急性無菌性髄膜脳炎の経過中に局所性皮質反射性ミオクローヌスを呈し抗グルタミン酸受容体抗体が検出された2例  [in Japanese] Two cases of acute onset of focal cortical reflex myoclonus following acute aseptic meningoencephalitis with positive anti-glutamate receptor autoantibody  [in Japanese]

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Author(s)

    • 人見 健文 Hitomi Takefumi
    • 京都大学医学部附属病院神経内科|京都大学大学院医学研究科呼吸管理睡眠制御学講座,臨床病態検査学講座 Department of Neurology, Kyoto University Hospital|Department of Respiratory Care and Sleep Control Medicine & Department of Clinical Laboratory Medicine, Kyoto University Graduate School of Medicine
    • 松本 理器 Matsumoto Riki
    • 京都大学医学部附属病院神経内科|京都大学大学院医学研究科てんかん・運動異常生理学講座 Department of Neurology, Kyoto University Hospital|Department of Epilepsy, Movement Disorders and Physiology, Kyoto University Graduate School of Medicine
    • 高橋 幸利 Takahashi Yukitoshi
    • 国立病院機構静岡てんかん・神経医療センター臨床研究部・小児科 National Epilepsy Center, Shizuoka institute of Epilepsy and Neurological Disorders
    • 池田 昭夫 Ikeda Akio
    • 京都大学医学部附属病院神経内科|京都大学大学院医学研究科てんかん・運動異常生理学講座 Department of Neurology, Kyoto University Hospital|Department of Epilepsy, Movement Disorders and Physiology, Kyoto University Graduate School of Medicine

Abstract

症例1(40歳男性),頭痛,発熱の軽快後に右下肢ミオクローヌスが出現し,クロナゼパム投与後軽快した.症例2(42歳男性),右下肢ミオクローヌス,発話停滞,全般強直間代発作が出現しステロイドパルス療法で一旦軽快も,症状再燃し,パルス療法を再度おこない軽快した.2例とも髄液蛋白と細胞数上昇,右脛骨神経刺激でC反射,脳波上筋放電に先行する頭蓋頂最大の棘波,頭部MRI上頭頂部に点状高信号域をみとめた.以上より急性髄膜脳炎経過中に局所性皮質反射性ミオクローヌスをきたしたと考えられた.血清・髄液中に抗グルタミン酸受容体ε2抗体が検出されたことより,自己免疫機序が病態に関与している可能性が示唆された.

Patient 1 was a 40-year-old man, who suffered from right leg myoclonus 1 week after an episode of fever and headache. Myoclonus disappeared 4 months after administration of clonazepam. Patient 2 was a 42-year-old man, who suffered from right leg myoclonus, attacks of speech arrest and a generalized tonic-clonic seizure. His symptoms disappeared after steroid-pulse therapy, but right leg myoclonus and episodic impairment of consciousness recurred within a month. He underwent another steroid-pulse therapy and his symptoms disappeared. In both patients, cerebrospinal fluid (CSF) study showed pleocytosis and elevated protein level, electrophysiological study showed cortical reflex by stimulation of the right tibial nerve, and brain MRI showed the high intensity area in the left parietal lobe. In addition, on electroencephalogram (EEG) spikes at vertex preceded myoclonic jerk of the right tibialis anterior muscle in both patients. These findings indicate that focal cortical reflex myoclonus was accompanied by acute central nervous system (CNS) infection. Furthermore, in both patients, autoantibody against glutamate receptor subunits ε2 was detected both in serum and CSF, which also suggest that autoimmune mechanism contributed in the pathophysiology of acute development of focal cortical reflex myoclonus.

Journal

  • Rinsho Shinkeigaku

    Rinsho Shinkeigaku 54(7), 543-549, 2014

    Societas Neurologica Japonica

Codes

  • NII Article ID (NAID)
    130004678995
  • NII NACSIS-CAT ID (NCID)
    AN00253207
  • Text Lang
    JPN
  • ISSN
    0009-918X
  • NDL Article ID
    025633153
  • NDL Call No.
    Z19-298
  • Data Source
    NDL  J-STAGE 
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