Atypical Childhood Epilepsy With Centrotemporal Spikes

(formerly known as BECT variant type 1, also known as atypical benign partial epilepsy of childhood, atonic benign childhood epilepsy with centrotemporal spikes, or pseudo Lennox syndrome)

Etiologies: consider genetic correlation (some mutations of GRIN2A gene are found, and others may involve complex polygenic genetic patterns) [1].

Age of onset: onset of seizures generally from age 2 to 8 years (peak 5 to 6 years).

Seizure characteristics: it can have a variety of seizure types, often including atonic seizures and nocturnal focal seizures, as well as generalized tonic clonic seizures and absence seizures (Note: in fact, some are not true absence seizures, but pseudo absence, which still belongs to focal seizures. It is more appropriate to describe them with dialeptic seizure (use symptomatic classification), usually paroxysmal sluggishness, reduced consciousness, but rarely complete loss) [2-3].

EEG: Background: it can be normal, focal or diffuse slowing. Interictal: it can manifest as spike (sharp) and wave in rolandic area, usually bilateral. Persistent spikes (sharp) and waves (SWI index can reach 50-90%) can appear during NREM sleep, which are easy to be seen mainly in the active period of atonic seizure. Ictal: local transient atonic seizures (negative myoclonus) can manifest as a single spike (sharp) and wave in the contralateral rolandic area. When the symptoms progress to the onset of atonic or atonic absence seizures, the focal epileptiform discharge can be generalized to comprehensive discharge. Some dialeptic seizures can show a large number of spike (sharp) and wave in bilateral rolandic area during awake period, which often spread to the whole brain and show semi rhythmic paroxysms (pseudo absence seizure) (Note: sometimes it is easy to take it as the interictal discharge without watching the video carefully, and sometimes children can often manifest as frequent blinking (in fact, in most cases, blinking is the inducement of rolandic area discharge, not the seizure performance caused by discharge) [2-3].

Brain MRI: Most of them are normal.

Developmental progress: Most of them are basically normal. They are often normal before the seizure onset. There may be some cognitive damage in the active stage of the seizure, which can be alleviated or disappeared after remission.

Reference

Lemke, J.R., et al., Mutations in GRIN2A cause idiopathic focal epilepsy with rolandic spikes.Nat Genet, 2013. 45(9): p. 1067-72.
Panayiotopoulos. 癫痫综合征及临床治疗. 北京 : 人民卫生出版社, 2012.
刘晓燕. 临床脑电图学. 第2版. 北京 : 人民卫生出版社, 2017.