[Opsoclonus-myoclonus syndrome in children].
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Biomedical subjects
Publications and source records attributed to A S Petrukhin.
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Clinical efficacy and safety of reminyl monotherapy has been studied in patients with dementia of Alzheimer's and mixed vascular-degenerative types. The reminyl treatment has been conducted in dosage of 16 mg/kg daily during 6 months in 30 patients (20 female, 10 male) aged from 59 to 86 years. The results of the study revealed high efficacy of reminyl in the treatment of patients with mild and moderate dementia and good tolerability of the medication.
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.
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The aim of the study was to evaluate efficacy and tolerability of topamax in patients of different age with various types of epilepsy. The drug was prescribed as monotherapy and combined therapy to 114 patients (53 men, 61 women) who were divided into age groups as follows: early age children--16 patients, preschool and school children--20, pubertal children--16, adolescent--23, middle-age patients--38, elderly--1. During topamax treatment, a complete remission was achieved in 48% patients, reduction of seizures frequency (more than by 50%)--in 44% patients. Topamax was more effective by remission index in the pubertal children, adolescent and adults as compared to early age children. The same peculiarity was characteristic of topamax in the treatment of symptomatic epilepsy. This drug was well-tolerable in all the groups studied, with isolated cases of moderate side-effects (body mass reduction, irritability, allergic skin reactions, paresthesias).
The article considers behavioral disturbances in children with anomalies of the cerebellum found by MRI studies. Presented are literature data on the relations between pathology of the cerebellum and early autism in children. The cerebellum is involved not only in movement coordination but also in social adaptation and speech communication. Cerebellum-specific genes expressed in early age are similar to those of hippocampus. Our own study of children with agenesis of the vermis cerebelli detected by MRI and behavioral disturbances included 20 children aged 3-15 years (mean age 7,05 years, 12 male, 8 female). Some autistic features have been found.
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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.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
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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.
The study aimed at a comparative analysis of safety and efficacy of valproic acid (valproate) and barbiturates in the treatment of epilepsy in children. Two hundred and forty children were treated with valproate, 94% being assigned to depakine and depakine chrono, and 210 children received barbiturates. Therapeutic effect (a decrease of seizures frequency by 2 and more times or remission) was detected in 82 of 127 (65 +/- 8.53%) patients for valproate monotherapy and only in 26 of 89 (30 +/- 9.45%) for barbiturates monotherapy. An efficacy of antiepileptic therapy in children was significantly higher (p<0.05) for valproates as compared to barbiturates. A drug withdrawal due to poor tolerability was recorded in 6 of 127 (5 +/- 3.7%) patients treated with valproate in monotherapy and in 14 of 210 (7 +/- 3.45%)--in polytherapy; in 53 of 89 (59 +/- 10.2%) patients treated with barbiturates in monotherapy and in 78 of 121 (65 +/- 8.53%) patients treated with barbiturates in polytherapy. Therefore, adverse effects occurred more often in barbiturates than in valproate treatment both for mono- and polytherapy (p<0.05). The results of the study confirmed the high efficacy and safety of valproates, specifically depakine chrono, in the treatment of epilepsy in children. depakine chrono in-take is associated with lower frequency of adverse effects; side-effects are mostly of dose-dependent character and do not result in the drug withdrawal. The authors do not recommend using barbiturates in the first-line treatment in children, because of the lower efficacy, high frequency of medical complications that might result in the drug withdrawal and reducing of the efficacy of other antiepileptic medications.
This study includes 90 children (41 female and 49 male) in the age range of 2-16 years with acute disseminated encephalomyelitis (ADEM). Thirty-three patients developed ADEM following rubella infection, 26 children following varicella infection, 20 suspected viral aetiology ADEM and 11 multiphasic disseminated encephalomyelitis (MDEM). All patients had neurological, routine laboratory and viral serology study with an enzyme-linked immunosorbent assay. Brain and/or spinal cord magnetic resonance imaging (MRI) were performed in 14 children. A follow-up study was in 1-5 years. Typing of DRB1 gene HLA class II was performed in 38 patients. We established that the varicella and rubella groups had preferential patterns. Rubella ADEM is characterized by acute explosive onset, seizures, coma and moderate pyramidal signs, whereas varicella infection is characterized by cerebella ataxia and mild pyramidal dysfunction. The suspected viral aetiology ADEM was characterized by polisymptomatic presentation. MDEM was characterized by older age of patients (11.6 +/- 2.8 years), more severe and prolonged local neurological symptoms, including myelitis symptoms and marked extrapyramidal signs, with distinct demyelination in MRIs. As a whole, ADEM is associated with DRB1*01 and DRB1*017(03) in the Russian population. Thus, ADEM is a separate autoimmune condition with a specific mechanism due to the type of genetic immunoregulatory base and specificity of viral trigger.
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.
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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.