Role of antineuronal antibodies in children with encephalopathy and febrile status epilepticus.
Status epilepticus in childhood is more common, with a different range of causes and a lower risk of death, than convulsive status epilepticus in adults. Acute central nervous system infections appear to be markers for morbidity and mortality. Nevertheless, central nervous infection is usually presumed in these conditions. Many aspects of the pathogenesis of acute encephalitis and acute febrile encephalopathy with status epilepticus have been clarified in the past decade. The pathogenesis is divided into direct pathogens invasion or immune-mediated mechanisms. Over the past few decades, the number of antineuronal antibodies to ion channels, receptors, and other synaptic proteins described in association with central nervous system disorders has increased dramatically, especially their role in pediatric encephalitis and status epilepticus. These antineuronal antibodies are divided according to the location of their respective antigens: (1) intracellular antigens, including glutamic acid decarboxylase and classical onconeural antigens such as Hu (antineuronal nuclear antibody 1, ANNA1), Ma2, Yo (Purkinje cell autoantibody, PCA1), Ri (antineuronal nuclear antibody 2, ANNA2), CV2/CRMP5, and amphiphysin; and (2) cell membrane ion channels or surface antigens including voltage-gated potassium channel receptor, N-methyl-d-aspartate receptor, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, γ-aminobutyric acid(B) receptor, leucine-rich glioma-inactivated protein 1, and contactin-associated protein-like 2. Identifying the mechanism of the disease may have important therapeutic implications.
Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.
Kuang-Lin
KL
Lin
Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan. Electronic address: wanghs444@cgmh.org.tw.
Huei-Shyong
HS
Wang
D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D015415:Biomarkers; D001927:Brain Diseases / Q000276:immunology*; D002648:Child; D004660:Encephalitis / Q000276:immunology; D005968:Glutamate Decarboxylase / Q000276:immunology; D006801:Humans; D009419:Nerve Tissue Proteins; D009474:Neurons / Q000276:immunology*; D024642:Potassium Channels, Voltage-Gated; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology; D003294:Seizures, Febrile / Q000276:immunology*; D013226:Status Epilepticus / Q000276:immunology*
D001323:Autoantibodies; D015415:Biomarkers; D009419:Nerve Tissue Proteins; D024642:Potassium Channels, Voltage-Gated; D016194:Receptors, N-Methyl-D-Aspartate; C075637:amphiphysin; D005968:Glutamate Decarboxylase
antineuronal antibodies; childhood; encephalitis; status epilepticus