A case of acute encephalitis with refractory, repetitive partial seizures, presenting autoantibody to glutamate receptor Gluε2


      An 11-year-old male was admitted to our hospital because of high-grade fever, repetitive seizures, and prolonged impairment of consciousness (Glasgow coma scale E1, M5, V1). His seizures were repetitive complex partial seizures that expanded from the unilateral face to the corresponding side of the body. He sometimes developed secondary generalized seizures. While most seizures lasted 1 or 2 min, intractable seizures also frequently (about 5 times/h) occurred. We diagnosed him as encephalitis/encephalopathy, and treated him with artificial respiration, thiamylal sodium, mild hypothermia therapy, steroid pulse therapy, massive γ-globulin therapy, etc. Afterwards, he had sequelae, such as post-encephalitic epilepsy (same seizures continued to recur), hyperkinesia, impairment of immediate memory, change in character (he became sunny and obstinate), dysgraphia, and mild atrophy of the hippocampus, amygdala, and cerebrum. However, he could still attend a general junior high school. He was diagnosed as acute encephalitis with refractory, repetitive partial seizures (AERRPS). In this case, he was positive for autoantibody to glutamate receptor Gluε2 IgG or IgM in an examination of blood and spinal fluid, and we presumed that this may have influenced his sequelae. In this case, a combination of mild hypothermia therapy, steroid pulse therapy, and massive γ-globulin therapy was effective.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Brain and Development
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Awaya Y.
        • Fukuyama Y.
        Epilepsy sequelae of acute encephalitis or encephalopathy (third report).
        Jpn J Psychiatry Neurol. 1986; 40: 385-387
        • Sakuma H.
        • Fukumizu M.
        • Kohyama J.
        Efficacy of anticonvulsants on acute encephalitis with refractory, repetitive partial seizures (AERRPS) (in Japanese).
        No To Hattatsu (Tokyo). 2001; 33: 385-390
        • Rogers S.W.
        • Andrews P.I.
        • Gahring L.C.
        • Whisenand T.
        • Cauley K.
        • Crain B.
        • et al.
        Autoantibodies to glutamate receptor GluR3 in Rasmussen's encephalitis.
        Science. 1994; 265: 648-651
        • Hamano K.
        • Watanabe A.
        • Kohyama J.
        A case of acute encephalitis with refractory, repetitive partial seizures (AERRPS) showing transient disappearance of the seizure with occurrence of choreo-ballistic movement (in Japanese).
        No To Hattatsu (Tokyo). 2003; 35: 59-64
        • Chase T.N.
        • Oh J.D.
        Striatal dopamine- and glutamate-mediated dysregulation in experimental parkinsonism.
        Trends Neurosci. 2000; 23: 586-591
        • Kimura S.
        • Ohtsuki N.
        • Adachi K.
        • Nezu A.
        • Aihara Y.
        Efficacy of methylprednisolone pulse and mild hypothermia therapies in patients with acute encephalopathy (in Japanese).
        No To Hattatsu (Tokyo). 2000; 32: 62-67
        • Munakata M.
        • Kato R.
        • Yokoyama H.
        • Haginoya K.
        • Tanaka Y.
        • Kayaba J.
        • et al.
        Combined therapy with hypothermia and anticytokine agents in influenza A encephalopathy.
        Brain Dev. 2000; 22: 373-377
        • Ichiyama T.
        • Nishikawa M.
        • Yoshitomi T.
        • Hayashi T.
        • Furukawa S.
        Tumor necrosis factor-alpha, interleukin-1beta, and interleukin-6 in cerebrospinal fluid from children with prolonged febrile seizures. Comparison with acute encephalitis/encephalopathy.
        Neurology. 1998; 50: 407-411
        • Kimura S.
        • Ohtsuki N.
        • Adachi K.
        • Nezu A.
        Marked efficacy of steroid pulse therapy in an infant with acute encephalopathy having elevated cerebrospinal fluid level of neopterin (in Japanese).
        No To Hattatsu (Tokyo). 1999; 31: 282-283
        • Sandstedt P.
        • Kostulas V.
        • Larsson L.E.
        Intravenous gammaglobulin for post-encephalitic epilepsy.
        Lancet. 1984; 2: 1154-1155
        • Takahashi Y.
        • Sakaguchi N.
        • Kondo N.
        • Ariki M.
        • Hattori S.
        • Matsuo N.
        • et al.
        Investigation about diagnosis and therapy of higher brain function disorders by autoantibodies against NMDA-GluRs.
        in: Kaga M. (in Japanese) Development and Developmental Dysfunction of Higher Cortical Function, (Report of Research Grant (12B-2) for Nervous and Mental Disorders from the Ministry of Health, Labor and Welfare) (Ichikawa). 2003: 93-99