title: Intraepidermal nerve fibre density in POEMS (Crow-Fukase) syndrome and the correlation with sural nerve pathology.
abstract: OBJECTIVE
  To examine intraepidermal nerve fibre densities (IENFDs) in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin change (POEMS) syndrome.
  
  
  METHODS
  The IENFDs of 11 patients with POEMS syndrome were estimated. We determined whether IENFD was associated with patient clinical features or the estimated number of nerve fibres on complete cross-sections of biopsied sural nerves.
  
  
  RESULTS
  IENFD was significantly reduced (9.7±4.4fibres/mm) compared with normal controls (p<0.05), although the individual values varied from 1.4 to 14.4fibres/mm. The presence of glucose intolerance was significantly associated with a reduction of IENFD (p<0.05). The number of unmyelinated fibres was preserved at the sural nerve level and was not correlated with IENFD. In contrast, the number of myelinated fibres was correlated with IENFD (p<0.05).
  
  
  CONCLUSIONS
  Some of the patients presented with a severe IENFD reduction. Because the number of unmyelinated fibres was well preserved at the level of the sural nerve biopsy, this severe reduction may indicate involvement at the most distal nerve terminals of unmyelinated fibres. Although the reduction of IENFD becomes evident as polyneuropathy becomes severe, the effects of glucose intolerance should also be considered in patients with moderate to severe reductions.
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Ken
authors.initials: K
authors.lastname: Ohyama
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan. Electronic address: koike-haruki@med.nagoya-u.ac.jp.
authors.forename: Haruki
authors.initials: H
authors.lastname: Koike
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Rina
authors.initials: R
authors.lastname: Hashimoto
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Mie
authors.initials: M
authors.lastname: Takahashi
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Kawagashira
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Iijima
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Masahisa
authors.initials: M
authors.lastname: Katsuno
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan; Research Division of Dementia and Neurodegenerative Disease, Nagoya University Graduate School of Medicine, Nagoya, Japan. Electronic address: sobueg@med.nagoya-u.ac.jp.
authors.forename: Gen
authors.initials: G
authors.lastname: Sobue
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D024141:Collagen Type IV / Q000378:metabolism; D004817:Epidermis / Q000294:innervation* / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D036421:Nerve Fibers, Unmyelinated / Q000473:pathology*; D016878:POEMS Syndrome / Q000150:complications* / Q000473:pathology*; D013497:Sural Nerve / Q000473:pathology*; D043222:Ubiquitin Thiolesterase / Q000378:metabolism
chemical_list: D024141:Collagen Type IV; C482860:UCHL1 protein, human; D043222:Ubiquitin Thiolesterase
keywords: Glucose intolerance; Intraepidermal nerve fibre; POEMS syndrome; Skin biopsy; Sural nerve biopsy