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J Neurol Neurosurg Psychiatry 83:476-479 doi:10.1136/jnnp-2011-301706
  • Neuromuscular disease
  • Research paper

Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinating polyneuropathy

  1. Satoshi Kuwabara
  1. Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan
  1. Correspondence to Dr S Kuwabara, Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan; kuwabara-s{at}faculty.chiba-u.jp
  1. Contributors SN, SM and SK designed the study and wrote the manuscript. YS, KS, KK, YF, SO, SaM and ShK collected and analysed the data.

  • Received 24 October 2011
  • Revised 19 December 2011
  • Accepted 4 January 2012
  • Published Online First 15 February 2012

Abstract

Background 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.

Methods 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.

Results 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.

Conclusions 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.

Footnotes

  • See Editorial commentary, p 474

  • Funding This work was supported in part by the Health and Labour Sciences Research Grant on Intractable Diseases (neuroimmunological diseases) from the Ministry of Health, Labour and Welfare of Japan (SK).

  • Competing interests None.

  • Ethics approval Ethics approval was provided by the ethics committee of Chiba University School of Medicine, Chiba, Japan.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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