Benign rolandichesky epilepsy
Benign rolandichesky epilepsy — genetically determined focal epilepsy connected with age hyper excitability of bark of central temporal area of a brain. Benign rolandichesky epilepsy is shown by the rare, arising mainly at night, convulsive attacks in one half of the face, language and a throat; in some cases — generalized epipristupa. The diagnosis is established on the basis of clinical features of a disease and data of EEG, if necessary the polisomnografiya and MRT of a brain is carried out. Benign rolandichesky epilepsy does not lead to violations of psychophysical development of the child and completely passes by the end of the teenage period.
Benign rolandichesky epilepsy
In pediatrics benign rolandichesky epilepsy is the most widespread pathology from group of benign focal epilepsies. 15% of all cases of epilepsy at children aged up to 15 years fall to its share and children have about 20% of cases of a convulsive syndrome. Benign rolandichesky epilepsy meets frequency of 21 a case on 100 thousand children. Benign rolandichesky epilepsy can arise aged from 2 up to 14 years, but in 85% of cases it demonstrates in the age period from 4 to 10 years. The good-quality course of classical rolandichesky epilepsy is characterized by lack of psychoneurological changes and total disappearance of epipristup by 15-18 years.
Causes of benign rolandichesky epilepsy
The exact reasons of emergence of benign rolandichesky epilepsy are not established yet. In certain cases (on different these 20-60%) the hereditary nature of a disease is traced, however direct autosomno-recessive or autosomno-prepotent inheritance is not observed. It formed the basis for the polygenic theory of inheritance of benign rolandichesky epilepsy according to which the disease is determined by two different genes.
In modern neurology and an epileptologiya idea that benign rolandichesky epilepsy arises owing to violation of maturing of a cerebral cortex in central temporal area is dominating. Benign rolandichesky epilepsy is age - a dependent disease and is possibly connected with hypererethism of a brain at children. The last is caused by a number of factors: functional and structural features of epileptogenny areas (neocortex, a hippocampus, amygdala), the prevalence of exciting neurotransmitters, the low maintenance of GAMK, immaturity of GAMK-receptors increased by number of exciting returnable synapses. In process of maturing of a brain of the child there is a decrease in his excitability and gradual disappearance of an epileptogennost of the centers of benign rolandichesky epilepsy. Reduction of number of epileptic seizures and absolute recovery of patients at achievement of the pubertatny period by them is as a result observed.
Symptoms of benign rolandichesky epilepsy
The simple partial epipristupa proceeding without consciousness loss are the most typical for classical benign rolandichesky epilepsy. As a rule, the attack is preceded by touch aura in the form of unilateral feeling of a tingling, a pricking or a sleep on a face, lips, gums, language and in a drink. Further there is a motor paroxysm which can be presented by both tonic, and clonic muscular contractions, and also their combination. Depending on localization of spasms attacks of benign rolandichesky epilepsy can be gemifatsialny and faringooralny. The Gemifatsialny attack proceeds with spasms in muscles of a half of the face. The Faringooralny attack is characterized by unilateral spasms in muscles of language, lips, drinks and throats. It is followed by a gipersalivation and violation of the speech. Leads convulsive reductions of muscles of a throat to emergence of the characteristic guttural sounds reminding gurgle, rinsing of a throat or grunting. In process of development of benign rolandichesky epilepsy convulsive attacks can "change" the party.
80% of epipristup by which benign rolandichesky epilepsy is followed arise at night and are closely connected with the periods of falling asleep and awakening. The combination of night attacks to the attacks occurring in the afternoon is much less often observed. In 20% of cases benign rolandichesky epilepsy proceeds with brakhiofatsialny attacks during which spasms of front area extend to a gomolateralny hand. In 8% of cases involvement of a gomolateralny lower extremity is observed. In certain cases benign rolandichesky epilepsy is followed by difficult partial epipristupa for which short-term loss of consciousness is typical. Approximately 20% of patients have secondary and generalized epipristupa during which spasms take all muscles of a body. Such attacks proceed with dead faint.
Course of benign rolandichesky epilepsy
Benign rolandichesky epilepsy is characterized by the small duration of epipristup (up to 2-3 minutes) and their small frequency. Attacks happen single or are observed several times in a year. At the beginning of a disease attacks can sometimes be more frequent, however in process of a growing of the child their urezheniye is observed.
Unlike the epilepsy described by Jackson, classical benign rolandichesky epilepsy is not followed by a delay of psychomotor development of the child and does not bring to mental retardation. However it is impossible to tell about absolute lack of neurologic violations as approximately 3% of patients after attacks have a passing hemiparesis.
Diagnosis of benign rolandichesky epilepsy
The main way of diagnosis of benign rolandichesky epilepsy is EEG. As at some patients of change of EEG are observed only in the period of a dream, at normal day EEG carrying out night EEG-monitoring or a polisomnografiya is in addition necessary.
Patognomonichny symptom of benign rolandichesky epilepsy detection against the background of normal main activity of the high-amplitude sharp waves or peaks located in central temporal assignments is considered. Often after peak slow waves follow, together with peak they make a so-called "rolandichesky complex" lasting up to 30 ms. Visually such complexes remind the QRST complexes on the electrocardiogram. Usually "rolandichesky complexes" are localized on the party opposite to convulsive attacks, but can have also bilateral character. To features of EEG-patterns of benign rolandichesky epilepsy their variability from one record EEG concerns to another.
Differential diagnosis of benign rolandichesky epilepsy
First of all benign rolandichesky epilepsy needs a differentiation from the symptomatic epilepsies arising at intracerebral tumors, craniocereberal injuries, inflammatory damages of a brain (abscess, encephalitis, purulent meningitis). In confirmation of rolandichesky epilepsy tells lack of pathological changes in the neurologic status and disorders of behavior, safety of intelligence, anamnestichesky data, normal main activity on EEG. In certain cases for specification of the diagnosis brain MRT is carried out.
Big difficulties are represented by differential diagnosis of benign rolandichesky epilepsy and night epilepsy with difficult partial epipristupa. It is connected with the fact that violations of the speech, typical for an attack of benign rolandichesky epilepsy, can be interpreted as consciousness violations. On the other hand it is rather difficult to diagnose night disorders of consciousness adequately. In such cases in diagnosis a crucial role is played by data of EEG which at frontal and temporal epilepsy show focal changes of activity of a brain in the corresponding assignments.
Certain difficulties are caused by a differentiation of classical benign rolandichesky epilepsy from a syndrome of a psevdolennoks which is observed at 5% of patients with symptoms of rolandichesky epilepsy. A combination of typical rolandichesky epipristup to atypical absansa, mioklonichesky and asthenic attacks, intellectual violations, and also identification on EEG of diffusion peak activity or slow complexes characteristic of Lennox's syndrome — Gasto, testify in favor of a syndrome of a psevdolennoks.
Treatment of benign rolandichesky epilepsy
For children's neurologists and epileptolog the question of expediency of treatment of benign rolandichesky epilepsy is very diskutabelny. As even without performing treatment benign rolandichesky epilepsy comes to an end with recovery, according to some authors, it is not the indication to purpose of anti-epileptic therapy. Other neurologists, pointing out probability of a diagnostic mistake at diagnosis "benign rolandichesky epilepsy" and considering a possibility of its transition to a syndrome of a psevdolennoks, recommend to carry out anti-epileptic therapy at repeated epipristupa.
In treatment of children with benign rolandichesky epilepsy only 1 antiepileptic medicine (monotherapy) is always used. Treatment is usually begun with purpose of one of medicines of valproyevy acid. At its intolerance or inefficiency, as a rule, pass to reception of a topiramat or levetiratsetam. At children 7 years perhaps use of carbamazepine are more senior, however it is necessary to remember that in certain cases it can lead to an aggravation phenomenon, i.e. to increase of attacks.