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A pediatric case of autoimmune encephalitis with chronologically moving seizure foci and cortical lesions: A case report
Affiliation:1. Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan;2. Department of Pediatrics, Tottori Prefectural Kosei Hospital, Kurayoshi, Japan;3. Department of Pediatrics, Tokyo Metropolitan Tobu Medical Center, Tokyo, Japan;4. National Epilepsy Center, NHO Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan
Abstract:
IntroductionAutoimmune encephalitis (AIE) is a relatively newly described category of immune-mediated diseases involving the central nervous system with a wide spectrum of clinical presentations, ranging from relatively mild or insidious onset of cognitive impairment to more complex forms of encephalopathy with medically refractory seizures. Single or multifocal seizures accompanied by neuropsychiatric symptoms and cognitive or memory impairments are suggestive of clinical features at AIE onset.Case reportA six-year-old boy presented with repetitive focal seizures, slowly progressive emotional liability, and attention-deficit/hyperactivity disorder-like symptoms. Seizure types varied during the clinical course, sometimes emerging as clusters or statuses. MRI performed during seizure clustering/status revealed moving signal abnormalities. We successfully treated the patient with high-dose intravenous methylprednisolone. Cerebrospinal fluid analysis revealed pleocytosis and marked elevation of antibodies against N-terminals of N-methyl-d-aspartate type glutamate receptor subunits and granzyme B.ConclusionWe report a case of moving seizure foci with abnormal MRI findings. Although the onset of psychiatric symptoms slowly progressed to those atypical for AIE, responsiveness to immunotherapy, cerebrospinal fluid pleocytosis, and autoantibodies all indicated AIE. We thus suggest that moving seizure foci and abnormal MRI signals may be findings of AIE.
Keywords:Autoimmune encephalitis  Autoantibodies  Glutamate receptors  Granzyme B  Moving seizure foci  Moving cerebral lesions  AIE"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_YMj9PKKIvt"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  autoimmune encephalitis  FIAS"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_GsGxd40nPY"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  focal impaired awareness seizures  ELISA"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_ZH83lz24me"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  enzyme-linked immunosorbent assay  FAS"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_jcGydl6qpU"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  focal aware seizure  NMDAR"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_EKxZEHdQIQ"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  N-methyl-D-aspartic acid receptor  GluR"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_WySGfmEVDM"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  glutamate receptor  MS"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_hUyOpNJTwU"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  multiple sclerosis  FSIQ"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_kgCkxovpPb"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  full-scale intelligence quotient  CSF"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_tgIxhKI8go"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  cerebrospinal fluid  WISC-Ⅳ"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_LX0oL5HtBU"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  wechsler intelligence scale for children-fourth edition  VCI"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_8mhgVg2BZ5"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  verbal comprehension index  PRI"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_0c13yqLS3M"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  perceptual reasoning index  WMI"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_zWRPz3QDOX"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  working memory index  PSI"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_3BVBLcbNSX"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  processing speed index  mPSL pulse"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_RniQOB7lFx"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  intravenous high-dose methylprednisolone  FLAIR"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_xCE0oACfKv"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  fluid-attenuated inversion recovery  ASL"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_6IZC6iqBoj"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  arterial spin labeling  GABAAR"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  pc_KxgX4Z6CXL"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  anti-c-aminobutyric acid type A receptor
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