Rolandic Epilepsy: Clinical and EEG Hallmarks
Rolandic epilepsy (benign childhood epilepsy with centrotemporal spikes) is diagnosed by the combination of brief focal seizures with orofacial involvement occurring in a neurologically normal child aged 3-13 years, paired with the pathognomonic EEG finding of high-voltage sharp spikes in the centrotemporal region that are activated by drowsiness and sleep. 1
Clinical Presentation
Seizure Characteristics
- Brief, simple partial seizures involving the lower face with paresthesias (tingling sensations), tonic or clonic activity of the facial muscles, mouth deviation, drooling, and dysarthria (speech difficulty) 2, 3
- Nocturnal predominance: More than half of children experience seizures exclusively during sleep 4
- Secondary generalization is common: Approximately 60% of children develop generalized tonic-clonic seizures, while 40% have purely focal attacks 4
- Seizures are typically infrequent and easily controlled with antiepileptic medications when treatment is required 3
Age and Demographics
- Onset between ages 3-13 years, with peak incidence at 7-10 years 2, 3, 4
- Male predominance is observed 2
- Genetic predisposition is frequent in affected families 2
Neurological Status
- Children are neurologically and cognitively normal on examination, which is a defining feature distinguishing this from symptomatic focal epilepsies 3
EEG Hallmarks
Characteristic Interictal Findings
- Blunt, high-voltage sharp waves (spikes) in the centrotemporal (Rolandic) regions are the pathognomonic EEG feature 1, 2
- Spikes are often followed by slow waves 2
- Activation by drowsiness and sleep is a critical diagnostic feature—the spikes become more prominent and frequent during these states 1, 3
- Spikes tend to shift or spread from side to side, with bilateral independent centrotemporal discharges seen in some patients 2, 5
Functional Connectivity Changes
- Widespread increases in EEG coherence occur during spike epochs, involving theta, alpha, and beta bandwidths bilaterally and extending beyond the centrotemporal region 6
- Generalized increases in spectral power at all frequencies (particularly delta, theta, and alpha) are seen during spike activity, suggesting this may functionally resemble a generalized rather than purely focal disorder 6
Diagnostic Algorithm
When Imaging Is NOT Required
- Neuroimaging is not routinely necessary when clinical features are typical (brief orofacial seizures, nocturnal predominance, normal neurological examination) AND EEG shows characteristic centrotemporal spikes 1, 3
Red Flags Requiring MRI
Despite typical EEG findings, obtain MRI with epilepsy protocol if ANY atypical features are present: 5
- Abnormal neurological examination
- Cognitive or motor deficits
- Failure to return to baseline within several hours after seizure 1
- Seizures that are difficult to control
- Atypical seizure semiology (e.g., not orofacial/hemifacial pattern)
Common pitfall: Five documented cases of focal brain lesions (low-grade astrocytomas, cavernous angiomas) initially presented with clinical and EEG features suggestive of Rolandic epilepsy, but careful review revealed subtle atypical features in each case 5. This underscores the importance of thorough clinical assessment before forgoing neuroimaging.
Prognosis
- Excellent long-term outcome: Essentially all children enter long-term remission by mid-adolescence (age 15-16 years) 2, 3
- More than 50% become seizure-free after initiating antiepileptic therapy 4
- Three-quarters achieve practical cure (defined as attack-free period of at least 5 years) 4
- EEG normalizes in over 80% of cases within three years of follow-up, with complete disappearance of centrotemporal spikes 4