**7. Atypical rolandic epilepsy**

RE can present or evolve to an atypical form, characterized by atypical ictal semiology, different EEG findings, and poor neuropsychological outcomes [19, 140, 141].

Massa et al. described 5 interictal EEG patterns that significantly correlated with atypical evolutions of RE: [41] intermittent slow-wave focus; [2] multiple asynchronous spike–wave foci; [3] long spike–wave clusters; [4] generalized 3-c/s "absence-like" spike–wave discharges; [1] conjunction of interictal paroxysms with negative or positive myoclonia, and abundance of interictal abnormalities during wakefulness and sleep [136].

Several studies have shown an association between atypical rolandic epilepsy and known genes (**Table 1**). The identification of de novo or inherited mutations of N-methyl-D-aspartate (NMDA) receptor subunit-encoding genes (GRIN2A and GRIN2B) linked to speech and language, cognitive impairment, and behavioral difficulties have been a significant breakthrough in the understanding of the nature of atypical RE [142–145]. Another relevant gene is elongation factor protein 4 (ELP4), which is associated with language impairment, autism spectrum disorder, mental retardation, and epilepsy with centrotemporal spikes on EEG [146].

*Atypical rolandic epilepsy (ARE)* is a severe epileptic condition especially with regards to cognitive consequences. The first description of atypical features of RE was published by Aicardi & Chevrie in 1982 showed rolandic epilepsy presenting periods with new types of seizures, mainly atonic and myoclonic, associated with continuous spike-and-waves in slow-sleep EEG (CSWS/ESES), and transitory learning difficulties [147]. Doose and Baier described similar patients with atonic fits leading to daily falls which is the hallmark seizure type for Lennox–Gastaut syndrome and termed the condition "pseudo-Lennox syndrome" to differentiate this two distinct conditions [148]. Patients with ARE have significantly lower full-scale and verbal IQ than the patients with typical RE [149]. Neuropsychological impairment, which may sometimes be present before the onset of the disease, is constantly present during the clinical course, but in contrast to ESES and LKS, the cognitive outcome is always favorable [92, 150]. Clinical semiology consists of typical for RE focal seizures, generalized tonic–clonic seizures, atypical absences, myoclonic seizures, and atonic seizures. The atonic attacks may involve the whole axial musculature or be localized, causing repeated brief (0.5–2.0 s) atonic episodes in the head or a limb (epileptic negative myoclonus) that usually occur for periods lasting one to several weeks, separated by seizure-free intervals of weeks or months [6, 90, 92]. Such atonic attacks are associated with the slow-wave component of spike and wave complexes, and the location of the EEG discharges corresponds to that of the atonic episodes [151, 152]. Interictal awake EEG shows bilateral sharp and sharp-slow wave complexes with higher amplitude in the rolandic area, which increases during sleep with bilateral synchronization [90, 92, 116, 153].

Using carbamazepine may promote the diffusion of spike–wave activity from the rolandic focus to induce atonic seizures, atypical absences in patients with RE [154].

*Rolandic status epilepticus* refers to status epilepticus that can be convulsive or non-convulsive, and either generalized or focal lasting days or weeks including motor facial seizures, oromotor dyspraxia, anarthria with persistent drooling and swallowing problems [155]. The interictal EEG usually shows focally or bilaterally synchronous sharp waves or sharp and slow wave complexes predominant in the rolandic area with a tendency to become continuous during sleep [146, 155]. The condition can be resolved with a good neurocognitive outcome with appropriate treatment [146]. These seizures can persist for more than 1 month without treatment [156, 157].

## **8. Treatment**

The decision whether to treat children with RE or not requires a particularly careful risk–benefit analysis [2, 158–163]. Many authors suggest that drug treatment is not necessary for typical RE because of its good prognosis, and usually infrequent nocturnal seizures [114, 154]. Moreover, in 40–50% of cases, the seizures are difficult to control with drugs [148]. Besides, the treatment with AED usually does not influence the duration of active epilepsy [163].

However, treatment may be indicated in patients with frequently recurring daytime seizures, generalized tonic–clonic seizures, young age at onset [164], or when the ictal events are disruptive to the patient or family [161, 163]. Furthermore, the presence of cognitive and behavioral disturbances, either transitory or persistent has to be considered [2, 5, 91, 92]. There is no single solution supported by definitive evidence which AED is more effective in the treatment of RE.

Internationally, carbamazepine (CBZ 20-40 mg/kg/d [165]) and valproate (VPA 20-30 mg/kg/d [166]) are the most often prescribed AED for children newly diagnosed RE [167]. However, the possible worsening of EEG in rolandic epilepsy by some drugs and particularly by CBZ, increasing epileptiform abnormalities during sleep, and inducing epileptic negative myoclonus have been reported [154].

Sulthiame, levetiracetam, and gabapentin were studied in a randomized controlled trial [158, 159, 167–169]. Sulthiame administered varied between 3.1 and 5.7 mg/kg/day was effective in controlling seizures in children with RE [159].

A prospective, open-label, pilot trial evaluating the efficacy and tolerability of levetiracetam (LVT 20-30 mg/kg/d) or oxcarbazepine (OXC 20-35 mg/kg/d) as monotherapy in two parallel groups of newly diagnosed RE patients demonstrated effectiveness in controlling seizures a follow-up period up to 2 years [162].

A randomized controlled multicenter trial comparing the effects of either Levetiracetam or Sulthiame on EEG in RE showed a reduction of epileptiform discharges after 12 weeks of treatment [158]. Persistent epileptiform discharges after 12 weeks of treatment are associated with recurrent seizures [158].

When the presence of ESES associated or not with negative myoclonus, clinical status, or acquired aphasia is detected in children with RE, a change of antiepileptic drugs should be considered. Class IV studies suggest that sulthiame, benzodiazepines, ethosuximide, and, in most severe cases, corticosteroids might be useful [91, 92].

Duration of treatment in RE should not exceed 1 year following the last seizure, regardless of EEG changes [2].

#### **Acknowledgements**

We are grateful to Dr. Sándor Beniczky for his valuable comments on EEG figures.

#### **Disclosure**

None of the authors has any conflict of interest to disclose.

*Rolandic Epilepsy: Self-Limited Epilepsy with Centrotemporal Spikes DOI: http://dx.doi.org/10.5772/intechopen.96148*
