Authors :
Presenting Author: Brook Centofanti, DO – HFH
Jason Schwab, MD – HFH; Ahmad Yusuf Solaiman, MD – HFH; Vibhangini Wasade, MD – HFH
Rationale:
Ictal whistling is an uncommon and unique semiology observed in focal seizures usually localized to the medial temporal lobes. Accurate localization is crucial for diagnosis, especially in patients with intractable focal epilepsy undergoing pre-surgical evaluation for appropriate seizure treatment planning. Here, we report a case with unique localization of ictal whistling, with seizure onset originating from the left amygdala diagnosed on intracranial stereo-EEG (sEEG).
Methods:
A 45-year-old man diagnosed with intractable focal epilepsy, with onset during his teenage years. His focal seizures were described as aura feeling followed by staring, loss of awareness, and whistling sounds lasting for two to three minutes. He also had focal to generalized tonic-clonic seizures directly preceded by aura. He had a normal birth, denied history of CNS infection, but had a history of head trauma at age 10 without loss of consciousness. His seizures continued in spite of being on poly-therapy anti-seizure medication (ASMs). Epilepsy Monitoring unit during pre-surgical scalp EEG interictal and ictal recording indicated left hemispheric patterns with strong predominance over the left temporal derivations. MRI brain showed numerous sub-ependymal heterotopias lining the bilateral occipital horns extending to the level of the atria bilaterally. PET-CT showed decreased metabolism in the anterior, medial aspect left temporal lobe suggestive of seizure focus. Neuropsychological testing localized to the dominant temporal lobe (mesial temporal > cortical), with bilaterally equivocal memory functions. MEG-EEG indicated sources originating from the left insula and left frontal operculum which was seen on EEG. The MEG showed left inferior temporal gyrus. The patient underwent phase 2 monitoring in EMU with sEEG with depth electrode implantation over bilateral hippocampus, amygdala, left insula, left cingulate and orbitofrontal, and heterotopias over the occipital horns.
Results:
Phase 2 sEEG testing showed four focal electrographic-clinical seizures with electrographic onset over the deep contacts of the left amygdala quickly spreading to the left hippocampus. Clinically, the seizures presented with aphasia, loss of awareness, oral automatisms: blowing air, whistling, lip-smacking and swallowing movements were noted. Right tonic-clonic facial twitching, eye fluttering, right gaze, and head deviation was noted in the one focal to bilateral tonic-clonic seizure. Aura was not reported before these seizures, however, the patient confirmed to us that he never had the aura independently to consider it as a separate semiology. These findings helped in further surgical planning for this patient.Conclusions:
This case illustrates a unique localization of ictal whistling to the left amygdala during EEG monitoring. Few cases have been reported with most showing some localization to the temporal lobes without clear consistent lateralization. Understanding the localization and lateralization of this unique seizure semiology is vital for diagnosis and appropriate seizure treatment planning.
Funding: none