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- Limbic encephalitis
- clinical neurology
- limbic system
Limbic encephalitis (LE) is characterised by seizures and impairment of short-term memory as well as behavioural and psychiatric symptoms such as anxiety, depression, personality change and hallucinations. Onset of these symptoms is typically subacute over a few weeks or months but may also evolve over a few days. In many patients, limbic encephalitis is a paraneoplastic syndrome usually preceding diagnosis of the malignancy. Associated tumours are most commonly small-cell lung cancer (SCLC), breast cancer, testicular tumour, teratoma, Hodgkin lymphoma and thymoma.1 Antineuronal autoantibodies can be detected in the sera of about 60% of patients. These autoantibodies are classically directed to intracellular antigens (eg, anti-Hu, anti-Ma1/2, anti-CRMP5/CV2, anti-amphiphysin). However, LE is now being recognised frequently in the absence of malignancy and can be associated with antibodies to voltage-gated potassium channel (VGKC-ab). More recently, a new type of immunotherapy-responsive severe LE was described by Dalmau and colleagues that is associated with antibodies to the n-methyl-d-aspartate-receptor (NMDAR) and ovarian teratoma. In rare instances, these NMDAR-ab also occur in men with testicular teratoma or SCLC.2 3 For the first time, we describe a male patient with non-paraneoplastic limbic encephalitis and with serum antibodies to both NMDAR and VGKC.
This previously healthy 56-year-old man reported the first signs of disease …
Competing interests HP and TK have received personal compensations from Bayer Schering Pharma, Teva, Merck-Serono and Biogen Idec. TD has received personal compensations from Bayer Schering Pharma, Novartis, Merck-Serono and Biogen Idec.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.