Abstract
In the past few years, many autoimmune encephalitides have been identified, with specific
clinical syndromes and associated antibodies against neuronal surface antigens. There
is compelling evidence that many of these antibodies are pathogenic and most of these
encephalitides are highly responsive to immunotherapies. The clinical spectra of some
of these antibody-mediated syndromes, especially those reported in only a few patients,
are evolving. Others, such as anti-N-methyl-D-aspartate (NMDA) receptor encephalitis,
are well characterized. Diagnosis involves recognizing the specific syndromes and
identifying the antibody in a patient's cerebrospinal fluid (CSF) and/or serum. These
syndromes are associated with variable abnormalities in CSF, magnetic resonance imaging,
and electroencephalography. Treatment is often multidisciplinary and should be focused
upon neutralizing the effects of antibodies and eliminating their source. Overlapping
disorders have been noted, with some patients having more than one neurologic autoimmune
disease. In other patients, viral infections such as herpes simplex virus encephalitis
trigger robust antineuronal autoimmune responses.
Keywords
autoimmune - limbic - encephalitis - neuronal antibodies