Lennox-gastaut syndrome

Introduction Lennox-Gastaut syndrome (LGS), also known as minor motor seizures, is a child-refractory epilepsy syndrome with certain types of seizures, often accompanied by mental retardation and typical EEG changes. Characterized by 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. Causes of symptoms of LGS include prenatal, perinatal and postpartum factors, congenital brain development and metabolic abnormalities, infections, trauma, etc., 10% to 20% of cases have had infantile spasms before LGS. EEG background activity was abnormal at the onset of symptoms, with a <3 Hz spine slow wave, often showing multiple focal 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 whole body straight, sometimes difficult to distinguish from West syndrome, transient episodes without loss of consciousness, recurrent Conscious disorder, multiple sleep, especially 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 was 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 occurs from 4 months to 11 years old, more common before 4 years old, most 1 to 2 years old, male to female ratio of 1.4:1 to 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. Children with LGS with 20% to 60% of complications have mental retardation when they are 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 of infantile spasm (infantile spasm) drug treatment: the disease is not effective with conventional anti-epilepsy, in the 1950s tried hormones, trimethesone, phenobarbital, sodium valproate (dipropyl Treatment with acetic acid), vitamin B6, γ-globulin, monoamine compounds and tyrosine is not satisfactory. Currently commonly used: 1. ACTH Sorel (1958) first treated with ACTH, the amount of 25U, intramuscular injection, 1 / d, 4 to 6 weeks later changed to prednisone oral, gradually reduced, completely after 2 months Stop the drug. 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. Vassela was treated with clonazepam (clonazepam) in 24 patients, 13 patients with long-term or temporary control, starting from a small dose of 0.01 ~ 0.03mg / (kg d), divided into 2 to 3 times, gradually increased to 0.1 ~ 0.2 mg / (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. advocated the trial of aminoglycolic acid (vigabatrin) for the treatment of this disease. Japan advocates the use of vitamin B6 first. Some people in France advocate the use of lamotrigine. 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 (sulpiride); 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%. (b) Prognosis Most of the diseases in this group have various clear or possible central nervous system diseases, affecting the structure or function of the brain, such as chromosomal abnormalities, focal or diffuse brain diseases, and certain systemic diseases. To. 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. Early prognosis with ACTH or oral prednisone is better. Read more...