Autoimmune_Diseases

D0000375

ESSO:Autoimmune_Diseases

Suleiman 2013 Criteria and supportive features to suspect autoimmune epilepsy in children with seizures: The following two clinical criteria are used to suspect autoimmune epilepsy associated with NSAbs and GAD antibodies (both are needed) 1 Acute or subacute (<12 weeks) onset of symptoms. 2 Exclusion of other causes (CNS infection, trauma, toxic, tumor, metabolic, previous CNS disease). The following supportive features would strengthen the suspicion of autoimmune epilepsy (patients should have at least 1 of the following): 1 The presence of a well-defined clinical syndrome such as NMDAR or limbic encephalitis 2 CNS inflammation manifested by at least one of: a CSF pleocytosis (defined as >5 white cells/mm3) or presence of oligoclonal bands, elevated IgG index, or elevated neopterin (defined as >30 nM) b MRI abnormality compatible with an inflammatory or autoimmune encephalitis including increased signal in the mesiotemporal lobe (LE – like syndrome) c Inflammatory neuropathology on biopsy 3 History of other antibody mediated condition (e.g., myasthenia gravis), organ specific autoimmunity or other autoimmune disorders. (It is recognized that epilepsy is more common in many autoimmune disorders including multiple sclerosis, systemic lupus erythematosus, type 1 diabetes mellitus (T1DM), celiac disease, and autoimmune thyroid disease (Vincent & Crino, 2011).) 4 Response to immunotherapy Classification categories of suspected autoimmune epilepsy in children identified using the criteria and supportive features in Table 1 (Zuliani et al., modified) Classification categories expressing the likelihood of autoimmune epilepsy based on the presence of NSAbs and GAD Abs and the response to immunotherapy (see Fig. 1): Definite autoimmune epilepsy is present if: Known NSAbs are present in serum or CSF AND there is response to immunotherapy Probable autoimmune epilepsy is present if Known NSAbs are present and no immunotherapy responsiveness demonstrated (immunotherapy unsuccessful or not given) OR GAD antibodies are present AND there is response to immunotherapy Possible autoimmune epilepsy is present if known NSAbs are negative and GAD antibodies are present and no immunotherapy responsiveness demonstrated (unsuccessful or not given) OR GAD antibodies are negative and there is a response to immunotherapy Unlikely autoimmune epilepsy is present if Known NSAbs and GAD are negative and there is no response to immunotherapy Unknown autoimmune epilepsy(a) is present if Known NSAbs and GAD are negative and immunotherapy is not given (a)Patients in this category may move to a different category if they receive immunotherapy, such as “possible” if they respond or “unlikely” if they did not respond to immunotherapy.