Development of new treatments for Crow-Fukase syndrome

• Project/Area Number: 20590988
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Single-year Grants
• Section: 一般
• Research Field: Neurology
• Research Institution: Chiba University
• Principal Investigator: KUWABARA Satoshi Chiba University, 大学院・医学研究院, 教授 (70282481)
• Co-Investigator(Kenkyū-buntansha): MORI Masahiro 千葉大学, 大学院・医学研究院, 講師 (70345023) ; KANAI Kazuaki 千葉大学, 医学部附属病院, 助教 (10375751)
• Project Period (FY): 2008 – 2010
• Project Status: Completed (Fiscal Year 2010)
• Budget Amount: ¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000); Fiscal Year 2010: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000); Fiscal Year 2009: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000); Fiscal Year 2008: ¥2,990,000 (Direct Cost: ¥2,300,000、Indirect Cost: ¥690,000)
• Keywords: クロウ・深瀬症候群 / 自己末梢血幹細胞移植 / サリドマイド / 神経免疫学 / 神経病態免疫学

Research Abstract

POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome is potentially fatal disease, and patients' quality of life deteriorates because of progressive neuropathy. The aim of this study was to clarify the effects of (1) high-dose chemotherapy with autologous peripheral blood stem cell transplantation (PBSCT) and thalidomide therapy. PBSCT was performed in 21 patients under age 66 years, and resulted in obvious improvement in neuropathy as well as other symptoms. Fifteen patients aged 66 years or older received thalidomide, resulting in moderate clinical improvement (n=12) and stabilization of symptoms (n=3). Based on the findings, we proposed a treatment guideline with PBSCT or thalidomide therapy according to patients' age.

Research Products

• [Journal Article] Crow-Fukase (POEMS) syndrome. (2010)
  • Author(s): Kuwabara S.
  • Journal Title: Brain Nerve. 62(4) Pages: 395-400
  • NAID: 40017067161
• [Journal Article] Advances and perspectives in treatment for refractory neuropathies with special reference to immune-mediated neuropathies and Crow-Fukase syndrome. (2010)
  • Author(s): Kuwabara S.
  • Journal Title: Rinsho Shinkeigaku. 50(4) Pages: 219-224
• [Journal Article] Crow-Fukase症候群 (2010)
  • Author(s): 桑原聡
  • Journal Title: BRAIN and NERVE神経研究の進歩 Volume: 62 Pages: 395-400
• [Journal Article] 難治性末梢神経疾患の治療と展望:免疫性ニューロパチーとCrow-Fukase症候群を中心に (2010)
  • Author(s): 桑原聡
  • Journal Title: 臨床神経学 Volume: 50 Pages: 219-224
  • Peer Reviewed
• [Journal Article] Relapse of POEMS syndrome without increased level of VEGF. (2009)
  • Author(s): Kuwabara S, Kanai K, Misawa S, Nakaseko C.
  • Journal Title: Neuromuscul Disord 19(10) Pages: 740-741
• [Journal Article] Successful combination treatment with bevacizumab, thalidomide and autologous PBSC for severe POEMS syndrome. (2009)
  • Author(s): Ohwada C, Nakaseko C, Sakai S, Takeda Y, Abe D, Takeuchi M, Sakaida E, Masuda S, Shimizu N, Cho R, Nishimura M, Kanai K, Misawa S, Kuwabara S.
  • Journal Title: Bone Marrow Transplant 43(9) Pages: 739-740
• [Journal Article] Relapse of POEMS syndrome without increased level of VEGF. (2009)
  • Author(s): Kuwabara S, Kanai K, Misawa S, Nakaseko C.
  • Journal Title: Neuromuscular Disorders 19 Pages: 740-741
  • Peer Reviewed
• [Journal Article] Successful combination treatment with bevacizumab, thalidomide and autologous PBSC for severe POEMS syndrome. (2009)
  • Author(s): Ohwada C, Nakaseko C, Sakai S, Takeda Y, Abe D, Takeuchi M, Sakaida E, Masuda S, Shimizu N, Cho R, Nishimura M, Kanai K, Misawa S, Kuwabara S.
  • Journal Title: Bone Marrow Transplantation 43 Pages: 739-740
  • Peer Reviewed
• [Journal Article] Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome (2009)
  • Author(s): Kuwabara S, Dispenzieri A, Arimura K, Misawa S.
  • Journal Title: Cochrane Database Systematic Review (印刷中)
  • Peer Reviewed
• [Journal Article] Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. (2008)
  • Author(s): Kuwabara S, Dispenzieri A, Arimura K, Misawa S.
  • Journal Title: Cochrane Database Syst Rev 4
• [Journal Article] Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome. (2008)
  • Author(s): Kuwabara S, Misawa S, Kanai K, Suzuki Y, Kikkawa Y, Sawai S, Hattori T, Nishimura M, Nakaseko C.
  • Journal Title: Neurology 71 Pages: 1691-1695
• [Journal Article] New strategy of treatment for POEMS syndrome-autologous peripheral blood stem cell transplantation and thalidomide therapy. (2008)
  • Author(s): Kuwabara S.
  • Journal Title: Brain Nerve 60(6) Pages: 627-633
  • NAID: 40016092414
• [Journal Article] Restrictive usage of monoclonal immunoglobulin lambda light chain germline in POEMS syndrome. (2008)
  • Author(s): Abe D, Nakaseko C, Takeuchi M, Tanaka H, Ohwada C, Sakaida E, Takeda Y, Oda K, Ozawa S, Shimizu N, Masuda S, Cho R, Nishimura M, Misawa S, Kuwabara S, Saito Y.
  • Journal Title: Blood 112(3) Pages: 836-839
• [Journal Article] Thalidomide reduces serum VEGF levels and improves peripheral neuropathy in POEMS syndrome. (2008)
  • Author(s): Kuwabara S, Misawa S, Kanai K, Sawai S, Hattori T, Nishimura M, Nakaseko C.
  • Journal Title: J Neurol Neurosurg Psychiatry 79(11) Pages: 1255-1257
• [Journal Article] Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome (2008)
  • Author(s): Kuwabara S, Misawa S, Kanai K, et al.
  • Journal Title: Neurology 71 Pages: 1691-1695
  • Peer Reviewed
• [Journal Article] Thalidomide reduces serum VEGF levels and improves peripheral neuropathy in POEMS syndrome (2008)
  • Author(s): Kuwabara S, Misawa S, Kanai K, et al.
  • Journal Title: Journal of Neurology, Neurosurgery, and Psychiatry 79 Pages: 1255-1257
  • Peer Reviewed
• [Journal Article] POEMS syndrome with Guillan-Barre syndrome-like acute onset : a case report and review of neurological progression in 30 cases.
  • Author(s): Isose S, Misawa S, Kanai K, Shibuya K, Sekiguchi Y, Nasu S, Fujimaki Y, Noto Y, Nakaseko C, Kuwabara S.
  • Journal Title: J Neurol Neurosurg Psychiatry (in press)
• [Journal Article] POEMS syndrome with Guillan-Barre syndrome-like acuteonset : a case report and review of neurological progression in 30 cases
  • Author(s): Isose S, Misawa S, Kanai K, Shibuya K, Sekiguchi Y, Nasu S, Fujimaki Y, Noto Y, Nakaseko C, Kuwabara S.
  • Journal Title: J Neurol Neurosurg Psychiatry Volume: 印刷中
  • Peer Reviewed
• [Presentation] Crow-Fukase症候群における発症様式の多様性 (2010)
  • Author(s): 磯瀬沙希里、桑原聡, ら
  • Organizer: 第51回日本神経学会総会
  • Place of Presentation: 東京
  • Year and Date: 2010-05-22
• [Presentation] Crow-Fukase症候群における疾患活動性マーカーとしてのVEGFの意義 (2010)
  • Author(s): 三澤園子、桑原聡, ら
  • Organizer: 第51回日本神経学会総会
  • Place of Presentation: 東京
  • Year and Date: 2010-05-22
• [Presentation] Crow-Fukase症候群の電気診断 (2010)
  • Author(s): 那須彩子、桑原聡, ら
  • Organizer: 第51回日本神経学会総会
  • Place of Presentation: 東京
  • Year and Date: 2010-05-21
• [Presentation] Crow-Fukase症候群の新規治療展望 (2010)
  • Author(s): 桑原聡
  • Organizer: 第51回日本神経学会総会
  • Place of Presentation: 東京
  • Year and Date: 2010-05-20
• [Presentation] Crrow-Fukase症候群の新規治療展望 (2010)
  • Author(s): 桑原馨
  • Organizer: 第51回日本神経学会総会(教育講演)
  • Place of Presentation: 東京
  • Year and Date: 2010-05-20
• [Presentation] Crow-Fukase症候群の新規治療 (2009)
  • Author(s): 桑原聡、三澤園子、金井数明
  • Organizer: 第50回日本神経学会総会
  • Place of Presentation: 仙台
  • Year and Date: 2009-05-21
• [Presentation] Crow-Fukase症候群に対する新規治療 (2008)
  • Author(s): 桑原聡、三澤園子、金井数明
  • Organizer: 第49回日本神経学会総会
  • Place of Presentation: 横浜
• [Remarks]
  • URL: http://www.m.chiba-u.jp/class/neurol/