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POEMS syndrome with Guillan–Barré syndrome-like acute onset: a case report and review of neurological progression in 30 cases
  1. S Isose1,
  2. S Misawa1,
  3. K Kanai1,
  4. K Shibuya1,
  5. Y Sekiguchi1,
  6. S Nasu1,
  7. Y Fujimaki1,2,
  8. Y Noto1,3,
  9. C Nakaseko4,
  10. S Kuwabara1
  1. 1Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan
  2. 2Department of Neurology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan
  3. 3Department of Neurology, Kyoto Prefectural Medical University, Kyoto, Japan
  4. 4Department of Haematology, Graduate School of Medicine, Chiba University, Chiba, Japan
  1. Correspondence to Professor Satoshi 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

Abstract

POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome is a rare cause of demyelinating neuropathy with monoclonal plasma cell proliferation, and POEMS neuropathy is usually chronically progressive. Herein, the authors report a 34-year-old woman with POEMS syndrome presenting as acute polyneuropathy. Within 2 weeks of disease onset, she became unable to walk with electrodiagnostic features of demyelination and was initially diagnosed as having Guillan–Barré syndrome. Other systemic features (oedema and skin changes) developed later, and an elevated serum level of vascular endothelial growth factor led to the diagnosis of POEMS syndrome. She received high-dose chemotherapy with autologous peripheral blood stem cell transplantation, resulting in good recovery. The authors also reviewed patterns and speed of progression of neuropathy in the 30 patients with POEMS syndrome; 22 (73%) of them were unable to walk independently with the median period of 9.5 months from POEMS onset (range 0.5–51 months). Whereas the speed of neuropathy progression varies considerably among patients, some POEMS patients can show acute or subacute polyneuropathy. The early diagnosis and treatment could result in rapid improvement as shown in the present patient.

  • POEMS syndrome
  • Guillan–Barré syndrome
  • polyneuropathy
  • demyelination
  • peripheral blood stem cell transplantation
  • clinical neurology
  • neuropathy
  • neurophysiology

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Footnotes

  • Funding This work was supported in part by the Health and Labour Sciences Research Grant on Intractable Diseases (Neuroimmunological Diseases) (SK) and the Research Grant 16B-1 for Nervous and Mental Disorders (SK) from the Ministry of Health, Labour and Welfare of Japan.

  • Competing interest None.

  • Ethics approval Ethics approval was provided by the Ethics Committee, Chiba University School of Medicine.

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

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