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Biomedical subjects

K Iu Mukhin

Publications and source records attributed to K Iu Mukhin.

At least 19 recordsLinked to original sources

[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.

Adolescent↗

[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.

Adolescent↗

[Polymorphism of electroencephalographic pattern in benign epileptiform discharges in childhood].

Incidence and significance of benign epileptiform discharges of childhood (BECD), or rolandic spikes, have been studied in 2723 children, aged 2-15 years, with (841 patients) and without (1882) epilepsy. All the patients underwent standard electroencephalographic (EEG) study with video-EEG monitoring made in cases of epileptiform abnormalities. In the non-epileptic group, BECD frequency was 1.33%. There was a significant predominance of boys in both groups. The age of maximal BECD expression was 4-5 years in the non-epileptic group and 9-10 years--in children with epilepsy. The majority of children without seizures demonstrated different neurological and neurocognitive abnormalities, such as chronic headaches (25%), attention deficit hyperactivity disorder (25%) and speech delay (21%). Epileptiform abnormalities were observed mostly in the right hemisphere in children with epilepsy and in the left hemisphere in non-epileptic patients. There was a morphological similarity of electroencephalographic patterns in patients with rolandic epilepsy, benign occipital epilepsy, pseudolennox syndrome, Landau-Kleffner syndrome and electrical status epilepticus during slow sleep. The authors conclude that BECD are nonspecific feature of rolandic epilepsy and can occur in the broad spectrum of disturbances forming "hereditary impairment of brain maturation" group. Hereditary mechanisms involved in realization of various electroclinical features of focal brain dysfunction are suggested.

Adolescent↗

[Topamax in monotherapy of epilepsy].

Thirty-three patients, aged 3-29 years, with the following epileptic types: symptomatic forehead (15), symptomatic temporal (6), symptomatic occipital (2), juvenile myoclonic, in combination with eyelid myoclonus syndrome with absences, (5), epilepsy with isolated generalized seizures (3) and rolandic epilepsy (2), were treated with topamax. A medication dose was 50-200 mg per day in children younger 12 years and 100-550 mg per day in those older 12 years and in adults. The results obtained suggest the high efficacy and well tolerability of topamax in monotherapy of epilepsy. Therapeutic effect was achieved in 28 out of 33 patients (84.8%), i.e. seizures stopping--in 18 patients (54.5%). Monotherapy was mostly effective in symptomatic forehead epilepsy: seizures stopped in 53.3% patients and a frequency of seizures reduced in 33.3%. Side-effects were detected only in 18% cases, they were mostly transient and resulted in treatment withdrawal in 6% patients only.

Adolescent↗

[Gelastic seizures: etiology, semiology, therapeutic perspectives].

Gelastic seizures (laughing seizures) are a rare type of epileptic seizure in which laugh in a main and dominating manifestation of the seizure. As a rule, the seizures are caused by organic cerebral pathology and are often reported as a specific epilepsy marker related to hypothalamic hamartoma. The interictal EEG frequently shows a focal activity. Based on examination of 2 patients with gelastic seizures and hypothalamic hamartoma, clinical features, EEG characteristics and therapeutic perspectives for the disorder are discussed.

Adolescent↗

[Clinical efficacy and tolerability of topamax in combined therapy of epilepsy].

Topamax in combination with other antiepileptic medications was used in a treatment of 45 patients with different epilepsy types. The efficacy of topamax was shown in all types of epilepsy and in most types of epileptic seizures. Positive effect was revealed in 69% of the cases, in 22.5% complete remission was achieved. The best results were obtained in idiopathic generalized epilepsy (in respect to stopping generalized tonico-clonic seizures) and in the cases of symptomatic (cryptogenic) partial epilepsy (in the treatment of simple partial motor and secondary generalized seizures). Seizures aggravation was only 6.5% in complex partial and tonic axial paroxysms. In 19% of the patients, topamax efficacy decreased, with seizures frequency gradually returning to a baseline 1-3 months after the positive effect achievement. Side effects (SE) were detected in 24% of the cases. The most frequent ones were dose-dependent SE in relation to CNS in the absence of idiosyncratic reactions. Therapy withdrawal because of SE was necessary only in 11% of the cases. Topamax is considered a promising antiepileptic medication.

Adult↗

[Electroclinical characteristics of Landau-Kleffner syndrome].

The study aimed at investigating epileptic attack's semiology and other electroclinical characteristics in Landau-Kleffner syndrome as well as therapeutic efficacy. Six patients with Landau--Kleffner syndrome, 5 boys, 1 girl, aged 6-10 years, mean age 7.5 years, have been analyzed. Epileptic attacks were observed only in 3 patients with debut at the age of 2.5-6 years (mean 4 years). In 50% of the cases, the attacks were not detected. The types of epileptic attacks were specified as follows: atypical absences--3 patients; pharyngo-oral--2; secondary generalized--2; atonic falls--1; hemiconvulsive--1. In routine EEG study, regional epileptiform activity was observed in all the patients, being localized in central temporal (3 cases), posterior temporal (1) and fronto-temporal (1) leads. In 3 cases (50%), diffusive epiactivity was also detected. During sleep, emergence of prolonged diffuse epiactivity was revealed for all the patients, with appearance of electrical status epilepticus during slow sleep in 50%. Valproates in dosage 30-60 mg/kg daily were highly effective for stopping epileptic seizures. Significant improvement of speech functions was observed only if antiepileptics sulthiame (ospolot) or clobasam (frizium) were used in addition to basic therapy. Clobasam in dosage 0.5-0.75 mg/kg daily was the most efficient in blockade of EEG diffuse epileptiform activity, reduction of aphasia symptoms and behavior improvement.

Anticonvulsants↗

[Diagnostic differentiation between two syndromes of progressive myoclonus epilepsy].

The study aimed to differentiate, according to clinical and electroencephalographical criteria, between the most frequent types of myoclonus epilepsy--Unverricht-Lundborg disease (ULD) and Lafora disease (LD). Two patients with ULD and two with LD, aged 13-16 years, have been examined. In all cases, the diagnosis of myoclonus epilepsy has been verified by using molecular genetic analysis. The differential diagnostic criteria have been ascertained for ULD and LD: the earlier age-of-onset in ULD comparing to LD (8.8 and 12.5 years, respectively); tonic clonic paroxysms in ULD and partial ones with visual aura in LD; a presence of nonepileptical subcortical myoclonus, according to EEG data (visual monitoring); negative myoclonus emerging in manifested stage of LD; appearing of regional epileptiform activity for posterior regions on EEG in LD; more rapid progressiveness of extra pyramidal disturbances with organic dementia outcome in myoclonus LD.

Adolescent↗

[Electroencephalographic characteristics of West syndrome].

The study aimed at interictal electroencephalographic characteristics investigation in the patients with West syndrome. It has been carried out in 48 children, aged 3 months--2 years, the inpatients of Psychoneurology and Epilepsy Department, Russian Clinical Children Hospital No. 2, from March 1999 to march 2001. The following EEG awakeness types were detected: typical hypsarrithmia--8 (16.7%) patients; different variants of modified hypsarrithmia--35 (72.9%); a presence of focal epileptiform discharges, but not in the form of modified hypsarrithmia with focal component--3 (6.2%); an absence of epileptiform discharges--2 (4.2%). Typical and modified hypsarrithmia cases ratio was estimated as 18.6%:81.4%. Among the patients with modified hypsarrithmia, the following variants have been detected: synchronized variant of modified hypsarrithmia--35.3% of the cases with modified hypsarrithmia; asymmetric regional or unilateral hypsarrithmia--42.9%; hypsarrithmia with partial component--45.7%; hypsarrithmia with persisting "suppressive-burst" pattern--20% of the cases. Correct evaluation of clinical, electrophysiological and neurovisual data (clinico-electro-anatomical approach) facilitated the rational differential choice of antiepileptic therapy. The approach allows the early prognosis of disease course and its transformation to other epilepsy types.

Brain↗

[Diagnostic criteria of atypical benign partial epilepsy syndrome in childhood].

The paper presents an analysis of clinical-neuropsychological peculiarities of the syndrome of atypical benign partial childhood epilepsy (pseudolennox syndrome) of 6 patients (3 boys, 3 girls). An age of the onset of the disease was between 1.5-4 years. There was polymorphism of paroxysms, their high frequency with an obligate presence of hemifacial fits and atypical absences. Night generalized tonic-clonic attacks and the falling attacks were found in 67% of the patients. Spectrum of the neurological disorders included disorders of speech and a slight cerebella symptomatology. Regional "rolandic" activity and diffuse epileptiformed disorders, increasing into a phase of a slow sleep, were registered by EEG. Resistance to anticonvulsive therapy was revealed. The authors had demonstrated a nosologic independence of pseudolennox syndrome and had considered worth while to pick it out in a group of cryptogenic partial forms of epilepsy together with the epileptic aphasia of Landu-Kleffner and an epilepsy with the continuous peak-waves during the slow sleep.

Anticonvulsants↗

[Depakene (sodium valproate) in the treatment of epilepsy in children and adolescents: efficiency and safety].

375 patients 7 months-19 years of age with different forms of epilepsy were treated with depakine. High efficiency of the drug was found in all forms of children's epilepsy. A complete remission was observed in 60% of the patients, a good effect--in 19%, a satisfactory effect--in 15%, 6%--were non-responders. The highest efficiency of depakine was achieved in idiopathic forms of epilepsy (complete remission--in 74% of the cases) and, therefore, depakine can be used as a monotherapy. Meanwhile, the efficiency of depakine was lower in cryptogenic generalized and symptomatic partial epilepsy. In such cases depakine should be used as a basic preparation in polytherapy. Side effects were observed in 35% of the cases, but stopping of therapy was necessary only in 25% of such cases. Rare idiosyncrasy and no negative influence on the cognitive functions were important-properties of depakine.

Adolescent↗