Seizures in autoimmune encephalitis: specific features based on a systematic comparative study.

Epileptic Disord

AP-HP, Department of Neurology, Epilepsy Unit, Pitié-Salpêtrière Hospital, Paris, France, Paris Brain Institute (Inserm, CNRS, Sorbonne Université), Paris, France, Center of Reference for Rare epilepsies, Pitié-Salpêtrière Hospital, Paris, France.

Published: December 2021

Objective: To highlight specific characteristics of seizure semiology and EEG features associated with different subtypes of autoimmune encephalitis (AE).

Methods: We systematically reviewed the seizure semiology and all the EEG recordings from patients with AE managed in a tertiary referral centre for epilepsy and a neuro-intensive care unit. Each characteristic across the different subtypes of AE was compared by post hoc analysis.

Results: We identified 66 patients with anti-neuronal antibody-mediated AE or Rasmussen's encephalitis (RE) experiencing seizures, which were the most frequent symptom at onset. Anti-NMDAR and anti-LGI1 AE accounted for the majority of patients; 41% and 24%, respectively. We isolated specific semiological features, such as early tonic-clonic seizures (TCS) in anti-NMDAR AE, early mesial temporal lobe seizures with emotional symptoms in anti-GAD AE, somatosensory seizures in RE, and a lower frequency of TCS in anti-LGI1 AE. EEG analysis also provided additional insights into distinguishing the subtypes based on: (1) generalized rhythmic delta activity, which was more sensitive than extreme delta brush in identifying anti-NMDAR AE among all subtypes; and (2) temporal interictal epileptiform activity and temporal seizures on EEG in anti-GAD AE. We identified a new EEG pattern consisting of temporal low-voltage and periodic spikes associated with ipsilateral hippocampal abnormalities on MRI, which could be a sign of inflammatory mesial temporal involvement.

Significance: Specific clinical and EEG features can be useful in guiding the diagnosis of a subtype of AE with acute symptomatic seizures, particularly before the results of anti-neuronal antibody testing are available.

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http://dx.doi.org/10.1684/epd.2021.1355DOI Listing

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