PEDBASE.org - The Pediatric Database - Detailed information of BENIGN PARTIAL EPILEPSY WITH CENTROTEMPORAL SPIKES (BPEC)

BENIGN PARTIAL EPILEPSY WITH CENTROTEMPORAL SPIKES (BPEC)

DEFINITION:

An epileptic syndrome characterized by simple partial seizures with motor and somatosensory symptoms confined to the face and extremities.

EPIDEMIOLOGY:

incidence: 1.4/100,000
- most frequent childhood epilepsy
- occurs in 24% of children with epilepsy
age of onset:
- 3 to 13 years with peak at 9-10 years
risk factors:
- M > F (3:2)
- family history of epilepsy in 1st
- degree relatives in 30% of cases

PATHOGENESIS:

1. Background

defined by the International Classification of Epilepsy as an idiopathic localization-related epileptic syndrome
- no underlying cause
- localized origin of seizure
usually idiopathic with no underlying structural lesions

2. Seizure Characteristics

most common clinical presentation
- at night in a 9-10 yr old boy who suddenly wakes up 2-3 hours after falling asleep. The seizure lasts 1-3 minutes and is characterized by gutteral sounds, unilateral twitch-ing of facial muscles, and unintelligible speech
usually occur without march

CLINICAL FEATURES:

1. Simple Partial Seizures (80%)

1. SPS with Focal Motor Phenomenon (70%)

1. Facial-Oral-Pharyngeal Musculature (77%)

dysarthria, drooling, speech arrest
tonic or clonic contractions of one side of face

2. Upper (20%) and Lower (8%) Extremity Muscles

clonic jerks, dystonic posturing, clumsiness of hand or foot
may be accompanied by a Todd's paralysis

2. SPS with Somatosensory Symptoms (10%)

tingling, unilateral numbness of cheek, burning, cold, pain, paresthesias of the tongue, or a sense of movement in any part of the body (usually the face)

2. Generalized Tonic-Clonic Seizures (20%)

INVESTIGATIONS:

1. EEG

1. Centrotemporal Spikes

1. Interictal

unilateral (70-80%) and/or bilateral (15-30%) blunt high voltage centrotemporal spikes often followed by slow waves that are activated by sleep and tend to spread or shift from side to side (8%)

2. Background

normal

2. Imaging Studies

1. CT/MRI

normal

MANAGEMENT:

1. Antiepileptic Medications

indications:
- after 2nd or 3rd seizure
- parental pressure

1. Carbamazepine (Tegretol)

drug of choice
10 mg/kg/day po q12h and increased gradually until seizure control or side effects
will control seizures in 80% of patients within 1 month

2. Valproic Acid

2nd drug of choice
30 mg/kg/day po q12h
in most cases will control seizures in the remaining 20%

3. Dilantin

5 mg/kg/day po q12h
duration: 1-2 years

2. Prognosis

1. Remission

spontaneous remission usually occurs before ages 15-16 yrs
high remission rate (75%) after a 2 year seizure-free period on antiepileptic medication
excellent prognosis

2. Recurrence rate

25% and occurs in clusters

Pediatric Database - BENIGN PARTIAL EPILEPSY WITH CENTROTEMPORAL SPIKES (BPEC)

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