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BRAIN and NERVE Volume 62, Issue 11 （November 2010）

BRAIN and NERVE 62巻11号（2010年11月発行）

Japanese English

症例報告

再発に伴い難治化した抗VGKC抗体陽性脳炎の1例 A Recalcitrant Case of Encephalitis with Voltage-gated Potassium Channel Antibodies 藤盛寿一 ¹ , 遠藤実 ¹ , 入野樹美 ² , 志賀裕正 ² , 白石裕一 ³ , 本村政勝 ³ , 丹野尚 ⁴ , 久永欣哉 ⁵ , 糸山泰人 ⁶ Juichi Fujimori ¹ , Minoru Endo ¹ , Shigemi Irino ² , Yusei Shiga ² , Hirokazu Shiraishi ³ , Masakatsu Motomura ³ , Takashi Tanno ⁴ , Kinya Hisanaga ⁵ , Yasuto Itoyama ⁶ ¹東北厚生年金病院神経内科 ²東北大学医学部神経内科 ³長崎大学医学部・歯学部附属病院（第一内科） ⁴松島病院内科 ⁵国立病院機構宮城病院神経内科 ⁶国立精神・神経医療研究センター病院 ¹Department of Neurology,Tohoku Employees' Pension Welfare Hospital ²Department of Neurology,Tohoku University School of Medicine ³The First Department of Internal Medicine,Nagasaki University Hospital of Medicine and Dentistry ⁴Department of Internal Medicine,Matsushima Hospital ⁵Department of Neurology,Miyagi National Hospital ⁶National Center Hospital,National Center of Neurology and Psychiatry キーワード： limbic encephalitis , voltage-gated potassium channel antibody , immuno-suppressive treatment , relapse , recalcitrant Keyword: limbic encephalitis , voltage-gated potassium channel antibody , immuno-suppressive treatment , relapse , recalcitrant pp.1252-1257

発行日 2010年11月1日 Published Date 2010/11/1

DOI https://doi.org/10.11477/mf.1416100791

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参考文献 Reference

はじめに

　電位依存性カリウムチャネル（voltage-gated potassium channel：VGKC）抗体陽性の辺縁系脳炎は免疫抑制療法によって改善することが多く，比較的予後のよい疾患とされてきたが^1），近年再発例の報告も散見される^2－6）。われわれは再発時に病巣部位が移動し，治療への抵抗性が増大して予後不良であった抗VGKC抗体陽性脳炎の症例を経験したので報告する。

Abstract

　We report the case of a 65-year-old man who had encephalitis with a high titer of voltage-gated potassium channel antibodies (VGKC-Abs).　His initial symptoms included memory disturbance, confusion, and seizures.　Laboratory tests revealed a low plasma sodium concentration and a strong positive result for VGKC-Abs.　A diffusion-weighted magnetic resonance imaging (MRI) scan showed a high intensity lesion within the right basal ganglia, which later showed normal intensity.　The patient's initial symptoms resolved without any treatment.　During the first relapse, the patient experienced consciousness disturbance and an increased number of seizures than that observed initially.　A diffusion weighted MRI scan showed a high intensity lesion within the right hippocampus, and a fluid attenuated inversion recovery (FLAIR) weighted MRI scan showed high intensity lesions within the right hippocampus, right thalamus, and pons.　The patient's symptoms and the MRI abnormalities resolved with prednisolone therapy.　During the second relapse, he again experienced consciousness disturbance and an increased number of seizures than that observed initially.　Diffusion-and FLAIR weighted MRI scans showed high intensity lesions within the right thalamus.　However, the array of immunosuppressive treatments used during the first relapse was not as effective during the second relapse.　The serum VGKC-Ab titers before steroid therapy during the first relapse and after immunosuppressive treatment during the second relapse were 1,252 pmol/L and 22.4 pmol/L, respectively.　Brain MRI revealed signal changes in the basal ganglia at the onset of disease, in the limbic area during the first relapse, and in the thalamus during the second relapse.　VGKC-Ab-associated encephalopathy is usually considered a benign autoimmune disorder; however, in our case, the encephalitis gradually became intractable to various immunosuppressive treatments, and unique MRI abnormalities were observed.

(Received：October 21,2009,Accepted：May 26,2010)