Photosensitivity in Dravet syndrome is under-recognized and related to prognosis. Issue 2 (February 2017)

• Record Type: Journal Article
• Authors: Verbeek, Nienke; Kasteleijn-Nolst Trenité, Dorothée; Wassenaar, Merel; van Campen, Jolien; Sonsma, Anja; Gunning, W. Boudewijn; de Weerd, Al; Knoers, Nine; Spetgens, Willy; Gutter, Thea; Leijten, Frans; Brilstra, Eva
• Is Part Of: Clinical neurophysiology. Volume 128:Issue 2(2017:Feb.)
• Page Start: 323
• Page End: 330
• Publication Date: 2017-02
• Subjects: Photoparoxysmal EEG response -- PPR -- SCN1A -- Pattern stimulation -- Self-induction -- Prevalence; Neurophysiology -- Periodicals; Electroencephalography -- Periodicals; Electromyography -- Periodicals; Neurology -- Periodicals; 612.8
• DOI: 10.1016/j.clinph.2016.11.021
• Languages: English
• ISSNs: 1388-2457

Abstract

Highlights: Photoparoxysmal EEG responses (PPR) are very common (42%) in patients with Dravet syndrome (DS). PPR in DS occurs at a strikingly young age and seems to be connected with a more severe course. Repetitive intermittent photic stimulation with optimal protocol increases detection rate of PPR. Abstract: Objective: To detect determinants for photoparoxysmal EEG response (PPR) in SCN1A -related Dravet syndrome (DS). Methods: Data were studied from nationwide medical histories and EEGs of DS-patients ( n = 53; 31 males, age 2–19 years). Detailed questionnaires on visual stimuli were completed by parents ( n = 49). Results: PPR was found in 22 patients (42%; median age 1.25 yr), and repeatedly in 17%. PPR (17% of 249 intermittent photic stimulation (IPS)-EEGs) occurred more often with optimal IPS protocols (OR 2.11 [95%CI 1.09–4.13]) and in EEGs showing spontaneous epileptiform abnormalities (OR 5.08 [95%CI 2.05–12.55]). PPR-positive patients tended to be younger at first ( p = 0.072) and second seizure ( p = 0.049), showed severe intellectual disability ( p = 0.042), and had more often spontaneous occipital epileptiform abnormalities ( p < 0.001). Clinical sensitivity was reported in medical files in 22% of patients and by parents in 43% (self-induction 24%). Clinical or EEG proven visual sensitivity was detected in 65% of cases. Conclusions: Sensitivity to visual stimuli is very common in DS and more often noticed by parents than confirmed by EEG. Detection of PPR improves with repetitive tests using accurate IPS protocols. Significance: Photosensitivity is an important feature in DS and seems to be a marker of the severity of the disorder. Therefore repeated standardized IPS should be encouraged.