Evaluation of seizure treatment in anti-LGI1, anti-NMDAR, and anti-GABABR encephalitis

Marienke A. A. M. de Bruijn, Agnes van Sonderen, Marleen H. van Coevorden-Hameete, Anna E. M. Bastiaansen, Marco W. J. Schreurs, Rob P. W. Rouhl, Cees A. van Donselaar, Marian H. J. M. Majoie, Rinze F. Neuteboom, Peter A. E. Sillevis Smitt, Roland D. Thijs, Maarten J. Titulaer*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

115 Citations (Web of Science)

Abstract

Objective

This nationwide cohort study evaluates seizure responses to immunotherapy and antiepileptic drugs (AEDs) in patients with anti-leucine-rich glioma-inactivated 1 (LGI1), anti-NMDA receptor (NMDAR), and anti-gamma-aminobutyric-acid B receptor (GABA(B)R) encephalitis.

Methods

Anti-LGI1, anti-NMDAR, and anti-GABA(B)R encephalitis patients with new-onset seizures were included. Medical information about disease course, AEDs and immunotherapies used, effects, and side effects were collected. Outcome measures were (1) seizure freedom while using AEDs or immunotherapy, (2) days to seizure freedom from start of AEDs or immunotherapy, and (3) side effects.

Results

Of 153 patients with autoimmune encephalitis (AIE) (53 LGI1, 75 NMDAR, 25 GABABR), 72% (n = 110) had epileptic seizures, and 89% reached seizure freedom. At least 53% achieved seizure freedom shortly after immunotherapy, and 14% achieved seizure freedom while using only AEDs (p <0.0001). This effect was similar in all types (p = 0.0001; p = 0.0005; p = 0.013, respectively). Median time to seizure freedom from AEDs start was 59 days (interquartile range [IQR] 27-160), and 28 days from start of immunotherapy (IQR 9-71, p <0.0001). Side effects were psychotic behavior and suicidal thoughts by the use of levetiracetam, and rash by the use of carbamazepine. Carbamazepine was more effective than levetiracetam in reducing seizures in anti-LGI1 encephalitis (p = 0.031). Only 1 patient, of 86 surviving patients, developed epilepsy after resolved encephalitis.

Conclusion

Epilepsy after resolved encephalitis was rare in our cohort of patients with AIE treated with immunotherapy. In addition, seizure freedom is achieved faster and more frequently after immunotherapy. Therefore, AEDs should be considered as add-on treatment, and similar to treatment of other encephalitis symptoms, immunotherapy is crucial.

Original languageEnglish
Pages (from-to)E2185-E2196
Number of pages12
JournalNeurology
Volume92
Issue number19
DOIs
Publication statusPublished - 7 May 2019

Keywords

  • FACIOBRACHIAL DYSTONIC SEIZURES
  • RECEPTOR ENCEPHALITIS
  • ILAE COMMISSION
  • POSITION PAPER
  • FOLLOW-UP
  • IMMUNOTHERAPY
  • EPILEPSY
  • CLASSIFICATION
  • AUTOIMMUNE
  • DEFINITION

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