Autoimmune Encephalopathy

Eoin P. Flanagan, M.B.B.Ch.; Richard J. Caselli, M.D.

Disclosures

Semin Neurol. 2011;31(2):144-157. 

In This Article

Paraneoplastic Autoimmune Encephalopathy

In the last 5 years, there have been great advances in our understanding of paraneoplastic limbic encephalitis. Numerous novel neural-specific autoantibodies with strong association of malignancy have been discovered, including autoantibodies to the (NMDA) receptor,[14] amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPA) receptor,[21] gamma-aminobutyric acid B (GABA-B) receptor,[22] and Caspr2 antibodies.[6] A list of neural-specific autoantibodies and their cancer associations is outlined in Table 4.

Paraneoplastic encephalopathies frequently have a poor outcome. Long-term survival often depends on the ability to remove the provocative tumor,[14] but early aggressive immunotherapy may also improve outcomes.[23] Therefore, patients with NMDA receptor autoantibodies associated with ovarian teratomas[14] that can be completely removed have a better prognosis overall than those with GABA-B autoantibodies[22] associated with small cell carcinoma, which usually cannot be completely resected.

For a more detailed discussion on paraneoplastic autoantibodies, please refer to the paraneoplastic syndrome review article by Pruitt in this issue (Semin Neurol 2011;31:159–169).

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