Lennox-Gasto's syndrome

Description:

Lennox-Gasto's syndrome — the epileptic encephalopathy of children's age which is characterized by polymorphism of attacks, cognitive disturbances, specific changes by EEG and resistance to therapy. Frequency Lennox-Gasto's syndrome makes about 5% among all forms of epilepsy at children and teenagers; boys are ill more often.
The disease debuts preferential at the age of 2 — 8 years (more often 4 — 6 years).

Symptoms of the Syndrome of Lennox-Gasto:

The triad of attacks is characteristic: paroxysms of falling (atonic - and mio-clonic-astatic); tonic attacks and atypical absentias epileptica. The attacks of sudden falling caused tonic, myoclonic or atonic (a negative myoclonus) paroxysms are most typical. Consciousness can be kept or is switched off quickly. After falling spasms are not observed, and the child at once gets up. Frequent attacks of falling lead to heavy traumatization and an invalidism of patients.
Tonic attacks happen axial, proximal or total; symmetric or accurately lateralizovanny. Attacks include sudden bending of a neck and trunk, raising of hands in a condition of a semi-inflection or extension, leg extention, reduction of facial muscles, rotary motions of eyeglobes, an apnoea, a hyperemia of the person. They can arise as in the afternoon, and, especially often, at night.
Atypical absentias epileptica are also characteristic of Lennox-Gasto's syndrome. Their manifestations are diverse. Disturbance of consciousness happens incomplete. Some degree of motor and speech performance can remain. The gipomimiya, hypersalivation is observed; myoclonias century, mouth; atonic phenomena (the head falls on a breast, the mouth is slightly opened). Atypical absentias epileptica usually are followed by decrease in a muscle tone that causes as if "obmyakany" bodies, since face muscles and a neck.
In the neurologic status manifestations of pyramidal insufficiency, koordinatorny disturbances are noted. The decrease in intelligence which is not reaching, however, heavy degree is characteristic. Intellectual deficit is stated from early age, preceding a disease (symptomatic forms) or develops right after emergence of attacks (cryptogenic forms).
At EEG a research in big percent of cases irregular diffusion comes to light, it is frequent with amplitude asymmetry, slow peak - wave activity with a frequency of 1.5 — 2.5 Hz during wakefulness and bystry rhythmic categories with a frequency about 10 Hz — during sleep.
At neurovisualization various structural disturbances in a cerebral cortex, including malformations can take place: corpus collosum hypoplasia, gemimegalentsefaliya, cortical dysplasia and so forth.

Reasons of the Syndrome of Lennox-Gasto:

If Lennox-Gasto's syndrome develops at transformation from Vest's syndrome, then perhaps 2 options:
1. Infantile spasms are transformed to tonic attacks in the absence of stage of latency and smoothly pass into SLG.
2. Infantile spasms disappear; psychomotor development of the child improves a little; picture EEG is gradually normalized. Then there comes stage of latency which varies on duration at different patients; there are attacks of sudden falling, atypical absentias epileptica and the peak - wave activity on EEG accrues diffusion slow.

Treatment of the Syndrome of Lennox-Gasto:

It is necessary to avoid the drugs suppressing cognitive functions (barbiturates). Valproatums, ламотриджин, carbamazepine, benzodiazepines are most often applied. Treatment begins with valproic acid derivatives, gradually increasing them to the most tolerable dose (70 — 100 mg/kg/days and above). Carbamazepine is effective at tonic attacks of 15 — 30 mg/kg/days, but can speed up absentias epileptica and myoclonic paroxysms. A number of patients reacts to increase in a dose of carbamazepine paradoxical increase of attacks. Benzodiazepines render effect at all types of attacks, however this effect temporary. In group of benzodiazepines are applied clonazepam, to klobaza (фризиум) and nitrazepam (Radedormum). At atypical absentias epileptica Suxilepum can be effective (but not as monotherapy). We showed high performance of a combination of Valproatums with lamiktaly (1 — 5 mg/kg/days and above). This therapy is most optimum as concerning efficiency, and portability (does not oppress cognitive functions). In the USA the combination of Valproatums with felbamaty is widely used (Taloksa).
The forecast is heavy. Permanent control over attacks is reached only at 5 — 15% of patients. Predictively dominance of myoclonic attacks and lack of rough structural changes in a brain is favorable; negative factors — domination of tonic attacks and rough intellectual deficit.