Abstract
The understanding of the manifestations, mechanisms, and management of autoimmune
encephalitis has expanded dramatically in recent decades. Immune-mediated encephalitides
are comparable in incidence and prevalence to infectious etiologies, and are associated
with significant morbidity, especially when there is a delay in recognition and treatment.
As such, clinicians from many specialties must develop a functional understanding
of these disorders. Herein we provide an overview of the autoimmune and paraneoplastic
encephalitides, including those associated with either intracellular or cell surface/synaptic
neuronal autoantibodies. After briefly reviewing the current understanding of the
pathobiology of autoimmune encephalitis, we combine a neuroanatomical approach with
specific antibody syndromes to provide the reader with a clinically relevant review
of these disorders. The clinical manifestations, diagnosis, and management of autoimmune
encephalitis are reviewed, with an emphasis on clinical relevance. We also introduce
updates in the field, including autoimmune encephalitis associated with novel cancer
immunotherapies, infectious triggers of autoimmune encephalitis, and autoimmune encephalitis
with demyelinating overlap syndromes.
Keywords
autoimmune - paraneoplastic - encephalitis - antibodies - immunotherapy
