Epilepsy with eyelid myoclonia: A systematic review and meta-analysis.

BACKGROUND

Epilepsy with eyelid myoclonia (EEM), previously known as Jeavons syndrome, is an under-recognized idiopathic generalized epilepsy characterized by eyelid myoclonia, often triggered by photic stimulation or eye closure. Despite its classification under absence epilepsies, the clinical and neurophysiological spectrum of EEM remains heterogeneous.

METHODS

A systematic review and meta-analysis were conducted following PRISMA guidelines. Databases including PubMed, Scopus, and Cochrane were searched from inception until January 16, 2025. Eligible studies reported clinical, electrophysiological, and treatment outcomes in patients with confirmed EEM. Pooled proportions and means were estimated using random-effects models.

RESULTS

Twenty-eight studies encompassing 1,484 patients were analyzed. EEM predominantly affected females (69.35 %, 95 % CI: 65.53 %-72.93 %). The mean age at onset was 9.09 years (95 % CI: 8.02-10.16). Absence seizures were present in 55.61 % (95 % CI: 39.76 %-70.4 %), while generalized tonic-clonic seizures occurred in 52.65 % (95 % CI: 43.08 %-62.02 %). Photosensitivity was the most frequent trigger (75.91 %, 95 % CI: 60.11 %-86.82 %). Generalized spike-wave discharges were observed in 81.13 % (95 % CI: 70.91 %-88.35 %). Drug resistance was reported in 31.73 % (95 % CI: 17.36 %-50.67 %), with patients trialing a mean of 3.28 (95 % CI: 2.14-4.42) anti-epileptic drugs. Follow-up duration averaged 7.25 years (95 % CI: 5.67-8.82).

CONCLUSION

EEM remains a distinct but clinically variable epilepsy syndrome. The high prevalence of photosensitivity and frequent misclassification highlight the need for improved diagnostic criteria. Despite treatment, seizure persistence is common, necessitating individualized therapy. Future research should explore genetic underpinnings and non-pharmacological interventions.