Analysis of clinical-immunological feature of limbic encephalitis as Hashimoto's encephalopathy

• Project/Area Number: 26860663
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Neurology
• Research Institution: University of Fukui
• Principal Investigator: MATSUNAGA AKIKO 福井大学, 医学部, 助教 (40401971)
• Project Period (FY): 2014-04-01 – 2016-03-31
• Project Status: Completed (Fiscal Year 2015)
• Budget Amount: ¥3,510,000 (Direct Cost: ¥2,700,000、Indirect Cost: ¥810,000); Fiscal Year 2015: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000); Fiscal Year 2014: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
• Keywords: 橋本脳症 / 辺縁系脳炎 / 抗NAE抗体 / 自己抗体 / 間接免疫蛍光法 / 自己免疫性脳症 / 免疫組織蛍光染色

Outline of Final Research Achievements

We examined 14 patients with limbic encephalitis (LE) and suspected Hashimoto’s encephalopathy (HE) based on positive tests for anti-thyroid antibodies and anti-NH2-terminal of α-enolase (NAE) antibodies, a diagnostic marker for HE. The median age was 62.5 (20-83) years; 9 were women; 8 showed acute onset. Consciousness disturbance and seizures were commonly observed in the acute-onset group, whereas psychiatric symptoms and memory disturbance were common in the subacute-onset group. Tumours were not identified in any cases. All patients responded to immunotherapy or spontaneously remitted. Favourable therapeutic efficacy suggests that this LE can be considered a clinical subtype of HE and that anti-NAE antibodies may be a promising indicator of the need for immunotherapy.
The patients’ sera reacted with the cytoplasm of hippocampal neurons. Immunoreactivity of the patients’ sera suggests that anti-NAE antibodies may contribute to the pathogenesis of the LE.

Research Products

• [Journal Article] Dementia due to Endocrine Diseases (2016)
  • Author(s): 松永晶子、米田誠
  • Journal Title: BRAIN and NERVE Volume: 68 Issue: 4 Pages: 399-405
  • DOI: 10.11477/mf.1416200412
  • ISSN: 1344-8129, 1881-6096
  • Year and Date: 2016-04-01
• [Journal Article] 橋本脳症 (2015)
  • Author(s): 松永晶子、米田誠
  • Journal Title: 日本臨床 Volume: 別冊免疫症候群I Pages: 98-101
• [Journal Article] Consensus Paper: NeuroimmuneMechanisms of Cerebellar Ataxias (2015)
  • Author(s): Mitoma H, Adhikari K, Aeschlimann D, Chattopadhyay P, Hadjivassiliou M, Hampe CS, Honnorat J, Joubert B, Kakei S, Lee J, Manto M, Matsunaga A, Mizusawa H,Nanri K, Shanmugarajah P, Yoneda M, Yuki N.
  • Journal Title: Cerebellum Volume: on line Issue: 2 Pages: 213-32
  • DOI: 10.1007/s12311-015-0664-x
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] 橋本脳症 (2015)
  • Author(s): 松永晶子，米田誠
  • Journal Title: 精神科 Volume: 26
• [Journal Article] Presynaptic dysfunction caused by CSF from a patient with ataxic form of Hashimoto's encephalopathy. (2014)
  • Author(s): Mitoma H, Yoneda M, Saitou F, Suzuki H, Matsunaga A, Ikawa M, Mizusawa H.
  • Journal Title: Neurol Clin Neurosci Volume: 2 Pages: 104-108
  • Peer Reviewed
• [Presentation] 抗NAE抗体陽性辺縁系脳炎型橋本脳症19例の臨床的特徴 (2015)
  • Author(s): 岸谷 融，松永 晶子，井川 正道，林 浩嗣，山村 修，濱野 忠則，渡辺 修，田中惠子，米田 誠
  • Organizer: 第56回日本神経学会学術大会
  • Place of Presentation: 新潟、朱鷺メッセ
  • Year and Date: 2015-05-20
• [Presentation] Clinical and immunological features of limbic form of Hashimoto’s encephalopathy. (2015)
  • Author(s): Toru Kishitani, Akiko Matsunaga, Masamichi Ikawa, Tomomi Kame, Osamu Yamamura, Tadanori Hamano, Yasunari Nakamoto, Makoto Yoneda. Ap
  • Organizer: The 67th Annual Meeting of American Academy of Neurology
  • Place of Presentation: ワシントン（米国）
  • Year and Date: 2015-04-18
  • Int'l Joint Research
• [Presentation] Pathophysiological Decrease in the Regional Cerebral Blood Flow in Hashimoto’s Encephalopathy: A Multiple-Case SPECT Study. (2015)
  • Author(s): Masamichi Ikawa, Tomoko Muramatsu, Katsuya Sugimoto, Akiko Matsunaga, Osamu Yamamura, Tadanori Hamano, Yasunari Nakamoto, Hidehiko Okazawa, Makoto Yoneda.
  • Organizer: The 67th Annual Meeting of American Academy of Neurology
  • Place of Presentation: ワシントン（米国）
  • Year and Date: 2015-04-18
  • Int'l Joint Research
• [Presentation] Clinical and immunological features of Hashimoto’s encephalopathy presenting with limbic symptoms（フィラデルフィア、ポスター） (2014)
  • Author(s): Matsunaga A, Kishitani T, Muramatsu T, Kame T, Ikawa M, Nakamoto Y, Yoneda M
  • Organizer: The 66th American academy of Neurology Annual Meeting
  • Place of Presentation: フィラデルフィア（米国）
  • Year and Date: 2014-04-28 – 2014-05-03
• [Book] Neuroimmunological Diseases (2016)
  • Author(s): Makoto Yoneda, Akiko Matsunaga, Masamichi Ikawa.
  • Publisher: Springer, NY, USA