Rasmussen's syndrome treated with anakinra

Area of Science:

• Neurology
• Immunology
• Neuroinflammation

Background:

• Rasmussen's encephalitis (RE) is a rare autoimmune neurological disorder characterized by unilateral brain inflammation, leading to epilepsy, hemiparesis, and cognitive decline.
• The Interleukin-1 (IL-1) pathway, specifically the IL-1β-IL-1R1 axis, is implicated in the neuroinflammation associated with RE.
• Anakinra, an IL-1 inhibitor, has demonstrated efficacy in other refractory epilepsy syndromes.

Observation:

• A 38-year-old male with RE presented with persistent focal seizures (epilepsia partialis continua) and right-sided hemiparesis.
• The patient was refractory to standard treatments including pulse steroids, intravenous immunoglobulin, and anti-seizure medications.

Findings:

• Treatment with anakinra for three weeks resulted in near-complete resolution of continuous spasms.
• Significant improvement in muscle strength (hemiparesis) was observed following anakinra therapy.

Implications:

• Anakinra represents a potential therapeutic option for managing refractory seizures in Rasmussen's encephalitis.
• Targeting the IL-1 pathway may offer a novel treatment strategy for patients with severe, treatment-resistant autoimmune neurological conditions.