Introduction
Lennox-Gastaut syndrome(Lennox-Gastaut syndrome, LGS) Also known as minor motor seizures, it is refractory to children.epilepsySyndrome, characterized by certain types of seizures, often accompanied by mental retardation and typical EEG changes, accounts for 4.2% to 10.8% of children with epilepsy. Gibbs et al. (1939) first described the EEG characteristics of LGS. Compared with the typical absence episode 3 times/s spine wave synthesis (SSW), LGS is 2.5 times/s spine slow wave synthesis, called small attack variant (petit mal Variant, PMV), suggesting that patients with slow SSW have severe uncontrollable seizures. Later, Lennox and Gastaut discussed in detail the relationship between symptomology and EEG, which was later named Lennox-Gastaut syndrome.
Cause
Symptomatic LGS causes include prenatal, perinatal and postpartum factors, congenital brain development and metabolic abnormalities, infections, trauma, etc., 10% to 20% of cases have occurred before LGSInfantile spasm.
symptom
EEG background activity was abnormal at the time of onset, and there was a <3 Hz spine slow wave, which often showed multiple abnormalities. Usually EEG background activity is abnormal when awake, 1 ~ 2.5 times / s spine wave synthesis (SSW) is a prominent feature, often common synchronous appearance, one side is also quite common, a few are focal distribution, the forehead is most significant.
The main clinical types are as follows:
(1) tonic seizures: generally tonic axial (tonic axial), showing head, nodding and straight body, sometimes difficult to distinguish from West syndrome, transient episodes without loss of consciousness, recurrent episodes of conscious disorder, multiple episodes of sleep Especially in stage II sleep. EEG with bilateral moderate to high amplitude 10 to 25 beats / s fast rhythm outbreak, prominent anterior lead, especially slow wave sleep (NREM), short duration, sometimes clinical discharge; often seen before the burst discharge Low-level background activity or general-spinning slow-wave integrated discharge.
(2) Atypical absence seizures: seen in half of the patients, showing gaze or eyeball upturn, and the ongoing activity is interrupted. Compared with the typical absence episode, the seizure is not sudden, the stopping process is slow, the consciousness is not completely lost, and it can be accompanied by autonomic and autonomic abnormalities, which last for several seconds to ten seconds. At the time of onset, EEG showed irregularities ranging from 2 to 2.5 times per s of spine wave synthesis, which is often difficult to distinguish from the interspinning period.
(3) Disorder of tension: more common in infants, sudden disappearance of muscle tension can not maintain body posture, so that patients suddenly fall and trauma, instant attacks can be unconscious, and consciously lost in severe attacks, lasting for a few seconds. At the time of onset, EEG can be seen as a combination of spike, spike, slow wave or spine slow wave.
(4) clonic seizures: manifesting systemic or partial myoclonic tics. There is no tonic attack, which can be accompanied by loss of consciousness. The seizures are mostly in the NREM phase, and the EEG is a general 10 times/s activity, mixed with a spine slow wave integrated discharge.
(5) Sustained state of atypical absence seizures: seizures persist, consciousness is turbid, and there may be tension, short-term systemic myoclonic episodes, etc., also known as small seizure persistence, seen in 14% to 50% of LGS patients.
diagnosis
Usually 4 months to 11 years old, more common before 4 years old, 1 to 2 years old, the ratio of male to female is 1.4:1 ~ 3.3:1. Often accompanied by mental retardation, 60% of children have a history of encephalopathy. Two or more episodes of the child at the same time are important features of LGS, common tonic seizures and atypical absence episodes, as well as tonicity episodes, myoclonic seizures, GTCS, and simple partial seizures. Frequent seizures often occur. status.
complication
20% to 60% of children with LGS have mental retardation at the time of onset, and 75% to 90% have mental retardation after several years of onset. Intelligent barriers are related to the onset of illness. Half of the children had behavioral abnormalities, showing ADHD or aggressive, destructive behavior. Half of the children had no abnormalities in the nervous system and imaging examination, and the rest could be associated with neurological deficits such as cerebral palsy and speech abnormalities.
treatment
(a) treatment
Infantile spasm drug treatment: this disease is not effective with conventional anti-epilepsy. In the 1950s, it tried hormones, trimethanone,Phenobarbital,Sodium valproate(dipropionic acid),Vitamin B6Treatment with γ-globulin, monoamine compound and tyrosine is not satisfactory. Currently used:
1. ACTH Sorel (1958) first treated with ACTH, the amount of 25U, intramuscular injection, once / d, 4 to 6 weeks later changed to prednisone orally, gradually reduced, completely stopped after 2 months. It is easy to relapse after stopping the drug, which can cause side effects such as infection and electrolyte imbalance.
2. Benzodiazepine Volzke treated with sodium nitrite (nitrodiazepine) in 24 children, 13 patients were completely controlled, 6 patients had decreased seizures, and 5 patients were ineffective. The dose is 0.1-0.4 mg/(kg d), and the dosage of infant is 2.5-7.5 mg/d. For VasselaClonazepam(clonazepam) treatment of 24 children, 13 cases of long-term or temporary control, should start from a small dose of 0.01 ~ 0.03mg / (kg d), divided into 2 to 3 times, gradually increased to 0.1 ~ 0.2mg / (kg d ).
3. Dipropyl acetate Meunier reported an effective rate of 50% and a dose of 20 to 25 mg / (kg d).
4. Vitamin B6 dose of 300 ~ 900mg / d, divided into 3 times service, some children can achieve dramatic results.
5. Dihydroergotamine/caffeine (Loss blood) Dihydroergotamine/caffeine (hemolysin) is an α1/α2 receptor blocker, widely used in ischemic heart and cerebrovascular diseases. The disease has a controlling effect and the idiopathic effect is better.
6. Britain, Denmark, Germany, etc.Aminohexenoic acid(vigabatrin) treatment of this disease, Japan advocates the use of vitamin B6 first, some people in France advocate the use ofLamotrigine.
7. A group of 300 infantile spasms (i.e., idiopathic 10.3%, symptomatic 89.7%) showed: 1 single dose of conventional dose is: clonazepam (clonazepam) > nitrazepam (nitro Diazepam) > estazolam (suldol); 2 when vitamin B6 alone, 900mg / d is better than 3000mg / d; 3 single drug effect can be combined with drugs, such as dipropyl acetate plus benzodiazepines Or dipropyl acetate plus benzodiazepine plus carbamazepine; 4 alone with activating hemoglobin, intelligence recovery may be better; 5 after 1 to 10 years of follow-up, idiopathic infantile spasm is completely controlled after intelligence is normal or Near normal (IQ ≥ 70) up to 72%, symptomatic only 26%.
(two) prognosis
Most of the diseases in this group have various clear or possible central nervous system diseases, which affect the structure or function of the brain, such as chromosomal abnormalities, focal or diffuse brain diseases, and certain systemic diseases. The prognosis is related to the primary disease, and most of the prognosis is poor.
1. Infantile snoring Symptoms Infants with snoring have a poor prognosis. Idiopathic children have no mental retardation and do not develop GTCS in the future. The early stage of ACTH or oral prednisone has a good prognosis.
2. Lennox-Gastaut syndrome 20% to 60% of children with LGS have mental retardation when they are onset, and 75% to 90% have mental retardation after several years of onset. This syndrome is the most serious type of epilepsy in children. The prognosis is poor. About 80% of children have long-term uncontrollable seizures. Children with mental retardation and nervous system defects, only a few can live independently, and the short-term mortality rate is 4.2%-7%. . Symptomatic LGS is worse than idiopathic prognosis, early onset, early West syndrome, point hair as the first symptom, mental disorder at the time of onset, apparently abnormal EEG background activity, and poor prognosis in patients with persistent SSW.
3. Juvenile cerebrosideosis is chronic progressive debilitating, cerebellar ataxia, and poor prognosis.
4. Lafora disease has a very poor prognosis. Patients often die 2 to 10 years after the onset of symptoms, with an average of 5 to 6 years of death.
prevention
Prevention of epilepsy is very important. Prevention of epilepsy is not only related to the medical field, but also to the whole society. Prevention of epilepsy should focus on three levels: one is to focus on the cause, to prevent the occurrence of epilepsy; the second is to control the seizure; the third is to reduce the adverse effects of epilepsy on the physical, psychological and social aspects of patients.
Prevention, early diagnosis, and early treatment of primary disease leading to symptomatic epilepsy syndrome are also important. For those with genetic factors, special emphasis should be placed on the importance of genetic counseling. Family surveys should be conducted in detail to understand whether there are seizures and seizures in the parents, siblings and close relatives, and some serious hereditary factors that can cause mental retardation and epilepsy. For diseases, prenatal diagnosis or screening during neonatal screening should be performed to determine termination of pregnancy or early treatment.
简体中文