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February 12, 2008; 70 (7) Editorials

Limbic encephalitis

An expanding concept

Francesc Graus, Albert Saiz
First published February 11, 2008, DOI: https://doi.org/10.1212/01.wnl.0000299189.43164.96
Francesc Graus
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Albert Saiz
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Citation
Limbic encephalitis
An expanding concept
Francesc Graus, Albert Saiz
Neurology Feb 2008, 70 (7) 500-501; DOI: 10.1212/01.wnl.0000299189.43164.96

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Limbic encephalitis (LE) was initially described as a paraneoplastic syndrome characterized by rapid development of confusion, seizures, short-term memory loss, and high MRI T2 and FLAIR signal involving one or both medial temporal lobes.1 No more than 5 years ago, LE was considered to invariably have a paraneoplastic origin mostly associated with lung or testicular cancer and with antibodies against intracellular neuronal antigens. Neuropathologic studies show dominant parenchymal infiltrates of T-cells supporting the hypothesis, not proven yet, that the disorder is mediated by a T cell driven immune response, presumably against the same antigens recognized by the antibodies.1

The concept of LE expanded with the description of patients with otherwise classic LE in association with antibodies to voltage-gated potassium channels (VGKC).2 This variant of LE infrequently associates with cancer, and often responds to immunotherapy along with a decrease of serum antibody titers, suggesting that the antibodies are pathogenic.2

More recently, a new encephalitis was described in 12 women with ovarian teratomas.3 The syndrome predictably evolves in stages, including prodromal fever followed in a few days by prominent psychiatric symptoms or …

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