[Eye movement disorders in children].

The article includes review of literature on anatomy, physiology, symptoms of ocular movement and their disturbance in children. Differential diagnosis between early developmental disturbances of vision in the normal child and during the diseases of central nervous system is very hard. There is data on such pediatric neuro-ophthalmology complex disorders as nystagmus, paroxysmal tonic upgaze, opsoclonus, spasmus nutans, seizures (eyelid myoclonia, absences).

[Clinical presentation and diagnosis of epileptic auras].

To define clinical presentations of visual auras and to reveal their clinical, encephalographic and neuroimaging correlates, we examined 23 patients, aged from 5 to 25 years (mean 14±6 years), with focal forms of epilepsy. Patients had visual auras regardless of the etiology of epilepsy which developed immediately before epileptic seizures or were isolated. Patients had simple or complex visual hallucinations, the former occurring more frequently, visual illusions and ictal amaurosis. Positive visual phenomena were noted more frequently than negative ones. In most of the patients, visual hallucinations were associated with the pathological activity in cortical occipital regions of the brain and, in some cases, in temporal and parietal regions. The different pathologies (developmental defects, post-ischemic, atrophic and other disturbances) identified by MRI were found in a half of patients.

[Atypical absences: prevalence, electroclinical and neuroimaging characteristics].

The study included 1261 patients with different forms of epilepsy. Ages at onset of disease varied from the first days of life to 18 years old. Absence seizures were identified in 231 (18.3%) patients, atypical absences (AA) in 129 (10.2%) of patients, with the frequency of absence seizures 55.8% in the total group. Patients with AA had different forms of epilepsy with the prevalence of cryptogenic/symptomatic forms with the phenomenon of secondary bilateral synchronization in the EEG. If epilepsy manifested itself at the age above 12 years old, AA were not noted. The study demonstrated the relevance of using video-EEG monitoring for diagnosis of epilepsy with AA. The antiepileptic treatment was effective in 53.5% of patients.

[Features of epileptiform activity on EEG in children with periventricular leukomalacya and cerebral palsy without epilepsy].

We have analyzed morphologic and chronologic characteristics of epileptiform activity, with account for repeated EEG-study during the follow-up, in patients with periventricular leukomalacia and children cerebral palsy without epilepsy. The high frequency of "benign epileptiform patterns of childhood" (BEPC) was noted. The epileptiform activity recorded by chronologic criteria corresponded to BEPC in 67% of children. The high probability of epileptiform activity of symptomatic character was identified in 33% of children. The results obtained in this study of the parameters of epileptiform activity could be of great importance for predicting the risk for the development of epilepsy and tactics of rehabilitation of motor disorders.

[Efficacy of treatment of focal forms of epilepsy in children with antiepileptic drugs of different generations].

An aim was to study the efficacy of different groups of antiepileptic drugs in the treatment of focal symptomatic (or probably symptomatic) epilepsy in children. The study included 96 patients, aged from 1 month to 17 years, 55 boys and 41 girls. They were stratified into three groups by drug type: group 1 (34 patients) was treated with phenobarbital, group 2 (31 patients) received topiramate and group 3 (31 patients) received lamotrigine. It has been shown that antiepileptic drugs of the new generation have higher efficacy with regard to focal forms of epilepsy in children compared to phenobarbital. However therapeutic effect, especially regarding seizure frequency, was specific for each drug.

[The study of quality of life of epileptic children with the special questionnaire QOLCE].

The objective was to create a Russian version of the special quality of life (QOL) questionnaire QOLCE for children with epilepsy and to use it to study QOL. The language and cultural adaptation of the questionnaire has been carried out. The final pilot version was administered to 50 mothers of children with epilepsy. The age of patients was from 4 to 16 years (mean 8.94 +/- 4.21), mean age at-disease-onset was 5.30 +/- 3.33 years (from 1 month to 12 years), mean duration of epilepsy was 4.36 +/- 3.48 years, mean duration of treatment was 3.28 +/- 2.58 years. Idiopathic epilepsy was diagnosed in 17 (34%) children, symptomatic - in 13 (26%), cryptogenic - in 20 (40%). The QOLCE was acceptable for all parents. For the majority of items, the mean percentage of missing answers was 3.09%; for Social activity and Stigma subscales the values were 15%. The lowest scores were shown on subscales Physical Restrictions (49.16 +/- 20.31), Cognitive Function (55.78 +/- 23.36), General Health (59.90 +/- 26.16). The highest scores were on the subscale Social Activity (78.08 +/- 26.88). The authors believe that the further testing of the Russian version of the QOLCE is needed.

[Efficacy and safety of the monotherapy with trileptal (oxcarbazepine) in children and adolescents].

A prospective non-randomized non-controlled multicenter trial has been conducted. The trial included 254 children, aged from 11 months to 18 years (mean age 9.3 +/- 4.5 years), with predominantly focal forms of epilepsy treated with trileptal (oxcarbazepine). The observation period was 31 weeks. Efficacy and safety of therapy was assessed in 3 visits: screening and assignment to therapy (visit 1), the end of titration and achievement of maintenance dose (visit 2), assessment of maintenance therapy (visit 3). The percentage of patients with a positive response to the trileptal therapy (the decrease of seizure frequency by 50% and more) was 91.1%. The complete reduction of seizures was achieved in 59.4% of patients. Most of patients (95.3%) continued to receive trileptal until the end of the trial. The significant decrease (p < 0.001) of seizure frequency from visit 1 to visit 3, the reduction of epileptiform activity (p < 0.05) on the awake EEG in visit 3 were found. The mean effective dose of trileptal was 902.4 +/- 442.7 mg/day, i.e. was less than 30 mg/kg/day, daily doses did not exceed 600 mg. Adverse effects were observed in 11.2% of patients but in 40% of cases they seemed not be related to the drug. The adverse effects were from mild to moderate extent. In conclusion, trileptal as the monotherapy is effective and well-tolerated in the treatment of focal epilepsies in the age groups studied.

[The electrophysiological phenomenon of prolonged epileptiform peak-wave activity to the slow sleep phase].

Thirty-six cases of epilepsy with the phenomenon of prolonged epileptiform peak-wave activity in slow sleep (PESS) are presented. The peculiarities of this phenomenon are singled out. Basing on the obtained results, the authors describe a modified variant of PESS pattern which is characterized by the presence of the stable regional amplitude prevalence of peak-wave discharges and/or stable regional epileptiform activity in the "classic" PESS pattern.

[West syndrome: clinico-electro-anatomical characteristics and a differential therapeutical approach].

One hundred and thirty children with West syndrome, aged 1.5 months-2 years, were studied. The symptomatic form of West syndrome was diagnosed in 95.4% of cases. The prenatal etiological factors were observed in 57% of patients. Different variants of hypsarrhythmia at EEG were revealed in 87%. The percentage of cases with typical and modified hypsarrhythmia was 13.3% and 86.7%, respectively. The choice of treatment was based on the revealed disturbances on EEG and MRI that was useful for increasing the effectiveness of treatment of West syndrome resulting not only in the reduction of seizures and improvement of EEG but also in the stabilization of intellectual disintegration and recovery of functions. The basic drugs in the treatment were valproates used both as mono- and polytherapy.

[Clinical polymorphism of malignant epilepsy of infancy with migrating multifocal seizures (8 cases)].

Malignant migrating partial seizures in infancy are rare epilepsy syndrome that begins in the first 6 months of life and characterized by multiple continuous electroencephalographic and electroclinical focal ictal patterns which involved different independent areas of both hemispheres with arrest of psychomotor development. The present detailed review is based on the personal observation of 8 patients newly diagnosed at the Russian Children Clinical hospital, Moscow, Russia. At least three ictal patterns recorded from different independent areas of both hemispheres were fixed by video-EEG-monitoring in all patients. The high polymorphism and very frequent seizures (not less than five types at every child) were observed. The cases were pharmacoresistant, with the absence of reaction to antiepileptic therapy and progressive deterioration in 4 (50%) patients. Decreasing of seizure frequency by 50% was achieved in 3 (37.5%) patients treated with the combination of valproates, benzodiazepines and barbiturates and by 75% in 1 (12.5%) patient case treated with valproates, benzodiazepines and levetyracetam (keppra). The authors proposed a definition of this epileptic syndrome as: "malignant epilepsy of infancy with migrating multifocal seizures".

[A use of high dosages of piracetam in the treatment of Kozhevnikov epilepsy syndrome].

An efficacy of high dosages of piracetam in the treatment of children with Kozhevnikov epilepsy syndrome (epilepsia partialis continua) has been studied. The study included 6 patients, aged from 9 to 16 years, with diagnosis of Kozhevnikov epilepsy (KE) who received piracetam (nootropil, UCB) in mean dose of 1g/kg/day (up to 35 g/day) intravenous in drops. The treatment course was 30 days. During therapy, there was decrease of myoclonus frequency by 75% in 3 patients and its full stopping in 3 patients. Focal clonic seizures were stopped in 2 cases, their frequency decreased in 4 cases. Secondary generalized seizures did not relapse in 2 cases and there was a decrease of seizures by 50% and over in 4 remained cases. The use of nootropil led to the improvement of neurological status in 5 out of 6 patients with KE. The intensity of hemiparesis was reduced in 3 patients treated with nootropil. The improvement of cognitive functioning was observed in 5 out of 6 patients. The duration of clinical improvement was 1-2 months after infusions cessation. The study revealed the efficacy of high dosages of nootropil in combination with basic antiepileptic drugs in the treatment of Kozhevnikov epilepsy.

[Epilepsy with electrical status epilepticus during slow sleep: diagnostic criteria and approaches to therapy].

Fourteen patients, aged from 5 to 14 years, with syndrome of electrical status epilepticus during slow sleep (ESESS) have been studied. The absence of epileptic attacks was observed in 21.5% of patients and diagnosis was established by a combination of continuous diffuse epileptiform activity with marked cognitive disturbances. In 78.5% patients, epileptic attacks presented as follows: pharyngeal and oral, hemicephalgia (100% patients with attacks), hemiclonic (18%), atypical absences (27%), negative myoclonus (18%), automotor (18%), focal adverse with vomiting (18%), secondary generalized (36%). Drugs of choice were valproate (depakine) and topamax in patients with attacks; suxilep and frisium in the absence of attacks and in case of continuous diffuse epileptiform activity on the sleep EEG. Two variants have been singled out by the character of ESESS syndrome. The first one, a "symptom variant", was featured by mostly hemiclonic, secondary generalized and automotor epileptic attacks, presence of continuous regional or lateral, less frequent diffuse epileptiform activity, detected by the local structural lesions in MRI, marked cognitive disturbances persisting after stopping of the attacks. The second ("idiopathic") variant is characterized by normal development of children before attack manifestation, appearance of mostly "rolandic" attacks, atypical absences and negative myoclonus, presence of exclusively continuous diffuse epileptiform activity in the phase of slow sleep, standard MRI results (moderate cortical subatrophy in single cases), cessation or substantial decrease of cognitive disturbances after attack stopping. It is emphasized that prognosis of ESESS syndrome should be considered separately with regard to attacks and cognitive disturbances. A prognosis for attacks is always favorable. Cognitive disturbances despite the therapy can persist for a long time.

[Control of the efficacy of the treatment of juvenile idiopathic generalized epilepsy and "pseudoremission"].

The aim of the study was to evaluate efficacy of antiepileptic therapy (AT) in patients with juvenile types of idiopathic generalized epilepsy (IGE) and to elucidate factors that may reduce it. Forty-four patients with IGE, who received AT and were featured, according to self-rating and relative's estimation, by stopping of the seizures, have been studied. Prolonged video-EEG monitoring revealed a high frequency of registered epileptic seizures in the apparent remission: in 28 cases (59.5%) out of 47, there was epileptiform activity and in 16 (34%)--epileptic seizures. The results allowed us to formulate a definition of "pseudoremission"--a condition, which occurs in patients with epilepsy treated by antiepileptic drugs when the seizures are not diagnosed by the patient and his relatives. Criteria of pseudoremission are the following: a verified diagnosis of epilepsy, antiepileptic therapy, no seizures as assessed by the patient and relatives, VEM evidence of the present epileptic seizures. Pseudoremission requires urgent correction of antiepileptic therapy and prolongation of the treatment.