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Limbic Encephalitis and Related Cortical Syndromes

  • PARANEOPLASTIC SYNDROMES (F GRAUS, SECTION EDITOR)
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Patients with limbic encephalitis and related cortical syndromes develop neurologic and psychiatric symptoms due to autoimmune damage of limbic and extralimbic brain areas. Some patients develop limbic encephalitis as a paraneoplastic manifestation of systemic cancer, where immune tolerance is thought to be lost. Serum and cerebrospinal antibodies against neuronal antigens can be detected in most patients. According to the type of antibodies found, patients may be classified into two broad groups, with implications for neurological and cancer diagnosis, treatment and prognosis. Patients with antibodies to intracellular neuronal antigens (onconeuronal antibodies: Hu, Ma2, amphiphysin, CV2/CRMP5) almost invariably have an underlying neoplasm (lung, testis, breast, etc.), often require aggressive combined antineoplastic and immunomodulatory treatment and their prognosis is usually poor. Patients with antibodies to cell-membrane antigens may present with classical limbic encephalitis (anti-LGI1 antibodies, other) or with a more diffuse stereotyped encephalitis, combining psychiatric symptoms, seizures, dyskinesias, catatonia and central hypoventilation (NMDAR-encephalitis). These patients may or may not have an associated tumor (thymoma, ovarian teratoma, etc.), are likely to improve with immunomodulatory treatment (and tumor removal for paraneoplastic cases) and have, overall, a better prognosis. Immunotherapy in patients with limbic encephalitis may be escalated according to the severity of neurological symptoms, the associated antibodies and the predicted success of antineoplastic treatment. First-line immunotherapies include high dose steroids, intravenous immunoglobulins and plasma exchange. Second-line drugs include Rituximab and Cyclophosphamide. For some patients, maintenance treatment with immunosuppressants might be required to prevent deterioration or relapses. Supportive treatment includes antiepileptic drugs, psychopharmacologic agents and scrutiny and prophylaxis of opportunistic infections.

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Disclosure

Dr. Rubio-Agusti has received travel support from UCB Pharma and Ibsen.

Dr. Salavert has served as a consultant for Gilead Sciences and has received payment for development of educational presentations (including service on speakers’ bureaus) from Pfizer, Janssen Pharmaceutica, Merck Sharp & Dohme, and Novartis.

Dr. Bataller reported no potential conflicts of interest relevant to this article.

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Authors and Affiliations

  1. Department of Neurology, Hospital Universitari I Politècnic La Fe, Boulevar Sur s/n, 46026, Valencia, Spain

    Ignacio Rubio-Agusti MD & Luis Bataller MD

  2. Department of Infectious Diseases, Hospital Universitari I Politècnic La Fe, Boulevar Sur s/n, 46026, Valencia, Spain

    Miguel Salavert MD

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Rubio-Agusti, I., Salavert, M. & Bataller, L. Limbic Encephalitis and Related Cortical Syndromes. Curr Treat Options Neurol 15, 169–184 (2013). https://doi.org/10.1007/s11940-012-0212-7

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