What Is Benign Childhood Epilepsy?

Childhood epilepsy is a neurological syndrome caused by complex, recurrent, paroxysmal, and transient brain dysfunction that is common in childhood (0-18 years). The etiology is divided into two types: primary and secondary. Epilepsy in children is mostly caused by perinatal ischemia and hypoxia, cortical dysplasia, low-grade glioma, encephalitis, and trauma.

Basic Information

Visiting department

Pediatrics, Neurology

Common locations

Brain neuron

Common causes

Perinatal ischemia and hypoxia, cortical dysplasia, low grade glioma, encephalitis, trauma, etc.

Common symptoms

Partial or segmental polymyoclonus, convulsions, etc.

Epilepsy in children

Idiopathic epilepsy accounts for 20% of the total number of patients with epilepsy. No cause of disease has been found. Most of them are genetically related, so it is also called cryptogenic epilepsy or hereditary epilepsy.

Secondary childhood epilepsy refers to the seizures caused by abnormal brain structure or function and abnormal discharge of neural network during the growth and development of children due to some unexpected reasons. The main causes are as follows:

Perinatal ischemic hypoxic brain injury

It is mainly due to birth injury, asphyxia, intracranial hemorrhage, and hypoxic-ischemic encephalopathy. Among them, epilepsy caused by hypoxic-ischemic encephalopathy is the most common.

2. Congenital Brain Developmental Malformation

Such as anencephaly dysplasia, megacephaly dysplasia, multiple cerebellar dysplasia, gray matter ectopic, cerebral perforation malformation, congenital hydrocephalus, hypoplasia of the corpus callosum, arachnoid cyst, and megacephaly

3. Neurocutaneous Syndrome

The most common are nodular sclerosis and facial hemangioma.

4. Genetic Metabolic Disease

Such as phenylketonuria, hyperammonemia, cerebral lipidosis, vitamin B ₆ dependence.

5. Intracranial infection

Such as bacterial meningitis, viral encephalitis, brain abscess, fungal meningitis, cerebral parasitic disease, encephalitis after vaccination and so on.

6. Nutritional metabolism disorders and endocrine diseases

Common hypoglycemia, hypocalcemia, hypomagnesemia, and hypothyroidism.

7. Cerebrovascular disease

Such as cerebrovascular malformations, cerebrovascular inflammation.

8. Trauma

Intracranial hemorrhage caused by trauma, skull fracture, cerebral contusion and laceration.

9. Brain tumors

Such as glioma, astrocytoma, etc., tumors in the parietal, frontal and temporal lobe areas often cause epilepsy.

10 brain degenerative diseases

Macular degeneration, multiple sclerosis, subacute sclerosing panencephalitis.

11. Toxic encephalopathy

Drug poisoning; food poisoning, carbon monoxide (CO) poisoning, organic phosphorus poisoning, heavy metal poisoning (mercury, lead, arsenic), etc.

Clinical manifestations of childhood epilepsy

1. Benign familial neonatal convulsions

Has a family history of neonatal convulsions, which are idiopathic epilepsy. 80% of seizures occur 2 to 3 days after birth. The type of seizures is general seizures, the prognosis is good, the psychomotor development is normal, and secondary epilepsy does not appear. Control drugs can be selected. Phenobarbital and sodium propionate.

2. Benign idiopathic neonatal convulsions

Children with benign idiopathic neonatal convulsions develop onset 1 to 7 days after birth, mostly with partial clonic or asphyxia attacks, and have a good prognosis.

3. Early-onset infantile epilepsy encephalopathy (Ohtahara syndrome)

The first attack occurs within 2 to 3 months after birth, usually within 1 month. It is mainly manifested by frequent seizures, outbreak suppression EEG, and poor prognosis.

4. Early-onset myoclonic encephalopathy

It usually occurs in the neonatal period, and is mainly manifested as partial or segmental polymyoclonus and partial motor seizures. In the later stage, it can be ankylosing spasm, and the EEG is mostly burst suppression. The disease is severe and has a poor prognosis.

5. WEST syndrome

Also known as infantile spasms, the etiology mechanism is not completely clear. More than 60% of the children are symptomatic, and 40% are idiopathic. Symptoms are more common in perinatal hypoxia, nodular sclerosis, multiple neurofibromas, brain hypoplasia, Sturge-Weber syndrome, various perinatal brain injuries, and intracranial infections.

55% to 77% of children develop symptoms within 3 to 7 months after birth. There was no significant difference in the incidence of sex. The disease is characterized by flexor infantile seizures, stagnation of psychomotor development, and irregular EEG rhythms. Generally worsen gradually after onset. Seizures are manifested by short tonic spasticity, which is more pronounced with flexors, often with sudden neck flexion and waist movements, and can also involve limbs. Each spasm is about 1 to 15 seconds, often in clusters, each cluster is composed of several or dozens of spasms. Cluster attacks are one of the characteristics of this disease.

Symptomatic infantile spasms are often accompanied by other seizure types, such as tonic seizures, tonic seizures, atypical absence seizures, and partial seizures. They can also be found in other epilepsy syndromes such as epileptic encephalopathy. 70% to 90% of children have developmental disorders and signs of the nervous system before the onset of spasms. Normally, children with normal development become retarded or even retrogressed after the illness. It manifests as weakened response to the surrounding environment, loss of active grasping ability, irritability, apathy, and low muscle tone.

High arrhythmia is a typical manifestation of EEG, which is irregular high amplitude spikes and slow waves.

Antiepileptic drugs are not effective. Can try to apply nitrazepam, clonazepam, valproic acid, ACTH, hormones and ketogenic diet therapy, vagus nerve stimulation has a certain effect.

The prognosis of infantile spasm is mainly determined by the cause, and symptomatic (secondary) surgery can be cured. Idiopathic or inoperable symptoms have a poor long-term prognosis, and more than 90% have different degrees of psychomotor retardation. Many children develop other types of seizures in later childhood and may develop Lennox-Gastaut syndrome.

6. Infant benign myoclonic epilepsy

The age of onset of this disease is mostly 1 to 2 years old, and often has a family history of convulsions or epilepsy. The clinical feature is a transient generalized myoclonic seizure without other types of seizures. The EEG showed a wide range of spikes and slow waves during the attack, mostly bilaterally synchronized. Seizures are normal. Should be treated early.

7.Dravet syndrome

Also known as infant myoclonic epilepsy in infancy (SMEI), the overall incidence is about 1/20000 to 40,000, male: female about 2: l, accounting for about all types of myoclonic epilepsy in children. 29.5%, accounting for 7% of infants and young children with epilepsy under 3 years old.

Dravet syndrome can cause severe epilepsy encephalopathy. It has the characteristics of early age of onset, complex attack form, high frequency of attacks, severe intelligence damage, low efficiency of drug treatment, poor prognosis, high mortality, etc. It is also a representative of refractory epilepsy. The disease is drug-refractory epilepsy with a poor prognosis.

8.LGS syndrome

Lennox-Gastaut syndrome (LGS) is an age-related epilepsy syndrome that can be idiopathic or caused by certain causes, such as congenital developmental disorders, metabolic abnormalities, perinatal hypoxia, and nervous system Hypoxia caused by infection or persistent state of epilepsy. The age of onset is 3 to 10 years old, and more common is 3 to 5 years old. A few can be secondary to other types of epilepsy such as WEST. The characteristics of typical LGS syndrome include three: seizures manifested as axonal rigidity, dystonia, and atypical absence seizures; EEG manifests as diffuse-slow wave bursts, violent rhythmic fast waves, waves in wakeful states Slower multi-spikes, and finally 10HZ fast sexual rhythms in sleep; mental retardation and accompanying personality disorders.

Treatment of this symptom is very difficult and the efficacy of antiepileptic drugs is not good. 80% to 90% of children have uncontrolled seizures. The disease is one of the indications for vagus stimulation. Curable for symptomatic (secondary) surgery.

The disease has a poor prognosis, with most cases turning into chronic attacks with a 10-year mortality rate of about 5%.

9. Fever convulsions

Fever convulsions, also known as febrile convulsions, are common in children and most have a good prognosis. The prevalence of convulsions is usually from March to 5 years, and convulsions are relieved due to the development of the brain after 6 years of age. It usually occurs at the beginning of upper respiratory infections or other infectious diseases Convulsions occur when the temperature rises above 38 ° C. Diagnosis of the disease requires exclusion of intracranial infections and other organic or metabolic abnormalities that cause convulsions. The cause of febrile seizures is not fully understood, and it is believed to be related to immature brain tissue development, fever, and genetic susceptibility.

Febrile seizures usually resolve in 2 to 5 minutes, and no special treatment is needed. If the seizures do not resolve for more than 5 minutes, the emergency hospital should seek medical treatment. First-aid treatment such as diazepam, antipyretic and antipyretic drugs. The disease focuses on prevention. It is especially important to identify and actively use antipyretic drugs or physical cooling to prevent the temperature from rising above 38 ° C. If the child is complicated with febrile seizures, frequent febrile seizures (more than 5 times per year) or the febrile seizures are persistent, long-term oral antiepileptic drugs can be used to control the seizures to prevent febrile seizures, and phenobarbital The appropriate course of treatment with sodium valproate or valproate lasts until 6 years of age.

10.Epilepsy with spikes in the central temporal region (BECT)

This type of epilepsy is benign child epilepsy. There are more males than females, and there is a significant correlation with age. Most of them occur between the ages of 2 to 14, and they are more common between the ages of 6 to 10. They alleviate or disappear before the age of 16, and have a good prognosis. This symptom group is a transient, simple partial facial lateral movement, such as unilateral muscles, oropharyngeal muscles, transient rigidity of the lips or clonic tics. Often accompanied by somatosensory symptoms, some may develop tonic or clonic seizures. Most of these attacks are related to sleep. More than half of these attacks occur only during sleep, and most of them occur during sleepiness or rest during the day. During the onset of the EEG, there are high amplitude spikes and slow spikes in the central temporal region. These waves can be induced by sleep and easily spread. If the seizures are infrequent, they can be observed without medication. If the seizures are frequent, sodium propionate can be used, and carbamazepine can control the seizures.

11. Childhood epilepsy

Childhood absence epilepsy (CAE) is one of the most common epilepsy syndromes in childhood. Onset of epilepsy occurs at the age of 3 to 9 years, and the peak is at 6 to 7 years. It has obvious inheritance. Tendency, girls are more common than boys. It is characterized by frequent absences, several times a day, and each episode is short, but the degree of loss of consciousness is heavier. EEG can see bilateral symmetrical synchronous slow wave, generally 3Hz, background Activity is normal. Most children can gradually relieve or disappear with age. Good drug control is one of the factors that have a good prognosis.

12. Adolescent missing epilepsy

The disease mostly occurs between the ages of 7 to 17, and the peak age is 10 to 12 years. No significant gender difference. Seizure frequency is low, often sporadic, seizures are often accompanied by generalized tonic-clonic seizures, 15% to 20% are myoclonic seizures, background EEG activity is more normal, and interim and seizure EEG manifestations are full or symmetrical Sexual spine-slow wave discharge, generally 3.5 ~ 4Hz. The prognosis is worse than in children with epilepsy.

13. Adolescent myoclonic epilepsy

Onset before and after puberty, typically manifested as single or recurrent bilateral, non-rhythmic, irregular myoclonic tics, mainly in the upper limbs. Associated with heredity, no gender differences. Systemic tonic-clonic seizures are more common, with a few patients seeing loss of sight, and epilepsy often occurs shortly after awakening, generally without significant disturbance of consciousness. There are fast and widespread irregular spine slow waves and multi-spike slow waves in the inter-episode and electroencephalograms. Deprivation of sleep or light stimulation can often be induced. The drug treatment effect is good, and can be treated with propionic acid, phenobarbital, lamotrigine, and levetiracetam.

Childhood epilepsy treatment

Children with epilepsy can be treated with drugs, surgery, and neuromodulation. Drug therapy is preferred for epilepsy with no clear cause. For epilepsy with clear cause, such as focal cortical dysfunction and brain-facial hemangiomatosis, early surgical treatment is recommended. Such secondary epilepsy has a low possibility of self-healing. Brain function can be further compensated for development after surgery.

Drug treatment of epilepsy has advantages and disadvantages, mainly the impact on brain function and the impact of drugs on physical development. Drug treatment is recommended for patients with frequent and severe seizures. For benign epilepsy with low frequency of seizures and mostly at night, it can be cautious. Consider using drugs.

Some children's epilepsy is benign epilepsy, such as febrile seizures, benign child myoclonus, BECT, most children's absence epilepsy, etc., can heal with age, some secondary children's epilepsy: if focal cortical hair is bad, brain face Hemangiomatosis, partial nodular sclerosis, etc. can be cured surgically.

Common epilepsy in surgically curable children:

Focal cerebral cortical dysplasia

Focalortical dysplasia (FCD) is a disease caused by cerebral cortical neuron migration disorder or cell proliferation disorder, is a type of cortical developmental malformation, and is the most common cause of refractory epilepsy. In epilepsy surgery, FCD accounts for about 40% to 50% of children with epilepsy surgery and about 20% of adults with epilepsy surgery. Surgery is an effective method to treat refractory epilepsy caused by FCD. The prognosis of surgery is related to the location of the brain lobe and whether the epileptogenic focus is completely removed. With the advancement of surgical methods, the postoperative epilepsy remission rate reaches 80%.

Nodular sclerosis

Nodular sclerosis (TSC) is an autosomal dominant inherited neurodermal syndrome, which is mostly caused by abnormal organ development in the ectodermal tissue, and may involve multiple organs such as the brain, skin, peripheral nerves, and kidneys. Clinical features It is facial sebaceous adenoma, seizures, and decreased intelligence. The incidence rate is about 1/6000 live babies, and the male to female ratio is 2: 1. More than childhood onset, more men than women. Rapamycin can be used in childhood, and some patients can control nodular growth to a certain extent. Early antiepileptic drugs to control seizures can help prevent secondary epilepsy encephalopathy and cognitive behavioral damage. For drug-refractory epilepsy, responsible nodules (nodules that cause epilepsy) can be surgically removed. Most patients can achieve satisfactory results.

3. hypothalamic hamartoma

It is a rare, congenital brain dysplasia that occurs frequently around the lower hindbrain. The disease is common in infants and preschool children, and clinical symptoms are related to the location and size of the "tumor" and the composition of the "tumor". The first symptoms of most patients are seizures and precocious puberty; some cases can be manifested as mental, behavioral and intellectual disorders; a few cases are combined with other organs' abnormal development, such as multi-finger, facial deformity, heart defects, etc .; very few cases Without any symptoms. The treatment of hypothalamic hamartoma is mainly surgical. The surgical methods include thermal coagulation and surgical resection, and thermal coagulation and damage are recommended.

4. Brain Facial Hemangiomatosis

Cerebral facial hemangioma syndrome is often called Sturge-Weber syndrome, which is a congenital neurocutaneous syndrome. It is mainly manifested as the trigeminal nerve distribution area on one side, and the purple-red hemangiomas that are more commonly distributed on the branch of the eye nerve can be severely spread on one side of the face. On the ipsilateral intracranial side of the cerebral hemi-brain, there are different extents of pial meningeal vascular hyperplasia, and the patient mainly presents with seizures. Children often have delayed development and mental retardation. If the choroid has lesions, it can cause glaucoma, optic nerve atrophy, and blindness. Facial hemangiomas can be treated with cosmetic surgery or laser treatment, and patients with severe seizures can be treated with lobes or hemispheres with good surgical results.

5.Rasmussen encephalitis (syndrome)

Rasmussen encephalitis (Resmussen encephalitis, RE) is a sporadic disease of unknown etiology that starts in childhood. It is mostly manifested as partial epilepsy or persistent epilepsy in the early stage, and its imaging manifestations are progressive atrophy of one side of the hemisphere and T2 and / or FLAIR abnormal signals. Drug treatment has no obvious effect, and the development of the disease to the advanced stage will lead to hemiplegia and mental retardation in sick children. Antiepileptic drugs are ineffective, and some patients with hormonal shock or immunoglobulin therapy can provide short-term relief. The constant seizures and hemiplegia seriously affect children's normal development, education and adult work. Removal of the affected hemisphere is the only effective method to control epilepsy.