Focal Features in Jeavons Syndrome
Abstract number :
2.21
Submission category :
4. Clinical Epilepsy / 4A. Classification and Syndromes
Year :
2024
Submission ID :
363
Source :
www.aesnet.org
Presentation date :
12/8/2024 12:00:00 AM
Published date :
Authors :
Presenting Author: Simpson Benjamin, MD – Ochsner Health
Michael Kolesnik, DO – University of Colorado Anschutz
Lesley Kaye, MD – UC Health University of Colorado Hospital
Aaron Geller, MD – University of Colorado Anschutz
Rationale: Jeavons syndrome is primarily classified as a genetic generalized epilepsy, but focal findings are well documented. We present three cases of Jeavons syndrome with focal features.
Methods: Patients’ seizures were characterized with video-EEG. These findings were reviewed for both features of photosensitive seizures and other captured seizure types. We reviewed the chart for subsequent clinical management and patients’ response.
Results: We observed typical Jeavons features (eyelid myoclonia, eye-closure induced seizures, and photosensitivity) as well as independent focal seizures in three cases. All three patients had a history of frequent eyelid myoclonia, and all three patients presented with a history of one alternate seizure type. Focal seizure semiologies are as follows. Patient 1 experiences behavioral arrest followed by a compulsion to drink water progressing to cyclic, bilaterally independent upper extremity movements. Patient 2 experiences behavioral arrests, oral and manual automatisms, and left versive head turn with progression to a tonic-clonic convulsion. Patient 3 experiences visual-spatial changes and difficulty speaking progressing to tonic-clonic convulsion.
Seizures consistent with the reported semiology of the focal seizure type were captured on video EEG and confirmed to be focal in onset. In patient 1, seizures were captured on scalp-EEG and onset localized mid-posterior temporally (maximal at T4/T6). In patient 2, seizures were captured on scalp-EEG and onset localized more diffusely mid-temporally (maximal at T3). In patient 3, seizures were captured on stereo-EEG and onset localized to the posterior basal temporal region at the base of the temporal-occipital sulcus.
Patient 2 is two years seizure free of her focal seizures and has an 80% reduction in eyelid myoclonia on cenobamate 300mg daily + VNS. Patient 3 had an RNS system placed (left posterior temporal depth electrode and a left lateral temporal strip electrode). After one year, she notes reduction in focal seizure severity without significant change in seizure frequency. Patient 1 was recently diagnosed, so response to treatment is to be determined.
Conclusions: Jeavons syndrome may include focal seizures. When focal seizures are refractory, these patients may benefit from epilepsy surgery as generally appropriate for focal epilepsy.
Funding: None
Clinical Epilepsy