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Autoimmune Encephalitis: Clinical Features, Pathophysiology, and Treatment

Ramin Zand

Autoimmune encephalitis represents a group of disorders characterized by various immunologic mechanisms, clinical manifestations, and therapeutic outcomes. They can be associated with paraneoplastic syndromes or nonneoplastic autoimmune processes. Autoimmune encephalitis is usually associated with antibodies that can acutely or subacutely affect any part of the central or peripheral nervous system including neuromuscular junctions and muscles. Antibody-associated encephalitis can be divided into two main categories: (1) encephalopathy associated with antibodies against intracellular antigens and (2) encephalopathy associated with antibodies directed against the neuronal surface and synaptic antigens [1, 2]. The discovery of various antibodies related to autoimmune encephalitis has given us a new insight into pathogenic mechanisms and treatment of these syndromes. That is important since many patients with autoimmune encephalopathy are children and young adults, and they may respond well to immunosuppressive treatment if diagnosed without delay. In this chapter, we review the epidemiology, pathophysiology, clinical characteristics, diagnosis, and treatment of different syndromes associated with autoimmune encephalitis.

 
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