Autoimmune encephalitis in children and adolescents

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.

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.

With few exceptions, the experience in adult patients with autoimmune encephalitides can be applied to patients in the pediatric age range.