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Jeavons syndrome featured with visual sensitivity existing as occipital cortex originating focal-to-generalized continuum epilepsy

      Highlights

      • Classify and analyze the interictal and ictal EDs with ECLS and photosensitivity.
      • Explain and demonstrate the epileptic neural network of JS.
      • ECLS was more common than photosensitivity in JS.
      • Photosensitivity was more likely to disappear than ECLS after ASMs.

      Abstract

      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.

      Keywords

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