Overview.
Benign epilepsy syndromes are neurological disorders that resolve on their own, or are better treated with antiepileptic drugs and have a favorable prognosis. The onset of epilepsy occurs in childhood and adolescence, and the seizures resolve with age and may stop after a certain age. Seizures are slightly more common in boys than in girls.
Causes
The cause of benign epilepsy syndrome is unknown and is closely related to genetic factors and age.
Symptoms
1. Benign familial neonatal epilepsy
This disease is a rare autosomal dominant disorder. It is mainly characterized by tonic, clonic convulsive seizures, often combined with autonomic symptoms and motor automatisms, occurring shortly after birth (mostly within 7 days) in normal full-term newborns, and the seizures are frequent and brief. In the interictal period, the child is generally in good condition. The prognosis is good, and most of the convulsive seizures disappear within 24 weeks.
2. Benign infantile epilepsy
This disease was also known as benign infantile convulsions, its clinical characteristics are the age of the first 3 to 20 months, with or without a family history of benign infantile epilepsy, before and after the onset of normal psychomotor development, the performance of focal seizures or secondary generalized seizures, seizures are often in clusters, there is no epileptic status epilepticus. Cranial imaging was unremarkable. The disease is well treated with antiepileptic drugs, and the prognosis is good with no further seizures after 2 years of age.
3. Benign infantile myoclonic epilepsy
It is a rare clinical epilepsy syndrome. It is mainly characterized by generalized myoclonic seizures occurring at the age of 1 to 2 years (before the age of 3 years), basically not accompanied by other types of seizures. The seizures are easy to control, growth and development are normal, and the prognosis is good.
4. Benign epilepsy with central temporal spikes in children
It is also known as benign Rolandic epilepsy. It is the most common epilepsy syndrome in childhood, with obvious age dependence, and most patients have onset at the age of 5 to 10 years. It is mainly characterized by focal motor and sensory seizures of the face and oropharynx, occasionally secondary to generalized seizures. Most children have seizures only during sleep, and they are usually infrequent. The prognosis is favorable, with almost all patients resolving by age 16.
5. Panayiotopoulos syndrome
It is also known as early-onset childhood benign occipital lobe epilepsy (Panayiotopoulos type). It develops in early to middle childhood and is characterized by autonomic symptomatic seizures with vomiting as the main symptom and persistent seizures. It is generally believed that the onset of the disease is genetically related, and the prognosis is good.
6. Childhood catatonic epilepsy
It is a common idiopathic generalized epilepsy syndrome in childhood, which is genetically related to the onset of epilepsy and usually starts at the age of 4 to 10 years. Clinical manifestations include frequent and typical atonic seizures. The children have normal physical and intellectual development, and the disease usually resolves before the age of 12, with a good prognosis.
7. Adolescent atonic epilepsy
The age of onset is mostly between 7 and 16 years old, with a peak between 10 and 12 years old. The main clinical features are typical apoplectic seizures, with generalized tonic-clonic seizures in about 80% of the children, and myoclonic seizures in about 15% of the children. Most of the children remit after treatment and have a good prognosis.
Examination
1. Electroencephalography
Electroencephalography is the most important test for diagnosing epilepsy. It helps to determine the type of epilepsy and locate the epileptogenic foci by detecting abnormal neuronal discharges (epileptiform discharges) in the brain. Most of the interictal electroencephalograms (EEGs) are normal, but there may be abnormalities such as generalized spiking-slowing wave or focal spiking discharges during the seizure period.
2. Neuroimaging examination
(1) CT and magnetic resonance imaging (MRI): children with benign epilepsy syndrome do not have organic changes in the brain, so the results of cranial CT and MRI are normal, which can help to differentiate epilepsy from epilepsy caused by intracranial lesions.
(2) Positron emission computed tomography (PET) examination of the brain: it can detect localized abnormal metabolic changes in the brain, which helps to localize the epileptic foci.
Diagnosis
Diagnosis can be made on the basis of medical history and the manifestations of epileptic seizures, combined with electroencephalography and so on. The diagnosis is based on the following.
1. There are seizure manifestations of benign epilepsy syndrome, such as convulsions of the corners of the mouth, dysphagia, strange noises coming from the throat, visual hallucinations, and clonic jerks of the limbs.
2. Some children have family history.
3. EEG during seizure has characteristic epileptiform discharges, which may be manifested as generalized spike-slow complex wave, or focal spike wave discharges.
4. CT and MRI examinations show brain atrophy and soft foci.
Treatment
1. Treatment principle
The treatment of benign epilepsy syndrome is based on drug therapy. The principle of treatment is to select drugs according to the classification of seizure types and syndromes; for the first seizure in children, it is necessary to consider the etiology of epilepsy, seizure types, epilepsy syndromes and so on, in order to judge whether to start antiepileptic drug treatment. Benign epileptic syndromes can generally achieve better results after drug treatment, and there is no need to consider surgical treatment.
2. Medication
Commonly used antiepileptic drugs include sodium valproate, oxcarbazepine, lamotrigine and ethosuximide. It should be noted that the corresponding first-line drugs should be selected according to the type of epilepsy syndrome. In most children, seizures can be controlled after standardized use of an antiepileptic drug; for those with poor therapeutic effect or those who cannot tolerate it, change of medication or combination therapy should be considered as appropriate according to the type of syndrome.
Usually, if a patient with epilepsy has been seizure-free for more than 2 years, there is a possibility to reduce or stop the medication. However, whether and how to reduce or stop the medication needs to take into account the type of epilepsy of the patient (etiology, seizure type, classification of syndromes), the response to the previous treatments and the patient’s personal situation, and to carefully assess the risk of recurrence after stopping the medication, to determine that the risk of recurrence after reduction or discontinuation of the medication is relatively low, and to communicate fully with the patient or his/her guardians to ensure that they understand the relationship between reduction of medication and continuing to take medication. If the risk of recurrence after drug withdrawal is determined to be low, and after thorough communication with the patient or his/her guardian, and after he/she understands the risk/benefit ratio of drug withdrawal versus continuation of the medication, he/she can be considered to start tapering the antiepileptic drugs.
Prognosis
Prognosis is dependent on the individual and the syndrome, and the overall prognosis for benign epileptic syndromes is good with appropriate interventions.
Nursing care
1. Monitor and record seizure frequency, presentation, and duration, and follow up.
2. Observe the prodromal symptoms of seizures.
3. Take medication as prescribed by the doctor and do not stop, reduce or change medication at will.
4. Add protective rails on the bed to prevent the child from falling out of bed during sleep seizures; and install protective pads on the corners of the bed and table to prevent crashes during seizures.
5. Parents should give their children more care and understanding, encourage him/her to face the disease positively and overcome the low self-esteem.