Abstract
ObjectiveTo evaluate the seizure characteristics and outcome after immunotherapy in adult patients with autoimmune encephalitis (AE) and new-onset seizure.MethodsAdult (age ≥18 years) patients with AE and new-onset seizure who underwent immunotherapy and were followed-up for at least 6 months were included. Seizure frequency was evaluated at 2–4 weeks and 6 months after the onset of the initial immunotherapy and was categorized as “seizure remission”, “> 50% seizure reduction”, or “no change” based on the degree of its decrease.ResultsForty-one AE patients who presented with new-onset seizure were analysed. At 2–4 weeks after the initial immunotherapy, 51.2% of the patients were seizure free, and 24.4% had significant seizure reduction. At 6 months, seizure remission was observed in 73.2% of the patients, although four patients died during hospitalization. Rituximab was used as a second-line immunotherapy in 12 patients who continued to have seizures despite the initial immunotherapy, and additional seizure remission was achieved in 66.6% of them. In particular, those who exhibited partial response to the initial immunotherapy had a better seizure outcome after rituximab, with low adverse events.ConclusionAE frequently presented as seizure, but only 18.9% of the living patients suffered from seizure at 6 months after immunotherapy. Aggressive immunotherapy can improve seizure outcome in patients with AE.
Highlights
Autoimmune encephalitis (AE) is an emerging cause of diffuse or limbic encephalitis that frequently presents with seizure or status epilepticus.[1, 2]
Seizure Outcome after Immunotherapy in Autoimmune Encephalitis patients, four patients died during hospitalization
Rituximab was used as a second-line immunotherapy in 12 patients who continued to have seizures despite the initial immunotherapy, and additional seizure remission was achieved in 66.6% of them
Summary
Autoimmune encephalitis (AE) is an emerging cause of diffuse or limbic encephalitis that frequently presents with seizure or status epilepticus.[1, 2]. Neuronal antibodies of either paraneoplastic or nonparaneoplastic origin have been discovered to be associated with patients with autoimmune encephalitis.[3, 4] Paraneoplastic antibodies, including those against Hu, Ma2/Ta, amphiphysin, and CRMP5, involve the limbic system and cause seizures with memory deficit or psychiatric symptoms.[5] Nonparaneoplastic antibodies, including those against the anti-N-methyl-D-aspartate receptor (NMDAR), voltage-gated potassium channel (VGKC, leucine-rich glioma inactivated 1 (LGI1), or Caspr2), and gamma-aminobutyric acid b (GABAb), directly target synaptic proteins that play a critical role in synaptic transmission, which can cause seizures.[6, 7] Seventy percent of the patients with anti-NMDAR encephalitis develop complex partial seizure, generalized tonic–clonic seizure, or status epilepticus.[8, 9] The antibodies against LGI1 or GABAb are associated with classic limbic encephalitis and early prominent seizures.[6, 10] Notably, LGI1 encephalitis often presents with a characteristic seizure called faciobrachial dystonic seizure [11], which can precede the other symptoms of encephalitis.[12] Seizures can occur during the acute phase and later, resulting in postencephalitic epilepsy because of frequent involvement of epileptogenic structures. Studies of seizure characteristics and outcome in AE are lacking
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