Seizure control after late introduction of anakinra in a patient with adult onset Rasmussen's encephalitis

Monika Mochol; Erik Taubøll; Line Sveberg; Bjørn Tennøe; Ketil Berg Olsen; Kjell Heuser; Sigrid Svalheim

doi:10.1016/j.ebr.2021.100462

Seizure control after late introduction of anakinra in a patient with adult onset Rasmussen's encephalitis

Epilepsy Behav Rep. 2021 May 31:16:100462. doi: 10.1016/j.ebr.2021.100462. eCollection 2021.

Authors

Monika Mochol^{1

2

3}, Erik Taubøll^{2

3}, Line Sveberg³, Bjørn Tennøe⁴, Ketil Berg Olsen^{1

3}, Kjell Heuser³, Sigrid Svalheim³

Affiliations

¹ Department of Neurology, Østfold Hospital Trust, PO Box 300, 1714 Grålum, Norway.
² Institute of Clinical Medicine, University of Oslo, PO Box 1072 Blindern, 0316 Oslo, Norway.
³ ERGO - Epilepsy Research Group of Oslo, Department of Neurology, Oslo University Hospital, PO Box 4950 Nydalen, 0424 Oslo, Norway.
⁴ Department of Radiology and Nuclear Medicine, Oslo University Hospital, PO Box 4950 Nydalen, 0424 Oslo, Norway.

Abstract

Neuroinflammation has been considered an important pathophysiological process involved in epileptogenesis and may provide possibilities for new treatment possibilities. We present the case of a 45-year-old female with drug resistant epilepsy and progressive right-sided cerebral hemiatrophy associated with adult onset Rasmussen's encephalitis. Over a period of 26 years, she was treated with 14 different antiseizure medications, intravenous immunoglobulins, glucocorticosteroids, underwent two operations with focal resection and subpial transections, and tried out trigeminal nerve stimulation. Extensive blood tests, including antibodies relevant for autoimmune encephalitis, and brain biopsy did not show any signs of neuroinflammation. Eventually, the patient received the interleukin-1 receptor antagonist, anakinra. Within 1-2 days after injection, seizure frequency decreased significantly, and, after one week, the seizures stopped completely. Anakinra treatment was continued for 2 months. Stopping medication led to a relapse of seizures after 2 weeks, with a frequency of up to 45 seizures per day. Reintroduction of anakinra led to rapid recovery. Treatment with anakinra was continued for 7 months. The treatment was discontinued in April 2020, and the patient has been completely seizure free since then. There have been no other changes in antiseizure medication.

Keywords: ASM, antiseizure medication; Anakinra; Drug resistant epilepsy; FBTC, Focal to bilateral tonic-clonic; FIRES, febrile infection-related epilepsy syndrome; GTC, Generalized tonic-clonic; Hemiatrophy; IVIG, Intravenous immunoglobulins; Neuroinflammation; Rasmussen’s encephalitis.

Publication types

Case Reports