A case of acute encephalitis with refractory, repetitive partial seizures, presenting autoantibody to glutamate receptor Gluepsilon2.
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 gamma-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 Gluepsilon2 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 gamma-globulin therapy was effective.
Ito
Hiromichi
H
Department of Pediatrics, Institute of Health Biosciences, The University of Tokushima Graduate School, 3-18-15, Kuramoto-Cho, Tokushima 770-8503, Japan. moriken@clin.med.tokushima-u.ac.jp
Mori
Kenji
K
Toda
Yoshihiro
Y
Sugimoto
Mayumi
M
Takahashi
Yukitoshi
Y
Kuroda
Yasuhiro
Y
D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D002648:Child; D004569:Electroencephalography; D004660:Encephalitis / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D008297:Male; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D012640:Seizures; D005719:gamma-Globulins / Q000627:therapeutic use
D001323:Autoantibodies; C121001:NR2B NMDA receptor; D016194:Receptors, N-Methyl-D-Aspartate; D005719:gamma-Globulins