Benign Rolandic Epilepsy

Current Terminology: Self-limited epilepsy with centrotemporal spikes (SeLECTS).
Definition: A common, genetic, age-dependent, self-limited focal epilepsy syndrome characterized by sensorimotor seizures affecting the face/oropharynx, typically occurring during sleep, with distinctive EEG patterns.
Epidemiology:
- Most common childhood focal epilepsy (15–25% of childhood epilepsies).
- Age of Onset: 3 to 13 years (Peak: 7–9 years).
- Sex: Slight male predominance (1.5:1).
- Course: Invariably resolves by adolescence (15–16 years).

Genetics: Complex inheritance pattern.
- Centrotemporal spikes (CTS) on EEG follow an autosomal dominant inheritance with age-dependent penetrance.
- Specific genes: ELP4 (11p13), GRIN2A (linked to speech disorders/epilepsy spectrum).
Pathophysiology: Cortical hyperexcitability in the lower Rolandic area (somatomotor cortex).

Timing: 70–80% occur during sleep (NREM) or upon awakening.
Type: Focal motor/sensory seizures, often evolving to focal to bilateral tonic-clonic seizures (FBTCS).
The "Rolandic" Symptoms (Sylvian Seizures):
- Unilateral facial sensorimotor symptoms: Paresthesia (numbness/tingling) of tongue, gum, cheek, or lips.
- Oropharyngolaryngeal manifestations: Guttural sounds, death rattle-like noises.
- Speech Arrest (Anarthria): Inability to speak despite preserved consciousness (due to motor aphonia, not aphasia).
- Hypersalivation (Sialorrhea): Prominent pooling of saliva.
- Clonic movements: Twitching of one side of the face, mouth, or pharynx.
Consciousness: Usually preserved in focal seizures; lost if secondary generalization occurs.
Frequency: Usually infrequent; 10–20% of patients have only a single seizure.

Normal neurological status.
Normal intelligence (though subtle neurocognitive deficits in language/attention may exist).

Background: Normal.
Interictal Features:
- Morphology: High voltage, blunt, diphasic spikes followed by a slow wave.
- Location: Centrotemporal (C3, C4, T3, T4) or Rolandic area.
- Field: Horizontal Dipole characteristic (positivity in frontal regions, negativity in centrotemporal regions).
- Activation: Spikes significantly increase (by >30%) during NREM sleep.
- Laterality: Can be unilateral, bilateral independent, or shifting side-to-side.

Indication: Not routinely required if clinical picture and EEG are classical.
Recommended if:
- Onset <3 years or >12 years.
- Abnormal neurological exam.
- Drug resistance.
- Atypical EEG features.
Finding: Usually normal.

Panayiotopoulos Syndrome: Autonomic seizures (vomiting), occipital spikes.
Benign Epilepsy with Occipital Paroxysms (Gastaut Type): Visual hallucinations.
Structural Focal Epilepsy: Tumor or cortical dysplasia (ruled out by MRI).
Landau-Kleffner Syndrome / CSWS: If significant language regression or continuous spike-waves in sleep occur.

Counseling parents regarding the benign nature and self-limiting course.
Sleep hygiene maintenance (sleep deprivation triggers seizures).

Observation (No medication): Preferred for:
- First seizure.
- Rare/infrequent seizures (e.g., <2 per year).
- Nocturnal-only seizures causing minimal disruption.
Indication for AEDs:
- Frequent seizures impacting life.
- Daytime seizures (social stigma/risk).
- Secondary generalization (GTCS).
- Co-morbid cognitive/learning issues (controversial).

First Line:
- Carbamazepine: 10–20 mg/kg/day. Highly effective.
- Oxcarbazepine: Better tolerability profile.
- Levetiracetam: Increasingly used due to low side effect profile.
Alternatives: Valproate (if overlap with generalized epilepsy), Sulthiame (common in Europe), Gabapentin.
Drugs to Avoid: Phenytoin, Phenobarbitone (cognitive side effects). Lamotrigine may rarely exacerbate myoclonus or spikes.

"Atypical" Rolandic Epilepsy: Early onset, developmental delay, or atypical EEG.
ESES/CSWS Spectrum: Rare progression to Electrical Status Epilepticus in Sleep leading to cognitive/language regression.
Opercular Syndrome: Drooling, dysarthria, and facial diplegia due to frequent epileptic activity.