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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

[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↗

[Diagnosis and treatment of Rolandic epilepsy].

Rolandic epilepsy belongs to the most frequent forms of epilepsy in children. 43 patients aged 2-14 years were observed. Complex neurologic, genealogic, neurophysiologic and neuroradiologic examinations was performed. There was a microfocal symptomatology in the form of ptosis and syndrome of infantile cerebral paralysis. The changes found at neuroradiologic examination were not related to the location of an epileptic focus and didn't influence the prognosis of the disease. In 10 cases electroencephalographic patterns were observed in clinically healthy relatives. In all the patients a remission of the fits was achieved. Administration of low doses (20 mg/kg daily) of valproic acid (depakin) resulted in a remission of the fits.

Anticonvulsants↗

[Benign myoclonic epilepsy in infancy].

Benign myoclonic epilepsy in infancy (NMEI) is one of rare epileptic syndromes. 5 patients (all female sex) aged 4-16 years were observed. NMEI debuted at the age from 7 months till 2.5 years (mean age 1.3 years). Pathology of pregnancy and labor, disorders in both psychomotor development and genetic predisposition were not found. In all the cases the disease began with typical transitory repeated myoclonic paroxysms of different intensity and frequency, without loss of consciousness and with primary involvement of the muscles of the neck and the upper extremities. Most patients had muscular hypotension, mild coordinatory disorders, delayed psycho-speech development, mental retardation, EEG signs of generalized epileptic activity. Valproates, suxilep, clonazepam and lamotrigin (lamiktal) were used for treatment. The most pronounced effect was achieved using either monotherapy with valproates (depakin) or a combination depakin + lamiktal. A stable clinical-encephalographic remission was achieved in all the patients, but during puberty in 2 patients (15 and 16 years old) rare generalized convulsive fits debuted. High frequency of intellectual-mnestic disorders were found even after a complete remission. So benign definition concerns only a course of the fits, but not NMEI prognosis.

Age of Onset↗

[A computed tomographic study of epilepsy in children: the primary diagnosis of a brain tumor].

There are presented experience of computer tomographic (CT) investigation of 435 children with different epileptic syndromes, which had been verified according to international Classification of Epilepsy and Epileptic Syndromes (New Delhi [correction of New-Daily], 1989). Tumors were diagnosed in 6 cases and were manifested as resistant epileptic fits. There was grounded the necessity of including of the neuroimaging methods in complex study of children with epilepsy for optimization of diagnostics, treatment and prognosing of the course of epilepsy. There were also formulated the practical recommendations concerning carrying out of CT investigations in children with prolonged resistant epileptic syndromes.

Adolescent↗

[Television epilepsy].

Television epilepsy (TE) appear to be the variation of photosensitive epilepsy when the fits are provoked by watching TV. 23 patients aged 6-20 years were observed (8-men, 15 women). TE included epilepsy with generalized convulsive fits (34.8%), juvenile absence-epilepsy (17.4%), juvenile myoclonic epilepsy (30.4%), children absence-epilepsy (13.0%) and partial occipital epilepsy (4.4%). "Pure" TE was diagnosed in 26.1% of patients, while the combined TE--in 73.9%. Diagnostic criteria of the syndrome of "pure" TE were formulated.

Adolescent↗

[Side effects of anticonvulsants in the treatment of idiopathic generalized epilepsy].

This investigation was ainced on detection of frequency and main features of side effects of anticonvulsants in therapy of idiopathic generalized epilepsy. There were observed 190 patients aged 5-29 years suffering from different forms of idiopathic generalized epilepsy. Side effects were revealed in 48.4% of patients as a result of the investigation. Almost half of patients (49.0%) who were administered valproic acid developed side effects. Neuroendocrine dysfunctions and digestive disturbances were the most frequent. Side effects were revealed within the first two months after administration of valproats. Only in 13.2% of cases the treatment and to be stopped at the expense of severeness of side effects. Barbiturates caused side effects in 39.6% of cases. Toxic damage of CNS was the most significant. Hyperkinetic syndrome with lack of attention was observed 37.9% of cases. Succinimides caused side effects in 37.9% of cases. Toxic damage of CNS was the most frequent--in 22.4% of cases, digestive disturbances was in 15.5% of cases. There are no really effective means for correction of above mentioned side effects.

Adolescent↗

[Rasmussen's chronic progressive focal encephalitis].

Chronic progressive focal encephalitis or Rasmussen encephalitis (RE) is chronic brain disease of viral etiology. It is manifested by either partial motor or myoclonic seizures combined with hemiparesis. RE was described in detail in a girl of 8.5 years. The disease debut was observed at the age of 5.5 and manifested in the form of simple partial motor fits with serial episodes of secondary generalization. Post-attack hemiparesis was observed from the age of 7.5 and became permanent after 8. From this time myoclonic fits became constant, changed for the worse, memory and attention deteriorated. NMR tomography revealed widening and deepening of the Sylvian fissure as well as a focal decrease of left temporal area density. The patient was resistant to anticonvulsant, hormonal and immunosuppressive drugs. The criteria of RE diagnosis, its difference from Kozhevnikov's epilepsy are provided. Estimation of drug and surgical treatment effectivity is given. The necessity of early surgical treatment (functional hemisphere ectomy) is emphasized.

Brain↗