Seizure control after late introduction of anakinra in a patient with adult onset Rasmussen’s encephalitis
| Autor: | Sigrid Svalheim, Ketil Berg Olsen, Line Sveberg, Erik Taubøll, Kjell Heuser, Bjørn Tennøe, Monika Mochol |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Rasmussen's encephalitis
musculoskeletal diseases Neurophysiology and neuropsychology Case Report Epileptogenesis Behavioral Neuroscience ASM antiseizure medication Neuroinflammation FIRES febrile infection-related epilepsy syndrome medicine FBTC Focal to bilateral tonic-clonic RC346-429 Autoimmune encephalitis Anakinra medicine.diagnostic_test GTC Generalized tonic-clonic Drug resistant epilepsy business.industry Brain biopsy Hemiatrophy QP351-495 medicine.disease Drug Resistant Epilepsy Pathophysiology Rasmussen’s encephalitis Neurology IVIG Intravenous immunoglobulins Anesthesia Neurology (clinical) Neurology. Diseases of the nervous system business Encephalitis medicine.drug |
| Zdroj: | Epilepsy & Behavior Reports, Vol 16, Iss, Pp 100462-(2021) Epilepsy & Behavior Reports |
| ISSN: | 2589-9864 |
| Popis: | Highlights • Ten percentage of patient with RE has adult onset of symptoms. • Experience with IL-1Ra like anakinra in RE patients is limited. • 26 years after RE onset anakinra was introduced and led to complete seizure control. • Premature withdrawal of anakinra (2 months) caused relapse of seizures. • Our patient remains seizure free 13 months after 2nd course (7 months) of anakinra. 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. |
| Databáze: | OpenAIRE |
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