Evolution of electroclinical features in SCN1A positive Dravet syndrome patients across the lifespan
Abstract number :
3.111
Submission category :
3. Neurophysiology / 3C. Other Clinical EEG
Year :
2016
Submission ID :
197898
Source :
www.aesnet.org
Presentation date :
12/5/2016 12:00:00 AM
Published date :
Nov 21, 2016, 18:00 PM
Authors :
Shimrit Uliel-Sibony, British Columbia children's hospital, Vancouver, BC, Canada., Vancouver, Canada; Linda Huh, British Columbia children's hospital, Vancouver, BC, Canada.; Fiona Cave, British Columbia children's hospital, Vancouver, BC, Canada.; Peter
Rationale: Rationale: To characterize the evolution of the electroclinical features in patients with Dravet syndrome. Methods: Methods: Retrospective review of 24 patients (14 males) aged 2-29 years (mean15.4 y, 9 >18y) with Dravet syndrome who were SCN1A positive. The study population was subdivided into 3 groups for data analysis; at presentation and during the first year, from 2nd to 5th year and from 6th year onwards. Results: Results: Seizures were recorded in 19/24 (79%) patients. 38 EEG recordings were available on 23 patients in the first year of life and showed abnormal background and epileptiform discharges in 21/38 (55%) recordings, mainly from 6-12 months. Myoclonic seizures were recorded in 5 patients associated with spikes and generalized spike and wave activity; a photoconvulsive response was observed in 3 patients. From the 2nd year onwards, background dysrhythmia and epileptiform discharges were more frequent. Spikes and sharp waves were most common followed by generalized spike wave. Spike and wave and polyspike wave was seen in only 24% of interictal recordings.143 seizures were recorded in 12 patients aged 2-5 years. Myoclonic seizures were the most common, 95/143 EEGs, (6 patients). Five patients had 37 focal seizures. The ictal EEG changes were located most commonly over the bifrontal regions. From age 6 onwards, 315 seizures were recorded in 8 patients. Tonic seizures were the most frequent but focal seizures were still common. Overall 171 tonic seizures were recorded in 4 patients; one patient had 114 tonic seizures. The ictal EEG was characterized by bilateral frontal or generalized rhythmic beta/spikes. Conclusions: Conclusion: In contrast to the published literature, EEG background was slowed and interictal epileptiform discharges were common in the first year of life in our series. Myoclonic seizures were the most frequent seizure type in the first year of life, followed by focal and tonic seizures with increasing age. Funding: There was no funding that has been received for this abstract
Neurophysiology