Limbic Encephalitis and Variants: Classification, Diagnosis and Treatment

摘要: Background and Objective:Recent studies suggest that a substantial number of patients with autoimmune limbic encephalitis may improve if properly diagnosed treated. This is due, in part, to the increasing recognition disorders associate antibodies neuronal cell membrane antigens. review focuses these disorders, framed clinically useful immunologic classification encephalitis. Review Summary:Patients usually present rapidly progressive short-term memory deficits, psychiatric symptoms, seizures. After excluding viral systemic many (paraneoplastic or not) have cerebrospinal fluid inflammatory findings, EEG MRI abnormalities temporal lobes, antineuronal antibodies. These are directed against 2 broad categories antigens: (1) intracellular classic paraneoplastic antigens, including Hu, Ma2, CV2/CRMP5, amphiphysin among others, (2) voltage-gated potassium channels,N-methyl- D-aspartate receptor, others expressed neuropil hippocampus cerebellum (pending characterization). Whereas related first category cancer (lung, testis other), prominent brain infiltrates cytotoxic T-cells, limited response treatment, second less frequently (thymoma, teratoma), seem be antibodymediated, respond significantly better immunotherapy. Conclusions:Once considered an extremely rare disorder, almost always cancer, refractory now regarded as relatively frequent often unrelated clinical-immunologic variants treatment.