Photosensitivity in Dravet syndrome is under-recognized and related to prognosis.
Clin Neurophysiol
; 128(2): 323-330, 2017 02.
Article
em En
| MEDLINE
| ID: mdl-28042998
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 2-19years). Detailed questionnaires on visual stimuli were completed by parents (n=49).RESULTS:
PPR was found in 22 patients (42%; median age 1.25yr), 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.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Epilepsias Mioclônicas
/
Epilepsia Reflexa
Tipo de estudo:
Guideline
/
Prognostic_studies
Limite:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
Idioma:
En
Ano de publicação:
2017
Tipo de documento:
Article