Preserved autonomic function in patients with POEMS syndrome Y. Fujinuma, M. Asahina ⁎, T. Fukushima, A. Katagiri, Y. Yamanaka, S. Misawa, S. Kuwabara Department of Neurology, School of Medicine, Chiba University, Chiba, Japan abstract article info Article history: Received 19 June 2011 Received in revised form 18 February 2012 Accepted 12 March 2012 Available online xxxx Keywords: POEMS syndrome Diabetic neuropathies Autonomic nervous system Cardiovascular function Sudomotor function Skin vasomotor function Aim: We systematically performed autonomic testing on patients with polyneuropathy, organomegaly, endo- crinopathy, M-protein and skin changes syndrome (POEMS) to determine whether autonomic function is preserved in such patients. Methods: We studied 17 POEMS patients, 17 diabetic neuropathy (DN) patients and 17 age-matched normal subjects. Blood pressure responses to the head-up tilt test and heart rate variability were used to evaluate cardiovascular autonomic function. Sweat responses and cutaneous vasoconstriction to several stimuli were recorded via the finger tips to estimate cutaneous sympathetic function. In addition, motor nerve con- duction studies were performed. Results: Although the results of the autonomic testing were normal in POEMS patients, motor disability was severe, and motor nerve conduction studies provided evidence of extensive axonal loss. The DN patients showed significantly impaired autonomic responses despite mild motor dysfunction. Conclusions: Autonomic function was normal in POEMS patients, indicating the preservation of autonomic fibers and selective involvement of large fibers. © 2012 Elsevier B.V. All rights reserved. 1. Introduction Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome (POEMS) is a disease that causes mixed axonal/ demyelinating polyneuropathy with multiple organ involvement and plasma cell dyscrasia. Its major clinical feature is chronic progressive polyneuropathy with predominant distal motor disability [1–3], which is presumably mediated by the overproduction of vascular endothelial growth factor [4]. POEMS syndrome is potentially fatal [2], and immedi- ate treatment, such as using high-dose chemotherapy with autologous peripheral blood stem cell transplantation or thalidomide therapy, is usually required [5,6]. However, early diagnosis is quite difficult, particu- larly when patients present with polyneuropathy without the associated systemic symptoms or signs such as hyperpigmentation of the skin, peripheral edema, hypertrichosis or organomegaly [7]. In POEMS, motor involvement follows the sensory symptoms which begin in the feet. Sen- sory and motor symptoms are distal, symmetric and progressive with a gradual proximal spread [7]. Information on the extent of autonomic involvement in this syndrome may be helpful for its early diagnosis. How- ever, no previous reports have focused on autonomic function in patients with POEMS. Therefore, we performed systematic autonomic testing in POEMS patients and compared the results with those in patients with diabetic neuropathy (DN). 2. Methods We prospectively studied 17 POEMS patients (13 men and 4 women; mean age, 54±9 years; mean disease duration, 3±3 years) in this study. They were consecutive patients who were referred to the Department of Neurology of Chiba University Hospital from 2005 to 2009. All patients fulfilled the published diagnostic criteria for POEMS that were proposed by Dispenzieri. Table 1 shows the clinical profiles of our POEMS patients. Seventeen patients with DN (11 men and 6 women; mean age, 60±10 years; mean disease duration of dia- betes, 13 ± 11 years) were included as disease controls. DN was defined by known diabetes mellitus criteria and the presence of symmetric sensory-dominant polyneuropathy [8]. The mean hemoglobin A1c levels in DN patients were 8.3%±1.6%. Seventeen healthy controls (13 men and 4 women; mean age, 55±9 years) were also evaluated. No participant received medications that could affect autonomic nervous system activity. Written informed consent was obtained from all patients. The ethics committee of Chiba University School of Medicine approved this study. Clinical motor disability was assessed using the following overall neuropathy limitation scale: a grade for each upper limb was scaled from 0 (normal) to 5 (most severe) and that for each lower limb was scaled from 0 (normal) to 7 (most severe) [9]. Motor nerve conduc- tion studies were performed on the tibial nerve using conventional pro- cedures with a lower limit of 5.6 mV for the normal range of compound motor action potentials (CMAPs) according to our laboratory data. Au- tonomic function tests were performed in a quiet room at an ambient temperature of 24–26 °C. Each subject was asked to relax, stay awake Journal of the Neurological Sciences xxx (2012) xxx–xxx ⁎ Corresponding author at: Department of Neurology, School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan. Tel.: + 81 43 226 2129; fax: +81 43 226 2160. E-mail address: asahina@faculty.chiba-u.jp (M. Asahina). JNS-12212; No of Pages 4 0022-510X/$ – see front matter © 2012 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2012.03.004 Contents lists available at SciVerse ScienceDirect Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns Please cite this article as: Fujinuma Y, et al, Preserved autonomic function in patients with POEMS syndrome, J Neurol Sci (2012), doi:10.1016/j.jns.2012.03.004