title: Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinating polyneuropathy.
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.
authors.affiliation: Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
authors.forename: Saiko
authors.initials: S
authors.lastname: Nasu
authors.forename: Sonoko
authors.initials: S
authors.lastname: Misawa
authors.forename: Yukari
authors.initials: Y
authors.lastname: Sekiguchi
authors.forename: Kazumoto
authors.initials: K
authors.lastname: Shibuya
authors.forename: Kazuaki
authors.initials: K
authors.lastname: Kanai
authors.forename: Yumi
authors.initials: Y
authors.lastname: Fujimaki
authors.forename: Shigeki
authors.initials: S
authors.lastname: Ohmori
authors.forename: Satsuki
authors.initials: S
authors.lastname: Mitsuma
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Koga
authors.forename: Satoshi
authors.initials: S
authors.lastname: Kuwabara
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction / Q000502:physiology; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis* / Q000503:physiopathology