What are Infantile Spasms?

Occurred from a few days to 30 months after birth, the peak of the disease before half a year old. As the baby stays in bed or nagging all day, young mothers are inexperienced and easily paralyzed, and they mistake the seizure as a result of child hunger, wet diapers, or uncomfortable head and neck conditions. After the spasm ceases, symptoms and signs of nerve damage such as speech impairment, partial blindness, strabismus, limb paralysis, or other types of seizures may remain. The mortality rate of this disease accounts for 13%, and more than 90% have low intelligence. Therefore, it is important to recognize the disease and control it in time.

Basic Information

English name: infantile spasm
Visiting department: Pediatrics
Multiple groups: baby
Common causes: Birth injury, congenital disease, brain hypoplasia, brain retardation, brain atrophy, etc., various encephalitis, meningitis, brain abscess, cerebral schistosomiasis, poisoning, etc.

Common symptoms: Bowing, nodding, lightning-like convulsions, after the spasms cease, symptoms and signs of nerve damage such as speech impairment, partial blindness, strabismus, limb paralysis, etc.

Causes of infantile spasms

Birth injuries are common causes of symptomatic epilepsy in infants and young children. The causes of birth injuries include forceps, midwifery suction, cephalopelvic disproportion, abnormal fetal position, too large fetus, long labor, and primipara Wait. Congenital diseases: brain abnormalities, hydrocephalus, chromosomal abnormalities, etc. Brain hypoplasia, brain retardation, brain atrophy, etc. Amniotic fluid inhalation Fetal asphyxia, umbilical cord around the neck, placental abruption, placenta previa, caesarean section, etc., the incidence will increase significantly in the future. Some patients with various encephalitis, meningitis, and brain abscess may have sequelae of infantile spasms. Cerebral schistosomiasis and cerebral cysticercosis can cause infantile spasms, which are rare. Intracranial tumors Pediatric tumors are rare. Cerebrovascular disease Infantile vascular malformations cause infantile spasms, which are rare. poisoning gas, pesticides and systemic diseases such as hepatic encephalopathy, rapid nephritis, uremia, etc. can cause infantile spasms. Nutritional metabolic diseases Hypoglycemia, diabetic coma, vitamin B6 deficiency, hyperthyroidism, etc. can cause infantile spasms. Trauma Including open trauma and closed trauma. Congenital factor refers to the damage that the fetus suffered in the mother before birth. It can cause abnormal brain development and infantile spasms. Such as pregnant women with abdominal injuries, uterine bleeding, ultraviolet radiation, taking harmful drugs to the fetus, various microbial infections, especially rubella and measles virus infections. Genetics Infants with epilepsy have 1 in 500 children with infantile spasms. Immunity is low Some infants and young children often have colds and fever which is the cause of their low immunity.

Clinical manifestations of infantile spasms

Bow-like cramps

Suddenly, a sudden generalized muscle spasm occurred, the trunk and legs flexed, and his arms stretched forward and outward.

2. Nodular spasms

Muscle spasms are confined to the head and neck, and nodular attacks occur, and the forehead and face are prone to bruises.

3. Lightning-like spasms

The duration is very short and can only be noticed when the child happens to stare at the episode. Atypical forms of seizures include asymmetric cramps. The head is rotated to one side or the limbs are convulsed, the extension is spasm, the head is tilted backwards, the eyes are turned upwards, the legs are stretched, and the angled bow is opened.

When a child grows into a few months, if there is a cramp like nodding, this situation may be infantile spasms.

In addition to systemic cramps, infantile spasms can cause mental retardation in children. Once diagnosed, treatment should be immediate. The earlier the treatment is started, the less likely the brain damage will be in the sick child. The main drugs currently used to treat infantile spasms are nitroazepam and hormones. If the two are used in combination, the effect is better. During medication, children should be prescribed medication regularly to maintain the necessary effective blood concentration.

In order to prevent the recurrence and transformation of infantile spasms into epileptic seizures or psychomotor seizures, the dose of the drug should not be reduced even after the seizure is completely stopped. Instead, continue to take the medicine for 2 to 4 years, and then carefully, under the guidance of a doctor, Gradually reduce the drug until it is discontinued. In addition, parents should pay special attention to when the child starts taking the medicine, he should go to the hospital every 2 to 3 weeks for follow-up, and then every 3 to 6 months to review. At the same time, we should pay close attention to the toxicity of the drug, and regularly take the child to the hospital to check liver and kidney function and blood routine. Once abnormalities are found, take further measures in a timely manner.

Infantile spasm test

Cryptogenic infantile spasms have the following characteristics: normal development before onset, normal neurological examination and neuroimaging; symptomatic spasms, no other types of seizures; the background of the electroencephalogram (EEG) is typical bilateral Peak rhythm disorder, in a series of spastic seizures, the EEG between each spasticity can restore the peak rhythm disorder pattern; evidence of infinite local abnormalities of EEG. Before the onset of symptomatic infantile spasms, there is a lack of psychomotor development. Neurological examination and neuroimaging can be found abnormally.

1.EEG characteristics

EEG during infantile spasms is characterized by peak rhythm disturbances. The typical peak rhythm disorder is a mixed wave pattern consisting of extremely high amplitude slow waves and spikes in the brain area, which are asymmetrical and asynchronous on both sides. There is no fixed relationship between spikes and slow waves.

Peak rhythm disorders are more pronounced during sleep. Normal sleep waveforms such as tip waves, sleep spindles, and K-complex waves often disappear. Studies have shown that the incidence of peak rhythm disturbances during wakefulness is 64%, NREM sleep stage I is 86%, and stage to IV is 99%. After 1 year of age, the peak rhythm disorder gradually decreases when awake, but still exists during sleep. The peak rhythm disorder during the deep sleep period gradually shows the characteristics of cycle-like distribution, and the asymmetry on both sides can be more obvious.

During infantile seizures, the peak rhythm disorder disappears, and EEG can manifest as high-amplitude slow waves or spike slow waves, and / or widespread low-voltage fast waves. Sometimes it can be shown as a pseudo-normalization, which is a low-to-medium amplitude fast wave or a slow-speed mixed wave that lasts for about 10 seconds, and looks like normal background activity.

2. Other auxiliary inspections

Neuroimaging tests such as CT, MRI, PET, and SPECT can help find structural or functional lesions in the brain. The SPECT study showed that the hypoperfusion area of cerebral blood flow is related to the cortical damage (often in the occipital area) of infantile spasms, the hyperperfusion area (often in the frontal area) is related to the persistence of epilepsy, and the hyperperfusion area is similar to the termination of spasticity cut back. MRI can detect brain structural abnormalities that are difficult to find with CT. Skin UV inspection can reveal nodular spots of skin with nodular sclerosis. Various metabolic tests, enzyme analysis, and chromosome tests can help find the cause. Intravenous injection of vitamin B6 under EEG monitoring can rule out pyridoxine dependence.

Diagnosis of infantile spasms

In typical cases, it is not difficult to make a diagnosis of infantile spasms based on the age of onset, clinical seizures, mental retardation, and EEG peak rhythm disorders. After the diagnosis is clear, try to find the relevant cause. Clinical symptoms may be atypical in the early stages of the disease, and EEG has not yet exhibited typical peak rhythm disturbances. At this time, diagnosis is difficult, and clinical and EEG changes should be closely followed. Typical clinical and EEG features generally appear within 1 to 2 months of onset.

Differential diagnosis of infantile spasms

Benign myoclonus of infant

Seizures like spasms can also occur in clusters and often occur while eating. It is obvious from weeks to months after birth and usually disappears by itself after 3 months. Before and after the onset of symptoms, psychomotor development was normal, and there were no abnormal signs of the nervous system. EEG and neuroimaging were normal. This condition is a non-voluntary exercise for small infants and requires no treatment.

2. Early infantile epilepsy encephalopathy

Also known as Otahara syndrome, the age of onset is earlier than that of infantile spasms, and it begins within a few days to 3 months after birth. Seizures are in the form of a single or series of convulsions, and EEG appears as a burst-inhibition pattern. Most have severe brain damage or structural abnormalities. Treatment is difficult and the prognosis is poor. Some children become infantile spasms at 4 to 6 months. It is also believed that Otahara syndrome is a variant of infantile spasms.

3. Infant benign myoclonic epilepsy

Clinically rare. One third of the children have a family history of epilepsy. Onset from 4 months to 2 years of age, the form of the attack is generalized myoclonic seizures. There are no other types of seizures in infancy, and generalized tonic-clonic seizures in adolescence. Before and after the onset of psychomotor development was normal. The EEG was normal during the onset, and the onset was a full-guide slow wave and multi-spike slow wave burst. Valproic acid is effective in controlling seizures.

4. Severe myoclonic epilepsy in infants

It is characterized by the normal deterioration of the nervous system after the onset of convulsions in infants with chronic severe brain damage. 25% of children have a family history of epilepsy. The first seizures were mostly febrile seizures, which lasted for a long time and were generalized or local seizures. There may be a state of epilepsy in the future. Generalized myoclonic seizures occur after 1 year of age with complex partial seizures that can be generalized throughout the body. Myoclonus began to slow down at the same time as it appeared, and there were neurological abnormalities such as ataxia and hyperteninosis. The inter-seizure EEG disease is normal at first, and then there will be widespread or slow wave release of more than 3Hz. Flashes, drowsiness, and sleep can induce abnormal discharges. Difficult to treat. Carbamazepine can increase the frequency of attacks, and valproic acid or benzodiazepines are effective.

5. Early onset Lennox Gastaut syndrome

Less than half of children with Lennox-Gastaut syndrome develop onset before the age of two. 20% have a history of infantile spasms. Most have neurological abnormalities before onset. The clinical manifestations are triads: epileptic seizures (atypical absence, loss of tension, myoclonus, tonic seizures, etc.), EEG is a widespread slow wave, and retarded mental motor development.

Infantile spasm treatment

Drug treatment

Antiepileptic drugs can eliminate or reduce seizures in two ways. One is to affect central neurons to prevent or reduce their pathological over-discharge; the other is to increase the excitement threshold of normal brain tissue and reduce the spread of excitement of the lesion, preventing Epilepsy recurs.

Antiepileptic drugs such as: phenytoin sodium, carbamazepine, ethosuccin, sodium valproate, etc. are called old antiepileptic drugs, of which phenobarbital, phenytoin, carbamazepine, sodium valproate are currently widely used First-line antiepileptic drugs. However, some developed countries have listed phenobarbital and phenytoin as second-line antiepileptic drugs due to some side effects. Only carbamazepine and sodium valproate are listed as first-line antiepileptic drugs. New anti-epileptic drugs such as gabapentin, lamotrigine, aminohexanoic acid, topiramate, etc., the newer ones are Lexiracetam tablets of Uzbekistan.

2. Surgical treatment

Cerebrocortical resection; Anterior temporal lobe resection; Cerebellar cortical resection; Combined brain amputation; Stereotactic surgery for epilepsy; Cerebellar electrical stimulation therapy for epilepsy. Patients who fail to undergo various treatments can also receive minimally invasive separation-type pacemaker implantation with significant effects.

Prognosis of infantile spasms

Most symptomatic infantile spasms have a poor prognosis, which is mainly manifested by retarded mental motor development and difficulty in controlling or converting seizures into other types of seizures. Spastic seizures last 3 to 30 months, and generally decrease after 1 year of age, and tend to disappear after 3 years of age. About half of the children changed to other types of attacks, mostly systemic attacks, including atypical absence, tonic seizures, tonic-clonic seizures, and tonic seizures. Partial seizures were also possible. Some infantile spasms develop into Lennox-Gastaut syndrome. Most children suffer from a lack of psychomotor development.