Log in

The well responsiveness of drug-resistant focal seizures in anti-AMPA2 receptor encephalitis to perampanel treatment

  • Original Article
  • Published:
Neurological Sciences Aims and scope Submit manuscript

Abstract

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor encephalitis is an anti-neuronal surface antigen autoimmune encephalitis and is relatively rare. Our study evaluated a patient who developed anti-AMPA2 receptor encephalitis with memory deficits and refractory focal seizures as paroxysmal jerking on right face as well as dystonic seizure on right hand. On this patient, the combination treatment of levetiracetam, carbamazepine, and clonazepam, monthly periodic intravenous immunoglobin and immunosuppressive therapies for 5 months was not effective for the focal seizures, while his memory loss was slightly improved. However, adjunctive perampanel treatment led to a rapid relief of seizures. Perampanel is suggested in seizures associated with anti-AMPA receptor encephalitis by directly attenuating nerve hyperexcitability caused by glutamate and Ca2+-permeable GluA4 subunit of AMPA receptors.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. Lai M, Hughes EG, Peng X, Zhou L, Gleichman AJ, Shu H, Matà S, Kremens D, Vitaliani R, Geschwind MD, Bataller L, Kalb RG, Davis R, Graus F, Lynch DR, Balice-Gordon R, Dalmau J (2009) AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. Ann Neurol 65:424–434

    Article  CAS  Google Scholar 

  2. Samad N and Wong J (2018) Anti-AMPA receptor encephalitis associated with Medullary thyroid cancer. BMJ Case Rep. 2018.

  3. Hoftberger R, van Sonderen A, Leypoldt F, Houghton D, Geschwind M, Gelfand J, Paredes M, Sabater L, Saiz A, Titulaer MJ, Graus F, Dalmau J (2015) Encephalitis and AMPA receptor antibodies: novel findings in a case series of 22 patients. Neurology. 84:2403–2412

    Article  Google Scholar 

  4. Shepherd JD, Huganir RL (2007) The cell biology of synaptic plasticity: AMPA receptor trafficking. Annu Rev Cell Dev Biol 23:613–643

    Article  CAS  Google Scholar 

  5. Bassani S, Valnegri P, Beretta F, Passafaro M (2009) The GLUR2 subunit of AMPA receptors: synaptic role. Neuroscience. 158:55–61

    Article  CAS  Google Scholar 

  6. Jia Y, Wang J, Xue L, Hou Y (2020) Limbic encephalitis associated with AMPA receptor and CRMP5 antibodies: A case report and literature review. Brain Behav 10:e01528

    PubMed  PubMed Central  Google Scholar 

  7. Fukumoto T, Miyamoto R, Fujita K, Murakami N, Matsui N, Izumi Y (2021) Reversible mixed perfusion on (123)I-IMP SPECT in anti-AMPA receptor encephalitis: A case report. J Neurol Sci 421:117306

    Article  CAS  Google Scholar 

  8. Laurido-Soto O, Brier MR, Simon LE, McCullough A, Bucelli RC, Day GS (2019) Patient characteristics and outcome associations in AMPA receptor encephalitis. J Neurol 266:450–460

    Article  CAS  Google Scholar 

  9. Plantone D, Renna R, Grossi D, Plantone F, Iorio R (2013) Teaching NeuroImages: Basal ganglia involvement in facio-brachial dystonic seizures associated with LGI1 antibodies. Neurology. 80:e183–e184

    Article  Google Scholar 

  10. Morano A, Fanella M, Cerulli Irelli E, Barone FA, Fisco G, Orlando B, Albini M, Fattouch J, Manfredi M, Casciato S, di Gennaro G, Giallonardo AT, di Bonaventura C (2020) Seizures in autoimmune encephalitis: Findings from an EEG pooled analysis. Seizure. 83:160–168

    Article  Google Scholar 

  11. Benarroch EE (2016) AMPA receptors: Dynamics and targets of disease. Neurology. 87:1281–1288

    Article  Google Scholar 

  12. Pelkey KA, Barksdale E, Craig MT, Yuan X, Sukumaran M, Vargish GA, Mitchell RM, Wyeth MS, Petralia RS, Chittajallu R, Karlsson RM, Cameron HA, Murata Y, Colonnese MT, Worley PF, McBain CJ (2015) Pentraxins coordinate excitatory synapse maturation and circuit integration of parvalbumin interneurons. Neuron. 85:1257–1272

    Article  CAS  Google Scholar 

  13. Akgul G, McBain CJ (2016) Diverse roles for ionotropic glutamate receptors on inhibitory interneurons in develo** and adult brain. J Physiol 594:5471–5490

    Article  Google Scholar 

  14. Akiyama H, Sasaki R, Hasegawa Y (2019) Efficacy of perampanel for anti-N-methyl-D-aspartate receptor encephalitis: a case report. Medicine (Baltimore) 98:e14033

    Article  Google Scholar 

  15. Abril Jaramillo J, Estevez Maria JC, Giron Ubeda JM, Vega Lopez O, Calzado Rivas ME, Perez Diaz H et al (2020) Effectiveness and safety of perampanel as early add-on treatment in patients with epilepsy and focal seizures in the routine clinical practice: Spain prospective study (PERADON). Epilepsy Behav 102:106655

    Article  Google Scholar 

  16. Di Giacomo R, Deleo F, Pastori C, Didato G, Andreetta F, Del Sole A et al (2019) Predictive value of high titer of GAD65 antibodies in a case of limbic encephalitis. J Neuroimmunol 337:577063

    Article  Google Scholar 

  17. Errichiello L, Perruolo G, Pascarella A, Formisano P, Minetti C, Striano S, Zara F, Striano P (2009) Autoantibodies to glutamic acid decarboxylase (GAD) in focal and generalized epilepsy: A study on 233 patients. J Neuroimmunol 211:120–123

    Article  CAS  Google Scholar 

  18. Bataller L, Galiano R, Garcia-Escrig M, Martinez B, Sevilla T, Blasco R, Vilchez JJ, Dalmau J (2010) Reversible paraneoplastic limbic encephalitis associated with antibodies to the AMPA receptor. Neurology. 74:265–267

    Article  CAS  Google Scholar 

Download references

Acknowledgements

The authors are grateful to the patient and his families for their participation

Availability of data and material

All data are available from the corresponding author upon reasonable request.

Funding

This work was supported by the National Science Foundation Research Grant of China (Grant No. 81701058) and Natural Science Foundation of Shandong Province (Grant No. ZR2017PH027).

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to **uhe Zhao.

Ethics declarations

Ethics approval

The patient provided informed consent for the use of the case data, and the ethics committee of Qilu Hospital, Cheeloo College of Medicine, Shandong University approved this study.

Informed consent

Informed consent has been taken from all patients.

Conflict of interest

The authors declare no competing interests.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary Information

ESM 1

(MP4 15173 kb)

ESM 2

(MP4 6697 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Wang, T., Wen, B., Chi, Z. et al. The well responsiveness of drug-resistant focal seizures in anti-AMPA2 receptor encephalitis to perampanel treatment. Neurol Sci 43, 525–532 (2022). https://doi.org/10.1007/s10072-021-05306-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10072-021-05306-y

Keywords

Navigation