患者的抗GM1 IgM抗体呈阳性。MMN-CB-Se可能是MMN-CB与MADSAM之间的重叠。它具有MADSAM中所见感觉障碍的分布,但在临床和治疗层面上更接近MMN-CB。研究更多患者将有助于更准确地对该亚组进行分类。

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