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Self-limited epilepsy with centrotemporal spikes

（previously known as benign childhood epilepsy with centrotemporal spikes (BCECTS), childhood epilepsy with centrotemporal spikes or Rolandic epilepsy）

Etiologies: It is considered that there is a certain relationship with heredity (some may involve complex polygenic genetic patterns, and some may be related to the mutations of GRIN2A, KCNQ2, ELP4 and other genes. Although some siblings of some children have no clinical seizures, some can also see the discharge of rolandic region at a specific age) [1-3].

Age of onset: onset of seizures between 3 and 14 years old (peak 8-9 years old), most of them remission 2-4 years after onset or before 16 years of age (click to see the proportion of remission in different age stages), 5-15% of children have a history of febrile seizure, it accounts for about 6-20% of children with epilepsy.

Seizure characteristics: It is mainly the focal motor and sensory seizures of the face and oropharynx (low rolandic area) (it can be manifested as the corner of the mouth tilted to one side, accompanied by facial convulsions on this side, sometimes one side of the mouth can be numb, there can be laryngeal chirping or salivation, sometimes the child has clear consciousness but can't speak or can't speak clearly). Seizure can involve the ipsilateral upper limbs or start with one upper limb (high rolandic area), which can progress to bilateral tonic clonic seizures. Most of them occur during sleep (usually shortly after falling asleep or before awakening), and they usually occur infrequently. About 10-20% of children can have only one seizure in their life, and most children will have several seizures, another 20% may have more frequent seizures (but if the seizures are particularly frequent and the therapeutic effect of antiepileptic drugs is not good, it is necessary to carefully evaluate whether the diagnosis is correct and whether there is the possibility of variant)[4-5].

EEG: Background: mostly normal. Interictal: it can be seen that the sharp (spinous) slow wave in rolandic area (the real anatomic rolandic area actually refers to the anterior central gyrus and posterior central gyrus, including the upper region of lateral fissure, which has nothing to do with the temporal lobe. The temporal area mentioned in the name of this syndrome is that the epileptiform discharge in scalp EEG 10-20 system is usually mainly in the middle temporal area and (or) the central area (the area on the scalp EEG of the 10-20 system that reflects the rolandic discharge), which is also related to whether it is in the low rolandic area or the high rolandic area. It can also spread to the parietal area and posterior temporal area, and sometimes the epileptiform discharge can also be seen in the frontal area or occipital area). It can occur synchronously or asynchronously on one side or both sides, which is easy to be induced by blinking, and the epileptiform discharge increases significantly during sleep (about 30% of patients can only see epileptiform discharges during sleep), and the number of sharp (spike) and slow complex waves gradually decreases around puberty in most patients, the amplitude gradually decreases, the waveform becomes blunt, and gradually merges into the background (the Rolandic area discharge that tends to disappear, before and after contrast). Of course, epileptiform discharges in rolandic area can also occur in other non epileptic children or other types of epilepsy, which should be identified in combination with clinical and other EEG characteristics. Sometimes positive frontal sharp (spike) waves synchronized with negative sharp (spike) waves in rolandic region can be seen. After superimposing multiple sharp (spike) waves and calculating by the dipole method, a dipole electric field close to the posterior-anterior horizontal direction can be formed, and the maximum negative potential is mostly located in the middle temporal region and/or central region, the largest positive electric field is located in the ipsilateral or contralateral frontal region. If there is such a constant phenomenon, the possibility of diagnosing this syndrome will be higher (the phenomenon caused by other reasons is rare). Of course, some such syndromes may not exist or may not be constant, and some people think that the prognosis of this phenomenon will be better). Ictal: rhythmic discharge at the beginning of rolandic area can be seen[4-5].

Brain MRI: Most of them are normal.

Developmental progress: Most of them are normal.

Treatment:

National Institute for health and Clinical Excellence (NICE) epilepsy guidelines 2022:

First-line treatment: lamotrigine, levetiracetam (select one of them for monotherapy).

Second-line treatment: If first-line treatments for self-limited epilepsy with centrotemporal spikes are unsuccessful, consider the following as second-line monotherapy treatment options: carbamazepine, oxcarbazepine, zonisamide (If the first choice is unsuccessful, consider the other second-line monotherapy options).

Third-line treatment: If second-line treatments tried are unsuccessful for self-limited epilepsy with centrotemporal spikes, consider sultiame as monotherapy or add-on treatment, but only after discussion with a tertiary paediatric neurologist.

Chinese clinical diagnosis and treatment guidelines epilepsy volume 2015:

First line drugs: carbamazepine, oxcarbazepine, levetiracetam, valproate, topiramate.

Drugs that can be added: carbamazepine, oxcarbazepine, levetiracetam, valproate, topiramate.

Other drugs that can be considered: phenobarbital, phenytoin, zonisamide, pregabalin, tiagabine, vigabatrin, eslicarbazepine, lacosamide.

2022 International League Against Epilepsy (ILAE) diagnostic criteria [6]:

Mandatory: Focal seizures with dysarthria, sialorrhea, dysphasia, and unilateral clonic or tonic–clonic movement of mouth in wakefulness or sleep and/ or nocturnal focal to bilateral tonic–clonic seizures in sleep only. If seizures occur during sleep, they are seen within 1 h of falling asleep or 1–2 h prior to awakening; High-amplitude, centrotemporal biphasic epileptiform abnormalities; Remission by mid to late adolescence. No developmental regression.

Alerts: Focal motor or generalized convulsive status epilepticus for more than 30 minutes. Usual seizure frequency more
than daily. Daytime seizures only; Sustained focal slowing not limited to the postictal phase. Persistently unilateral centrotemporal abnormalities non serial EEGs. Lack of sleep activation of centrotemporal abnormalities; onset age greater than 12 years; Moderate to profound intellectual disability; Hemiparesis or focal neurological findings, other than Todd paresis.

Exclusionary: Generalized tonic–clonic seizures during wakefulness, Atypical absences, Seizures with gustatory hallucinations,
fear, and autonomic features; age of first seizure onset younger than 3 years or older than 14 years; Neurocognitive regression with a
continuous spike-and-wave pattern in sleep (suggests epileptic encephalopathy with spike-and-wave activation in sleep (EE-SWAS)); Causal lesion on brain MRI.

 

References

  1. Lemke, J.R., et al., Mutations in GRIN2A cause idiopathic focal epilepsy with rolandic spikes.Nat Genet, 2013. 45(9): p. 1067-72.
  2. Neubauer, B.A., et al., KCNQ2 and KCNQ3 mutations contribute to different idiopathic epilepsy syndromes. Neurology, 2008. 71(3): p. 177-83.
  3. Epilepsy Res. 2014 Dec;108(10):1734-9. doi: 10.1016/j.eplepsyres.2014.09.005. Epub 2014 Sep 22.PMID: 25301525
  4. Panayiotopoulos.  癫痫综合征及临床治疗.  北京 : 人民卫生出版社, 2012.
  5. 刘晓燕.  临床脑电图学. 第2版.  北京 : 人民卫生出版社, 2017.
  6. Specchio, N., et al., International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions. Epilepsia, 2022.