Electrographic Features of Jeavons Syndrome with Photoparoxysmal Response: Further Evidence that It May Belong to Occipital or Frontal Lobe Initiated Epilepsies
Abstract number :
2.077
Submission category :
4. Clinical Epilepsy / 4A. Classification and Syndromes
Year :
2021
Submission ID :
1826570
Source :
www.aesnet.org
Presentation date :
12/5/2021 12:00:00 PM
Published date :
Nov 22, 2021, 06:54 AM
Authors :
ifrah Zawar, MD - University of Virginia; Bijina Shrestha, MD – Cleveland Clinic; Daniela Benech – Cleveland Clinic; Richard Burgess – Cleveland Clinic; Juan Bulacio – Cleveland Clinic; Elia Pestana Knight – Cleveland Clinic
Rationale: Jeavons Syndrome or Epilepsy with eyelid myoclonias is characterized by eyelid myoclonia, eyelid-closure sensitivity and photosensitivity. It may manifest with frontal-predominant (FPEDs) or occipital-predominant epileptiform discharges (OPEDs). Data on clinical and electrographic features of these two subtypes is lacking. The objective of our research is to look at baseline electrographic and clinical features of Jeavons Syndrome subtypes and to study electrographic findings of Jeavons Syndrome patients during intermittent photic stimulation.
Methods: We retrospectively identified all patients who had photoparoxysmal response on EEGs performed at Cleveland clinic between 01/01/2012 and 12/31/2019. Patients who met diagnostic criteria for Jeavons Syndrome were studied further for clinical and EEG findings.
Results: Of 249 patients with photoparoxysmal response, seventy (28.1%) had Jeavons Syndrome (62 (88.6%) female; mean age of epilepsy onset: 7.0+7.9). Jeavons syndrome patients had either FPEDs or OPEDs. Eleven (15.7%) Jeavons syndrome patients had seizures (absence [4], myoclonic [5], bilateral-tonic-clonic [2]) during photic stimulation. Patients with OPEDs were more likely to have refractory epilepsy, occipital focal interictal epileptiform discharges (IEDs) and other focal IEDs (other than frontal/occipital) on baseline EEG and generalized IEDs with occipital predominance, generalized IEDs with no predominance or focal IEDs during photic stimulation. Predictors of seizure occurrence during photic stimulation included presence of focal occipital IEDs on baseline EEG, generalized IED with frontal predominance on photic stimulation and photoparoxysmal response outlasting the stimulus. Four had typical absence seizures (all at 15Hz and higher frequencies), 5 had generalized myoclonic seizures (all at 15 Hz and higher frequencies) and 2 had bilateral-tonic-clonic seizures (at 12 and 20 Hz respectively).
Conclusions: Our study provides evidence that Jeavons syndrome has two distinct subtypes (with OPEDs or FPEDs) which differ in terms of clinical characteristics, baseline EEG and EEG during photic stimulation. We highlight diagnostic and prognostic implications of these findings. Our study also details EEG characteristics of Jeavons syndrome patients during photic stimulation.
Funding: Please list any funding that was received in support of this abstract.: N/A.
Clinical Epilepsy