Epilepsy With Eyelid Myoclonias (Jeavons Syndrome)

Etiologies: considering that there is a certain relationship with heredity (some may involve complex polygenic genetic patterns, and there are also reports of KCNB1 gene mutation leading to this syndrome), about 40-80% have a family history of seizures or epilepsy [1].

Age of onset: onset of seizures generally from age 2 to 14 years (peak 6 to 8 years).

Seizure characteristics: the incidence rate of female is about two times that of male. The children will have eyelid myoclonias, which can accompany or not with absence seizures, and can be induced by light stimulation. Some of them can be easily induced by eye closure under light conditions, and will not be induced in completely dark environment [2-3].

EEG: Background: normal. Interictal: brief bursts of fast (3-6 Hz) generalized polyspike-and-wave. Ictal: eyelid myoclonia is accompanied by high amplitude generalized spike-and-wave or polyspike-and-wave at a frequency of 3-6 Hz [2-3].

Brain MRI: Most of them are normal.

Developmental progress: Most of them are basically normal, and some individuals may have certain intellectual damage.

Reference

1. Bar, C., et al., Developmental and epilepsy spectrum of KCNB1 encephalopathy with long-term outcome. Epilepsia, 2020. 61(11): p. 2461-2473.
2. Panayiotopoulos.  癫痫综合征及临床治疗.  北京 : 人民卫生出版社, 2012.
3. 刘晓燕.  临床脑电图学. 第2版.  北京 : 人民卫生出版社, 2017.