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Jeavons syndrome featured with visual sensitivity existing as occipital cortex originating focal-to-generalized
Yue Niu1, Pan Gong1, Xianru Jiao1
1Department of Pediatrics, Peking University First Hospital, Beijing, China.
Objective:
To study the relationship between eye closure sensitivity (ECLS), photosensitivity, and the mechanism of Jeavons syndrome (JS).
Methods:
The interictal and the ictal epileptiform discharges (EDs) of 80 patients with electroencephalograms were classified (I: focal posterior EDs; II: the posterior spread into the frontal EDs; and III: generalized EDs) and analyzed under different provoked conditions.
Results:
During the interictal and the ictal period, the positive rates of ECLS were higher than those of photosensitivity (100% vs 57.5%, P = 0.001; 97.5% vs 27.4%, P = 0.001). After a one-year interval, eyelid-myoclonia with ECLS remitted in 16 out of 21 patients (P = 0.002), and eyelid-myoclonia with photo-convulsion response (PCR) disappeared in all the previous six patients (P = 0.021). For the interictal EDs with ECLS, grade I accounted for 11.2%; grade I, II, and III 51.3%; and grade III 32.5%. Interictal EDs classification of photosensitivity showed a similar trend as that of ECLS. For the ictal EDs, grade I accounted for 10.2% of patients with ECLS and 6.7% of patients with PCR; grade I, II, and III, 33.3% of patients with ECLS and 46.6% of patients with PCR; and grade III, 53.9% of patients with ECLS and 40% of patients with PCR.
Conclusion:
ECLS was more common than photosensitivity in JS. Photosensitivity was more likely to disappear than ECLS. Both eye closure and intermittent-photic-stimulation could induce three grades of EDs, confirming that visual stimuli could trigger occipital cortex originating epileptic neural network to varying degrees, which further recognizes JS as another continuum epilepsy.
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