It should also be emphasized that in a number of relatives of probands suffering from absences, as well as in rare cases in the general population, typical epi-activity on EEG of 3 Hz can be recorded in the absence of seizures. Consequently, the diagnosis of DAE cannot be established only by the presence of EEG-changes without clinical manifestations of the disease.
It is not necessary to conduct neuroimaging in patients with a clear clinical picture of AED and typical EEG changes, as with most generalized idiopathic epilepsies, due to the fact that organic changes in brain structures are not detected.
Thus, the criteria for the diagnosis of DAE, according to Panayiotopoulos, are as follows: – debut at the age of 2-8 years;
- typical absans (simple and complex);
- short duration of attacks (6-14 seconds);
- high frequency of attacks (tens per day);
- normal neurological status;
- normal intelligence;
- the presence of a generalized bilateral synchronous peak-wave activity of 3 Hz on the EEG; – favorable prognosis.
Treatment DAE. Complete therapeutic remission is achieved in patients with AED in 70-80% of cases. The drugs of choice for the treatment of absense epilepsy are ethosuccimides, convulex and valproates.
Ethosuximide (suksilep) is used in doses of 15 mg / kg / day (500-1500 mg / day) in 2 doses. Complete
drug remission absences in the appointment of suksilep reaches 65%, a significant improvement is observed in 20% of cases. A significant negative aspect of suxilep therapy is the complete absence of the effect of the drug on generalized tonic-clonic convulsions. Negative phenomena also include a significant impact on cognitive function and the gastrointestinal tract.
The use of valproic acid preparations (konvuleks, konvulsofin, depakin, apilepsin) is most preferable in absence forms, since valproates have a significant spectrum of therapeutic activity, and abstinence, as well as tonic-clonic generalized seizures, do not affect non-cognitive functions. Treatment should begin with monotherapy, 10-15 mg / kg / day, gradually increasing the dose to 30-50 mg / kg / day in 3 doses.
In some patients, the effect is observed only at high dosages of the drug, up to 100 mg / kg / day. A pronounced therapeutic effect is achieved within 10-14 days from the start of taking an adequate dose of the drug.
It should be noted that approximately 15% of patients with DAE are resistant to concoulex and valproic acid preparations. In the absence of a clinical effect with the alternate use of valproates and ethosuximide (in adequate therapeutic doses!), From monotherapy are transferred to combinations of drugs. Possible combinations:
- konvulex + suksilep (initial doses of drugs remain unchanged);
- Convulex + benzodiazepine: clonazepam (antelepsin) 2-8 mg / day or clobazam 5-25 mg / day; the dose of valproate remains 20-30 mg / kg / day;
- Convulex + lamotrigine (lamictal) at a dose of 0.2-5 mg / kg / day; the dose of valproate remains 20-30 mg / kg / day;
The clinical efficacy of therapy correlates with a positive trend in EEG. Use with DAE phenobarbital, carbamazepine is contraindicated!
There are observations that the use of barbiturates leads to the development of resistance
absences to other basic drugs. Derivatives of carbamazepine (finlepsin, tegretol, timonil) contribute to the increase in absences and can lead to the development of absences status. Some authors recommend that carbamazepine be prescribed for provocation of absences with a diagnostic purpose in stationary conditions. Liporace J.D. et al., 1994). In our opinion, such diagnostic approaches may significantly worsen the clinical picture of the disease, and it is better to limit ourselves to more traditional provocative tests (hyperventilation, sleep deprivation).
Forecast. In general, AED refers to benign forms of epilepsy. With adequate therapy in most cases it is possible to achieve complete remission. The duration of treatment with complete disappearance of attacks – 2-3 years. Late prescription of anticancer therapy, incorrect choice of the drug, and lower dosage can lead to the development of pseudoresistance.