milder forms and cases associated with CNS potentially treatable syndrome, but may inflammatory lesions. respond to a variety of is compelling evidence suggesting the role immunomodulatory agents, including for IgG1 and IgG2 antibodies in binding to the IVIg. GluN1 subunit of the NMDA-receptor. A proportion of cases are associated with underlying teratomas and tumour removal may be curative. Limbic encephalitis — Treatment thus consists of immunotherapy and nonparaneoplastic tumour resection. First line immunotherapy typically includes intravenous There appears to be a role for IVIg in methylprednisolone and IVIg or plasmapheresis. nonparaneoplastic limbic encephalitis Due to the behavioural and/or autonomic associated with neuronal antibodies to cell manifestations of the disease, plasmapheresis, surface antigens. This includes VGKC with large bore catheters may be clinically antibodies, as well as NMDA receptor inappropriate. Second line treatment includes antibodies and AMPA receptor rituximab and cyclophosphamide. The consensus antibodies. opinion is that one would progress to the addition of second line treatment in a standard Limbic encephalitis paraneoplastic — case if no clinical improvement is observed after approximately two weeks of first line therapy and IVIg may be indicated in select cases, in no tumour is found. combination with tumour therapy (tumour resection and/or oncological are a variety of rarer neuroimmunological treatment) where the latter has not led syndromes for which there is good evidence of to an improvement in the neurologic antibodies binding physiologically relevant neuronal surface antigens with a case literature syndrome; where other describing responses to immunotherapy often immunomodulatory therapies are
potentially treatable syndrome, but may respond to a variety of immunomodulatory agents, including IVIg. Limbic encephalitis — nonparaneoplastic There appears to be a role for IVIg in nonparaneoplastic limbic encephalitis associated with neuronal antibodies to cell surface antigens. This includes VGKC antibodies, as well as NMDA receptor antibodies and AMPA receptor antibodies. Limbic encephalitis — paraneoplastic IVIg may be indicated in select cases, in combination with tumour therapy (tumour resection and/or oncological treatment) where the latter has not led to an improvement in the neurologic syndrome; where other immunomodulatory therapies are milder forms and cases associated with CNS inflammatory lesions. is compelling evidence suggesting the role for IgG1 and IgG2 antibodies in binding to the GluN1 subunit of the NMDA-receptor. A proportion of cases are associated with underlying teratomas and tumour removal may be curative. Treatment thus consists of immunotherapy and tumour resection. First line immunotherapy typically includes intravenous methylprednisolone and IVIg or plasmapheresis. Due to the behavioural and/or autonomic manifestations of the disease, plasmapheresis, with large bore catheters may be clinically inappropriate. Second line treatment includes rituximab and cyclophosphamide. The consensus opinion is that one would progress to the addition of second line treatment in a standard case if no clinical improvement is observed after approximately two weeks of first line therapy and no tumour is found. are a variety of rarer neuroimmunological syndromes for which there is good evidence of antibodies binding physiologically relevant neuronal surface antigens with a case literature describing responses to immunotherapy often
autoimmune-encephalitis-mediated-by-antibodies-targeting-cell-surface-antigens_0.DOCX