Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
J Neurol Neurosurg Psychiatry. 2012 May;83(5):476-9. doi: 10.1136/jnnp-2011-301706. Epub 2012 Feb 15.
POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome, a rare cause of demyelinating neuropathy associated with multiorgan involvement, has been increasingly recognised. Polyneuropathy is often an initial manifestation and therefore the disorder can be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP). Objective To elucidate whether POEMS syndrome and CIDP are differentiated based on profiles of neuropathy.
Clinical and electrophysiological data were reviewed in consecutive POEMS syndrome (n=51) and typical CIDP (n=46) patients in a single Japanese hospital between 2000 and 2010.
Both POEMS and CIDP patients showed symmetric polyneuropathy, physiological evidence of demyelination (70% of POEMS patients fulfilled the electrodiagnostic criteria for definite CIDP) and albuminocytological dissociation; 49% of the POEMS syndrome patients had neuropathy onset and 60% of them were initially diagnosed as having CIDP by neurologists. Clinically, POEMS neuropathy more frequently showed severe leg pain (76% vs 7%; p<0.001), muscle atrophy (52% vs 24%; p=0.005) and distal dominant muscle weakness. Electrophysiologically, POEMS syndrome was characterised by less prolonged distal motor latency (mean 5.6 ms vs 8.1 ms; p<0.001) and higher terminal latency index (0.42 vs 0.33; p=0.006) in the median nerves, and unrecordable tibial and sural responses (p<0.001), suggesting demyelination predominant in the nerve trunk rather than in the distal nerve terminals, and axonal loss in the lower limb nerves.
Before development of typical systemic manifestations, POEMS neuropathy can be distinguished from CIDP by the clinical profile and patterns of nerve conduction abnormalities. Recognition of these features leads to early diagnosis and proper treatment for POEMS syndrome.
POEMS（多发性神经病、器官肿大、内分泌病、M 蛋白和皮肤改变）综合征是一种罕见的脱髓鞘神经病病因，与多器官受累相关，其发病率逐渐增高。多发性神经病通常是该病的初始表现，因此可能会误诊为慢性炎症性脱髓鞘性多发性神经病（CIDP）。目的：明确 POEMS 综合征和 CIDP 是否可以通过神经病学表现进行区分。
回顾 2000 年至 2010 年期间，一家日本医院连续收治的 51 例 POEMS 综合征患者和 46 例典型 CIDP 患者的临床和电生理资料。
POEMS 患者和 CIDP 患者均表现为对称性多发性神经病、脱髓鞘的生理学证据（70%的 POEMS 患者符合明确 CIDP 的电诊断标准）和白蛋白细胞分离；49%的 POEMS 综合征患者首发神经病，60%的患者最初被神经科医生误诊为 CIDP。临床上，POEMS 神经病更常出现严重腿部疼痛（76%比 7%；p<0.001）、肌肉萎缩（52%比 24%；p=0.005）和远端优势肌肉无力。电生理学上，POEMS 综合征的特点是正中神经的远端运动潜伏期更短（平均 5.6ms 比 8.1ms；p<0.001）和末端潜伏期指数更高（0.42 比 0.33；p=0.006），以及胫神经和腓肠神经不可记录（p<0.001），提示神经干脱髓鞘更明显，而非远端神经末梢，下肢神经存在轴索丢失。
在出现典型的系统性表现之前，POEMS 神经病可根据临床表现和神经传导异常模式与 CIDP 相鉴别。认识到这些特征有助于早期诊断和适当治疗 POEMS 综合征。
