Autoimmune limbic encephalitis
Keywords:
limbic encephalitis, SARS Cov 2, immunomodulatory treatmentAbstract
Introduction: Limbic encephalitis (LE) is an antibody- associated autoimmune encephalitis predominantly involving the limbic system. The diagnosis is complex and based on specific diagnostic criteria.
We present a case of a 60 years old man, hospitalized in our Clinic in a state of status epilepticus. He had a history of past SARS Cov 2 infection. The diagnosis „LE” was based on the clinical picture, imaging and laboratory examinations. The patient was treated with immunomodulatory therapy (corticosteroids and plasmapheresis), antiepileptic and symptomatic medications. He has been followed up for more than 1.5 years.
Conclusion: LE is a complex diagnosis, which should be suspected in cases with subacute onset of psychiatric, cognitive and epileptic syndromes, when infectious cause has been excluded. The treatment is with immunomodulatory drugs. Usually it includes corticosteroids and intravenous immunoglobulins /IVIG/ and plasmapheresis (first line therapy). In some cases, second line therapy (mostly Rituximab or Cyclophosphamide) may be used. The favourable outcome is highly dependant on the prompt diagnosis and the adequate and timely treatment.
References
Abboud, H., Probasco, J., Irani, S.R., Ances, B., Benavides, D.R., Bradshaw, M., Christo, P.P., Dale, R.C., Fernandez-Fournier, M., Flanagan, E.P., Gadoth, A., George, P., Grebenciucova, E., Jammoul, A., Lee, S.T., Li, Y., Matiello, M., Morse, A.M., Rae-Grant, A., Rojas, G., Rossman, I., Schmitt, S., Venkatesan, A., Vernino, S., Pittock, S.J., Titulaer, M.; Autoimmune Encephalitis Alliance Clinicians Network. Autoimmune encephalitis: proposed recommendations for symptomatic and long-term management. J Neurol Neurosurg Psychiatry, 2021, 92, 8, 897-907.
Budhram, A., Leung, A., Nicolle, M.W., Burneo, J.G. Diagnosing autoimmune limbic encephalitis. CMAJ, 2019, 191, 19, 529-534.
Cohen, J., Sotoca, J., Gandhi, S., Yeshokumar, A.K., Gordon-Lipkin, E., Geocadin, R.G., Frick, K.D., Probasco, J.C., Venkatesan, A. Autoimmune encephalitis: A costly condition. Neurology, 2019, 92, 9, 964-972.
Ding, J.B., Dongas, J., Hu, K., Ding, M. Autoimmune Limbic Encephalitis: A Review of Clinicoradiological Features and the Challenges of Diagnosis. Cureus, 2021, 13, 8, e17529.
Ellul, M.A., Wood, G., Tooren, H.V.D., Easton, A., Babu, A., Michael, B.D. Update on the diagnosis and management of autoimmune encephalitis. Clin Med (Lond), 2020, 20, 4, 389-392.
Graus, F., Titulaer, M.J., Balu, R., Benseler, S., Bien, C.G., Cellucci, T., Cortese, I., Dale, R.C., Gelfand, J.M., Geschwind, M., Glaser, C.A., Honnorat, J., Höftberger, R., Iizuka, T., Irani, S.R., Lancaster, E., Leypoldt, F., Prüss, H., Rae-Grant, A., Reindl, M., Rosenfeld, M.R., Rostásy, K., Saiz, A., Venkatesan, A., Vincent, A., Wandinger, K.P., Waters, P., Dalmau, J. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol, 2016, 15, 4, 391-404.
Nabizadeh, F., Balabandian, M., Sodeifian, F., Rezaei, N., Rostami, M.R., Naser Moghadasi, A. Autoimmune encephalitis associated with COVID-19: A systematic review. Mult Scler Relat Disord, 2022, 62, 103795.
Uy, C.E., Binks, S., Irani, S.R. Autoimmune encephalitis: clinical spectrum and management. Pract Neurol, 2021, 21, 5, 412-423.
Downloads
Published
How to Cite
Issue
Section
ARK
License
Copyright (c) 2023 Bulgarian Neurology
This work is licensed under a Creative Commons Attribution 4.0 International License.