Autoimmune encephalitis in children and adolescents
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(2020) 2:4
Neurological Research and Practice
REVIEW
Open Access
Autoimmune encephalitis in children and adolescents C. G. Bien1,2* and C. I. Bien2
Abstract Background: Autoimmune encephalitides with neural and glial antibodies have become an attractive field in neurology because the antibodies are syndrome-specific, explain the pathogenesis, indicate the likelihood of an underlying tumor, and often predict a good response to immunotherapy. The relevance and the management of antibody-associated encephalitides in the pediatric age group are to be discussed. Main body: Subacutely evolving, complex neuropsychiatric conditions that are otherwise unexplained should raise the suspicion of autoimmune encephalitis. Determination of autoantibodies is the key diagnostic step. It is recommended to study cerebrospinal fluid and serum in parallel to yield highest diagnostic sensitivity and specificity. The most frequently found antibodies are those against the N-methyl-D-asparate receptor, an antigen on the neural cell surface. The second most frequent antibody is directed against glutamic acid decarboxylase 65 kDa, an intracellular protein, often found in chronic conditions with questionable inflammatory activity. Immunotherapy is the mainstay of treatment in autoimmune encephalitides. Steroids, apheresis and intravenous immunoglobulin are first-line interventions. Rituximab or cyclophosphamide are given as second-line treatments. Patients with surface antibodies usually respond well to immunotherapy whereas cases with antibodies against intracellular antigens most often do not. Conclusion: With few exceptions, the experience in adult patients with autoimmune encephalitides can be applied to patients in the pediatric age range.
Background The discovery of immunoglobulin G (IgG) antibodies against proteins on nerve cell surfaces has been perceived as a major advance and even a breakthrough in neurology. The first specific antibodies that have relevance and validity until to date are those against the Nmethyl-D-aspartate receptor (NMDAR) [1]. These were followed by those against leucine-rich glioma inactivated protein 1 (LGI1) [2, 3], contactin-associated protein-2 (CASPR2) [3, 4] and others. These new “surface antibodies” are diagnosed by incubating diluted serum or undiluted (or mildly diluted) cerebrospinal fluid (CSF) with human embryonic kidney (HEK) cells transfected with the antigens of interest [5]. The binding of antibodies is visualized by a secondary anti-human IgG
* Correspondence: [email protected] 1 Epilepsy Center Bethel, Krankenhaus Mara, Maraweg 17-21, 33617 Bielefeld, Germany 2 Laboratory Krone, Bad Salzuflen, Germany
antibody coupled to a dye that can be visualized under the microscope. The combination of four properties makes these autoantibodies so valuable: – their syndrome specificity, – their pathogenetic explanatory power, – the frequently good treatability of the associated autoimmune central nervous system (CNS) syndromes – the indication of the underlying tumor probability (paraneoplastic
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