Asymmetric Acquired Demyelinating Polyneuropathies: MMN and MADSAM.
Asymmetric Acquired Demyelinating Polyneuropathies: MMN and MADSAM.
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DOI：
10.1007/s11940-001-0046-1
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发表时间：
2001-03-01
影响因子：
2
通讯作者：
Saperstein, David S.
中科院分区：
文献类型：
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作者：
Katz, Jonathan S.;Saperstein, David S.
在Austin首次描述慢性炎性脱髓鞘性多发性神经根神经病（CIDP）后的半个多世纪，慢性获得性脱髓鞘性多发性神经病的临床谱已经扩大。目前，有许多实体可以根据不同的临床表现放在慢性获得性脱髓鞘神经病（CADP）的标题下。在该方案中，CIDP仅用于指脱髓鞘神经病和全身对称性无力的患者。相比之下，多灶性运动神经病（MMN）和多灶性获得性脱髓鞘感觉和运动神经病（MADSAM）属于CADP的不对称、多灶性形式。它们之间的区别仅在于是否存在感官的参与。我们认为，将这些临床表现分为不同的实体是有实际原因的。虽然这些临床亚型中的每一种都有一些基本的相似之处，但也有重要的差异。MMN通常被认为对皮质类固醇治疗具有抗性，并且这种疾病的一线药物是静脉内免疫球蛋白（IVIg）。MADSAM神经病变可对泼尼松或IVIg有反应，并且在实验室特征和治疗反应方面与经典CIDP更相似。
More than a half a century after Austin's initial description of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), the clinical spectrum of chronic acquired demyelinating polyneuropathies has expanded. Currently there are a number of entities that can be put under the heading of chronic acquired demyelinating neuropathy (CADP) based on differing clinical presentations. In this scheme, CIDP is used only to refer to patients with demyelinating neuropathies and generalized symmetric weakness. In contrast, multifocal motor neuropathy (MMN) and multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) fall into the category of asymmetrical, multifocal forms of CADP. These are distinguished from each other only by the presence of sensory involvement. In our opinion, there are pragmatic reasons for splitting these clinical presentations into distinct entities. Although each of these clinical subtypes shares some basic similarities, there are important differences. MMN is usually considered resistant to corticosteroid therapy and the first line agent in this disorder is intravenous immunoglobulin (IVIg). MADSAM neuropathy can be responsive to prednisone or IVIg, and has a profile more analogous to classic CIDP with regards to its laboratory features and treatment response.