Autoimmune Encephalitis as a Rare Paraneoplastic Syndrome in Adrenocortical Carcinoma

Paraneoplastic neurologic syndromes (PNSs) are rare in pediatrics and are understood to be consequences of cross-reactivity against various neuroendocrine antigens expressed on cancer cells. Here, we report a case of autoimmune encephalitis, a type of PNS associated with a case of adrenocortical carcinoma (ACC), that had a clinical response to immunosuppressive therapy. ACC is a rare tumor with controversial tissue of origin but expresses various neuroendocrine antigens that could be the possible mechanism for this rare yet interesting association.