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Electroclinical characteristics and syndromic associations of "eye-condition" related visual sensitive epilepsies-A
Kalyani Dilip Karkare1, Ramshekhar N Menon1, Ashalatha Radhakrishnan1
1R Madhavan Nayar Centre for Comprehensive Epilepsy Care, Dept of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
Purpose:
The systematic study of visual phenomena such as eye closure (ECLS), eye-closed/fixation-off sensitivity (FOS) [terminology proposed as eye-condition sensitive (ECS) epilepsy] distinct from photosensitivity is rarely explored in literature.
Methods:
Clinical, electroencephalographic (EEG) and imaging records of patients attending an epilepsy clinic were screened. Inclusion criterion was demonstrable electrographic visual sensitivitiy in the form of ECS parameters with/without photosensitivity. Subsequently, detailed analysis of their data was undertaken.
Results:
Fifty two patients had one or more ECS parameters. Mean age of onset of epilepsy of the cohort was 10.26 ± 7.79 yrs. A family history of seizures was noted in 17% of the cohort. ECLS, FOS, combination of both, co-existent scotosensitivity and photosensitivity were noted in 32.6%, 48.1%, 19.2%, 23.1% and 30.8% respectively. Epilepsy syndromes included late-onset childhood occipital epilepsy-Gastaut variant (23.1%), reflex occipital lobe epilepsy with ECS without photosensitivity (21.2%), idiopathic photosensitive occipital epilepsy (13.5%), genetic generalized epilepsy (13.5%) and symptomatic epilepsy (28.7%) predominated by patients with posterior cortex gliosis or who were imaging negative. The idiopathic group also had lower seizure score and marked sleep activation of epileptiform abnormalities. Co-existent phostosensitivity was associated with a significantly higher predisposition for female gender, ECLS, generalized seizures and spikes with lower prevalence of FOS and extra-occipital spikes. Parieto-occipital spikes were noted in 88.4% patients.
Conclusion:
Syndromic heterogeneity is evident in ECS epilepsies. These form a unique subset of visual-sensitive epilepsy syndromes with focal or generalized seizure subtypes of idiopathic or symptomatic etiologies, with and without associated photosensitivity.