Clinical Characteristics

Autoimmune Epilepsy

Clinical Characteristics and Diagnosis

Identifying immune-mediated seizures can be challenging in clinical practice. However, several factors may raise suspicion.

  • Patients with autoimmune-induced seizures typically present with an explosive onset, and a higher frequency of seizures than normal. 1
  • In most cases, these seizures do not respond to anti-epileptic drugs and patients may be diagnosed with refractory or drug-resistant epilepsy. 2

In fact, a review of several studies that included 169 patients with autoimmune epilepsy found that anti-seizure medications were effective in only 10.7% of cases. 3

  • Patients with autoimmune epilepsy typically present with refractory seizures with at least one co-existing feature of autoimmune encephalitis including subacute progressive cognitive decline, psychiatric symptoms, viral prodrome, autonomic dysfunction, inflammatory [markers in] CSF, oncological association, or brain MRI changes consistent with autoimmune encephalitis. 4
  • Autoimmune seizures are typically focal, though may propagate to bilateral tonic-clonic seizures. 5
Autoimmune epilepsy presents with specific clinical manifestations, and various diagnostic approaches including cerebrospinal fluid analysis, neuroimaging, and autoantibody tests are essential for its differential diagnosis.” 6

Researchers have suggested utilizing the diagnostic criteria for autoimmune encephalitis: in patients with seizures, an autoimmune encephalitis is more likely when the patient also has the subacute onset of psychiatric, memory, or mental status deficits, and when alternative causes have been reasonably excluded. 7

To assist clinicians in making an early and accurate diagnosis, investigators at Mayo Clinic have developed predictive scoring systems — the Antibody Prevalence in Epilepsy (APE) and Response to Immunotherapy in Epilepsy (RITE), both of which can help identify seizure patients likely to respond to immunotherapy. 5

Response to Immunotherapy in Epilepsy
  1. Unraveling Autoimmune Epilepsy. Neurology Live. 8/20. Volume 3. Issue 4. Unraveling Autoimmune Epilepsy (neurologylive.com)
  2. MedPage Today. Nyberg. Identification and Treatment of Autoimmune Epilepsy. July 13, 2018.
  3. Cabezudo-García P, Mena-Vázquez N, Villagrán-García M, Serrano-Castro PJ. Efficacy of antiepileptic drugs in autoimmune epilepsy: A systematic review. Seizure. 2018 Jul;59:72-76. doi: 10.1016/j.seizure.2018.05.004. Epub 2018 May 7. PMID: 29754014.
  4. Schubert RD, Wilson MR. A tale of two approaches: how metagenomics and proteomics are shaping the future of encephalitis diagnostics. Curr Opin Neurol. 2015 Jun;28(3):283-7. doi: 10.1097/WCO.0000000000000198. PMID: 25923127; PMCID: PMC4418212.
  5. Dubey, D., Singh, J., Britton, J.W., Pittock, S.J., Flanagan, E.P., Lennon, V.A., Tillema, J.-M., Wirrell, E., Shin, C., So, E., Cascino, G.D., Wingerchuk, D.M., Hoerth, M.T., Shih, J.J., Nickels, K.C. and McKeon, A. (2017), Predictive models in the diagnosis and treatment of autoimmune epilepsy. Epilepsia, 58: 1181-1189.
  6. Jang Y, Kim DW, Yang KI, Byun JI, Seo JG, No YJ, Kang KW, Kim D, Kim KT, Cho YW, Lee ST; Drug Committee of Korean Epilepsy Society. Clinical Approach to Autoimmune Epilepsy. J Clin Neurol. 2020 Oct;16(4):519-529. doi: 10.3988/jcn.2020.16.4.519. PMID: 33029957; PMCID: PMC7541993.
  7. Graus F, Titulaer MJ, Balu R, Benseler S, Bien CG, Cellucci T, Cortese I, Dale RC, Gelfand JM, Geschwind M, Glaser CA, Honnorat J, Höftberger R, Iizuka T, Irani SR, Lancaster E, Leypoldt F, Prüss H, Rae-Grant A, Reindl M, Rosenfeld MR, Rostásy K, Saiz A, Venkatesan A, Vincent A, Wandinger KP, Waters P, Dalmau J. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016 Apr;15(4):391-404. doi: 10.1016/S1474-4422(15)00401-9. Epub 2016 Feb 20. PMID: 26906964; PMCID: PMC5066574.
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