Benign childhood epilepsy with centrotemporal EEG spikes - Clinicals, Diagnosis, and Management

Neurology

Clinicals - History

Fact Explanation
Introduction Seizure is an abnormal paroxysmal discharge from set of neurons in the brain which is sufficient cause clinical manifestation. Epilepsy is a tendency to have recurrent, unprovoked seizures. It may manifest as generalized, secondarily generalized or focal seizures. Benign epilepsy with centro-temporal spikes (BECTS) is one of the most frequent epileptic syndromes in children which is considered under the idiopathic localization-related epilepsies. Origin will be related to the motor and language area of the brain and is from the rolandic area of the brain. It has a characteristic specific age of onset, seizure pattern, neurodevelopmental profile, and electroencephalographic findings. Its incidence is reported as 21 in 100000 of 5-20 years of age. Introduction
Seizure is an abnormal paroxysmal discharge from set of neurons in the brain which is sufficient cause clinical manifestation. Epilepsy is a tendency to have recurrent, unprovoked seizures. It may manifest as generalized, secondarily generalized or focal seizures. Benign epilepsy with centro-temporal spikes (BECTS) is one of the most frequent epileptic syndromes in children which is considered under the idiopathic localization-related epilepsies. Origin will be related to the motor and language area of the brain and is from the rolandic area of the brain. It has a characteristic specific age of onset, seizure pattern, neurodevelopmental profile, and electroencephalographic findings. Its incidence is reported as 21 in 100000 of 5-20 years of age.
Age 75% of children develop the condition between 7 and 10 years. It is more common in boys than girls. Age
75% of children develop the condition between 7 and 10 years. It is more common in boys than girls.
Timing and duration Mostly seizures are occurring during NREM (Non-Rapid Eye Movement) sleep, mainly at sleep onset or just before awakening. Some will develop while awake and others will have seizures during both sleep and awake states. Seizures are very brief lasting only for 1-3 minutes. Timing and duration
Mostly seizures are occurring during NREM (Non-Rapid Eye Movement) sleep, mainly at sleep onset or just before awakening. Some will develop while awake and others will have seizures during both sleep and awake states. Seizures are very brief lasting only for 1-3 minutes.
Frequency of seizures Some may have only a single seizure during the entire course, some will have infrequent seizures, others have frequent seizures. Occasionally multiple seizures per day may be seen. Frequency of seizures
Some may have only a single seizure during the entire course, some will have infrequent seizures, others have frequent seizures. Occasionally multiple seizures per day may be seen.
Abnormal motor and somatosensory manifestations Focal seizures may occur and mainly begins in the face and oropharynx, sometimes upper limb is also involved. Seizures may spread from the rolandic area to the rest of the brain causing a secondarily generalized seizure. They may be tonic or tonic-clonic seizures. Mostly the tongue and occasionally of the lips, gums, inner cheeks or even a single tooth can be involved. Manifestation may be in the form of face or cheek twitching, numbness, tingling, , or unusual sensations in the face or tongue. Hemifacial sensory-motor seizures localized in the lower lip may spread to the ipsilateral hand. Clonic contractions sometimes concurrent with ipsilateral tonic deviation of the mouth. Somatosensory aura is common. Abnormal motor and somatosensory manifestations
Focal seizures may occur and mainly begins in the face and oropharynx, sometimes upper limb is also involved. Seizures may spread from the rolandic area to the rest of the brain causing a secondarily generalized seizure. They may be tonic or tonic-clonic seizures. Mostly the tongue and occasionally of the lips, gums, inner cheeks or even a single tooth can be involved. Manifestation may be in the form of face or cheek twitching, numbness, tingling, , or unusual sensations in the face or tongue. Hemifacial sensory-motor seizures localized in the lower lip may spread to the ipsilateral hand. Clonic contractions sometimes concurrent with ipsilateral tonic deviation of the mouth. Somatosensory aura is common.
Consciousness Consciousness is not impaired in most cases. Consciousness
Consciousness is not impaired in most cases.
Other associated features Seizure may be associated with gurgling/grunting noises, speech arrest which may be due to the inability to control the mouth muscles. Hypersalivation is a prominent autonomic manifestation associated with hemifacial seizures. Other associated features
Seizure may be associated with gurgling/grunting noises, speech arrest which may be due to the inability to control the mouth muscles. Hypersalivation is a prominent autonomic manifestation associated with hemifacial seizures.
Forgetfulness, lack of concentration on work and learning problems Recent evidence suggests that there is an impairment of cognitive functioning, concentration, and short-term memory, leading to poor school performance. Some studies suggest that the frontal lobe dysfunction is associated with these symptoms. Forgetfulness, lack of concentration on work and learning problems
Recent evidence suggests that there is an impairment of cognitive functioning, concentration, and short-term memory, leading to poor school performance. Some studies suggest that the frontal lobe dysfunction is associated with these symptoms.
Atypical features Though the condition is benign, nearly 10 to 50% may have diurnal seizures, screaming, aura, and Todd’s paralysis. Focal motor, hemiconvulsive and generalized convulsive status epilepticus are rare occuring nearly in 5% of the patients. Atypical features
Though the condition is benign, nearly 10 to 50% may have diurnal seizures, screaming, aura, and Todd’s paralysis. Focal motor, hemiconvulsive and generalized convulsive status epilepticus are rare occuring nearly in 5% of the patients.
Headache and migraine Headache may originate in the terminal nervous fibers (“vasomotor”) in cerebral blood vessels and consequently, headache is classified as an “autonomic” sensation. The epilepsy and migraine, are known to have common pathogenetic mechanisms that are related to the dysfunction of ion channels. Headache and migraine
Headache may originate in the terminal nervous fibers (“vasomotor”) in cerebral blood vessels and consequently, headache is classified as an “autonomic” sensation. The epilepsy and migraine, are known to have common pathogenetic mechanisms that are related to the dysfunction of ion channels.
Psychic symptoms, complex automatisms, amnesia and post-ictal confusion These features are not present in benign epilepsy with centro-temporal spikes and are important to differentiate it from the other seizure syndromes. Psychic symptoms, complex automatisms, amnesia and post-ictal confusion
These features are not present in benign epilepsy with centro-temporal spikes and are important to differentiate it from the other seizure syndromes.
Family history Though there are no genes has been identified, some studies have suggested an autosomal dominant inheritance. Family history
Though there are no genes has been identified, some studies have suggested an autosomal dominant inheritance.

Clinicals - Examination

Fact Explanation
Motor manifestations They can have focal or secondarily generalised seizures manifesting with the involvement of face and oropharynx. Mostly the tongue and occasionally of the lips, gums, inner cheeks or even a single tooth can be involved. Manifestation may be in the form of face or cheek twitching, or tingling. Motor manifestations
They can have focal or secondarily generalised seizures manifesting with the involvement of face and oropharynx. Mostly the tongue and occasionally of the lips, gums, inner cheeks or even a single tooth can be involved. Manifestation may be in the form of face or cheek twitching, or tingling.
Visuomotor skills, visuospatial memory and skills, language, and attention Children with benign epilepsy of childhood with centrotemporal spikes may have problems in certain areas such as visuomotor skills, visuospatial memory and skills, language, short term memory, concentration and attention. These may be transient and resolved with time coming back to completely normal after remission. Visuomotor skills, visuospatial memory and skills, language, and attention
Children with benign epilepsy of childhood with centrotemporal spikes may have problems in certain areas such as visuomotor skills, visuospatial memory and skills, language, short term memory, concentration and attention. These may be transient and resolved with time coming back to completely normal after remission.
Developmental abnormality Usually there are no developmental abnormalities. Developmental abnormality
Usually there are no developmental abnormalities.
Focal neurological deficits, cranial nerve examination Usually there are no evidence of focal structural disease. Therefore neurological abnormalities are not found on examination. Focal neurological deficits, cranial nerve examination
Usually there are no evidence of focal structural disease. Therefore neurological abnormalities are not found on examination.

Investigations - Diagnosis

Fact Explanation
Electroencephalogram There is a characteristic EEG findings which will be either high-voltage spikes or spike and waves in the centrotemporal region that may shift from side to side with a normal background. They might be either unifocal or bifocal. Centrotemporal spikes are maximal during drowsiness and sleep. Although called centro-temporal, these are mainly localized in the C3 and C4 (high central) or C5 and C6 (low central) supra-sylvian regeions, and not temporal electrodes. Electroencephalogram
There is a characteristic EEG findings which will be either high-voltage spikes or spike and waves in the centrotemporal region that may shift from side to side with a normal background. They might be either unifocal or bifocal. Centrotemporal spikes are maximal during drowsiness and sleep. Although called centro-temporal, these are mainly localized in the C3 and C4 (high central) or C5 and C6 (low central) supra-sylvian regeions, and not temporal electrodes.
Functional magnetic resonance imaging (fMRI) This is important to identify the focal cortical activation during partial seizures or interictal epileptiform discharges and to detect the cerebral localization or origin of focal spike discharges. This shows signal activation in the face area of the sensorimotor cortex, which is the supplementary motor area that is involved in attention and concentration. Functional magnetic resonance imaging (fMRI)
This is important to identify the focal cortical activation during partial seizures or interictal epileptiform discharges and to detect the cerebral localization or origin of focal spike discharges. This shows signal activation in the face area of the sensorimotor cortex, which is the supplementary motor area that is involved in attention and concentration.

Investigations - Management

Fact Explanation
Electroencephalogram EEG will come back to normal with the remission of the epilepsy usually during the adolescence. Electroencephalogram
EEG will come back to normal with the remission of the epilepsy usually during the adolescence.
Neuropsychological tests These tests may be done at the initial visit and during the follow up to assess multiple cognitive functions, including selective and sustained attention (visual, auditory), executive function, auditory-verbal learning, visual-spatial skills, auditory and verbal memory, receptive and expressive language, and motor function. Neuropsychological tests
These tests may be done at the initial visit and during the follow up to assess multiple cognitive functions, including selective and sustained attention (visual, auditory), executive function, auditory-verbal learning, visual-spatial skills, auditory and verbal memory, receptive and expressive language, and motor function.
Complete blood count This is important if the patient is started on antiepileptic drug therapy. Thrombocytopenia, agranulocytosis, and aplastic anemia are recognized associations with the carbamazepine therapy. Discontinuation of carbamazepine is recomendeed if WBC less than 3000 mm3, absolute neutrophil count (ANC) less than 1500 mm3, or platelet count less than 100,000 per mm3. Complete blood count
This is important if the patient is started on antiepileptic drug therapy. Thrombocytopenia, agranulocytosis, and aplastic anemia are recognized associations with the carbamazepine therapy. Discontinuation of carbamazepine is recomendeed if WBC less than 3000 mm3, absolute neutrophil count (ANC) less than 1500 mm3, or platelet count less than 100,000 per mm3.
Assessment of visuomotor skills, visuospatial memory and skills, language, and attention Children with benign epilepsy of childhood with centrotemporal spikes may have problems in certain areas such as visuomotor skills, visuospatial memory and skills, language, and attention. These may be transient and resolved with time coming back to completely normal after remission. Assessment of visuomotor skills, visuospatial memory and skills, language, and attention
Children with benign epilepsy of childhood with centrotemporal spikes may have problems in certain areas such as visuomotor skills, visuospatial memory and skills, language, and attention. These may be transient and resolved with time coming back to completely normal after remission.
CT scan and MRI scan Neuro-imaging is normal as there are no structural lesions in the brain. But this may be helpful in excluding the organic causes of seizures. CT scan and MRI scan
Neuro-imaging is normal as there are no structural lesions in the brain. But this may be helpful in excluding the organic causes of seizures.

Management - Supportive

Fact Explanation
Patient and parent education Parents and child should be educated about the nature of the disease, particularly benign course of the disease, and excellent prognostic outcome. Almost all patients will remit by the age of 14. Patient and parent education
Parents and child should be educated about the nature of the disease, particularly benign course of the disease, and excellent prognostic outcome. Almost all patients will remit by the age of 14.
Management of associated problems Some children may have learning difficulties due to poor short term memory and loss of concentration. They will also have behavioral problems. Children with these difficulties in school or in social functioning should have formal neuropsychometric testing to identify specific problem and specific interventions where needed. Speech therapy is needed in children with speech and language problems.
Particularly in the school system, there should be changes that are likely to be helpful in children with behavioural disorders and attention problems associated with rolandic epilepsy. eg:- classroom observation by a counselor, parent and teacher behavior rating scales, consideration of speech/language evaluation, screening intelligence testing
Management of associated problems
Some children may have learning difficulties due to poor short term memory and loss of concentration. They will also have behavioral problems. Children with these difficulties in school or in social functioning should have formal neuropsychometric testing to identify specific problem and specific interventions where needed. Speech therapy is needed in children with speech and language problems.
Particularly in the school system, there should be changes that are likely to be helpful in children with behavioural disorders and attention problems associated with rolandic epilepsy. eg:- classroom observation by a counselor, parent and teacher behavior rating scales, consideration of speech/language evaluation, screening intelligence testing

Management - Specific

Fact Explanation
Spontaneous resolution with no treatment Benign epilepsy with centro-temporal spikes has an excellent prognosis with a good neurodevelopmental outcome. The syndrome of benign rolandic epilepsy always remits and is able to manage without antiepileptic drug therapy. Children with infrequent seizures, mild or nocturnal, onset close to the natural age of remission may not require antiepileptic therapy. They will become seizure free by the age of 14. Spontaneous resolution with no treatment
Benign epilepsy with centro-temporal spikes has an excellent prognosis with a good neurodevelopmental outcome. The syndrome of benign rolandic epilepsy always remits and is able to manage without antiepileptic drug therapy. Children with infrequent seizures, mild or nocturnal, onset close to the natural age of remission may not require antiepileptic therapy. They will become seizure free by the age of 14.
Antiepileptics Where needed, Carbamazepine or Oxcarbazepine are the first line drugs to be used. Benign epilepsy with centro-temporal spikes associated with bilateral discharges on EEG are usually have a good response to Sodium Valproate or Carbamazepine or Oxcarbazepine, unilateral discharges on EEG will be best treated with Carbamazepine or Oxcarbazepine. Studies have shown that the drugs like carbamazepine hes a positive effect on cognition with improvement in memory in children with benign rolandic epilepsy. Antiepileptics
Where needed, Carbamazepine or Oxcarbazepine are the first line drugs to be used. Benign epilepsy with centro-temporal spikes associated with bilateral discharges on EEG are usually have a good response to Sodium Valproate or Carbamazepine or Oxcarbazepine, unilateral discharges on EEG will be best treated with Carbamazepine or Oxcarbazepine. Studies have shown that the drugs like carbamazepine hes a positive effect on cognition with improvement in memory in children with benign rolandic epilepsy.

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