Rationale:
Amity is defined as the quality of goodwill and friendly relationship between people. We describe a case of a patient with focal epilepsy with a semiology consisting of behaviors indicating an enthusiastic desire for those around him to get along and engage in friendly relations, which we refer to as “amity seizures." While ecstatic seizures are a rare but classic seizure semiology consisting of intense emotions of happiness, joy, or union with the universe, they are characterized by an individual,
internal sensation. Herein we report a case of SEEG-proven multifocal epilepsy producing a semiology of amity arising from conduction, in either direction, between two independent seizure-onset zones.
Methods:
The patient was a 41-year-old right-handed male with seizures since age 26. Semiology consisted of an aura of a sense of doom, followed by behaviors consisting of conciliatory statements such as “Peace! Peace!” and giving high-fives. He had no memory of these events. Seizures occurred three to four times per month, with rare secondary generalization. He underwent pre-surgical evaluation with scalp EEG, stereo-EEG, MRI, PET, SPECT, MEG, and WADA testing.
Results:
On SEEG evaluation, two areas of seizure onset were identified, specifically the right hippocampus and right mesial posterior orbital frontal area. Locally confined seizures, without spread outside either zone of onset, had bland manifestation. However, spread from right hippocampus to right orbital frontal area, or vice versa, elicited his typical amity seizure semiology. This consisted of stereotyped enthusiastic behaviors such as expressing “Peace! Peace!... Come on, we are all on the same team, right?!”, giving hugs and kisses to EEG techs, holding hands, and asking for and giving high-fives. Stimulation of seizure-onset zones failed to reproduce his characteristic semiology. However, stimulation of the right amygdala gave a vague, incomplete feeling reminiscent of his seizure aura. Stimulation of his amygdala and hippocampus demonstrated time-locked, low amplitude rhythmic activity in the right mesial posterior orbital frontal (RPOF) area, indicating strong effective connectivity, but stimulation of the RPOF did not elicit an electrographic response in the mesial temporal structures, which demonstrates a directionality to this connectivity.
Neuropsychology testing showed a reasonable risk of memory deficits after surgery. The patient thus elected to undergo neuromodulation rather than resection. RNS was placed to the right hippocampus. He had a reduction in seizure frequency from four per month to one per month, and remained on levetiracetam, cenobamate, and oxcarbazepine.Conclusions:
To our knowledge, this is the first report of the specific seizure semiology we have coined “Amity seizures." The fact that this behavior only occurred when seizures spread from either of two onset zones to the other suggests that this semiology results from network engagement beyond either onset zone.
Funding: No grant funding was received this case study