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Neurology, Neuropsychiatry, Psychosomatics

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No 1S (2016):
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https://doi.org/10.14412/2074-2711-2016-1S

LECTURES

4-10 369
Abstract
The paper depicts the author's view on how a neurologist/epileptologist chooses an antiepileptic drug in his practice under present-day conditions. It considers possible clinical situations and therapeutic tactics in relation to the efficiency of performed therapy, as well as methods for switching to other antiepileptic drugs if the previous/first monotherapy is ineffective. The author gives main international and Russian guidelines in terms of the type of epileptic seizure/form of epilepsy/epilepsy syndrome. Despite its almost semicentennial history of effective clinical application, valproate is shown to be now a first-line choice drug for the therapy of undifferentiated, cryptogenic, and symptomatic partial epilepsies in patients of different age groups. The properties of valproate, such as efficacy against different types of seizures and forms of epilepsy, good tolerability, minimal aggravation risk, high monotherapy retention rates, various dosage forms, including the brand-name extendedrelease drug Depakine chrono or Depakine chronosphere and its intravenous formulation, favorable pharmacokinetic and pharmacodynamics profiles, make it indispensable at the present developmental stage of epileptology.

ORIGINAL INVESTIGATIONS

18-23 480
Abstract
Chronic tic disorders (CTDs) are one of the relevant problems of pediatric neurology, the higher prevalence of which is associated with undifferentiated therapy without considering comorbidity.
Objective: to enhance the efficiency of diagnosis and therapy of tic disorders in children and adolescents in terms of video-electroencephalography (EEG) monitoring data.
Patients and methods. The investigation enrolled 116 patients, including 83 (71.6%) boys and 33 (28.4%) girls at the age of 3 to 15 years (mean age, 9.0±3.0 years), diagnosed with CTD who had been examined at the Specialized Neurology Department, Voronezh Regional Children's Clinical Hospital One, in the period 2010–2014. The investigation consisted of two steps: 1) clinical, laboratory, and neurophysiologic  examination; 2) differentiated therapy. The Yaele Global Tic Severity Scale (YGTSS) was used. The diagnosis was established in accordance with the draft classification of epileptic seizures and epilepsy syndromes by the Intern ational League Against Epilepsy. Video-EEG monitoring was carried out in an awake state and during daytime and/or nighttime sleep.
Results and discussion. EEC epileptiform activity was recorded in 46.6% of the patients; a concurrence of CTDs and epilepsy was ascertained in 16.4% of cases. The risk factors of epilepsy in children with CTDs and EEG epileptiform activity are isolated motor tics in the facial region (p=0.0023), MRI epileptogenic changes (p = 0.01), a remitting course (p=0.02), and an early age at the full-scaled picture of tic disorder (p=0.02). CTD therapy with antiepileptic drugs (extended-release valproic acid) was effective and safe in both epileptic seizures (remission in 85.7% of cases) and tics with an improvement in 81.5% of cases with retention in therapy during 2–3 years. Therapy with dopamine transmissionlowering drugs was effective against tics; however, their intake for more than 6 months increased a risk for added epileptic seizures in patients with EEG epileptiform activity (the secondary bilateral synchronization phenomenon and epileptic seizures were recorded in 42.9 and 14.3% of the patients, respectively).
24-30 243
Abstract
Objective: to reveal and investigate hormonal characteristics in women of childbearing age in juvenile myoclonic epilepsy (JME).
Patients and methods. The concentrations of sex steroid and tropic hormones were analyzed in 48 women of childbearing age who suffered from JME and received monotherapy or bitherapy with antiepileptic drugs (AEDs) for more than a year. For comparison of their values, a control group included 15 healthy women who did not take AEDs.
Results and discussion. 66.7% of the patients were found to have ovarian hormonal dysfunction characterized by a significant increase in the level of luteinizing hormone and testosterone in the follicular phase of the cycle and a decrease in that of progesterone in the luteal phase compared with the control group. The hormonal deviations were influenced by disease duration and age-related onset in JME. Generalized tonicclonic seizures concurrent with myoclonic ones, bitherapy, and disease onset before menarche and in the period of the menstrual cycle favored the  development of hormonal deviations to a greater extent than myoclonic seizures only, monotherapy, and disease onset after the establishment of the cycle. Valproates were most commonly used in the therapy of JME; however, there were no significant differences in the hormonal deficiencies when different chemical groups of AEDs were administered.
35-37 301
Abstract
Objective: to analyze refractory status epilepticus (SE) cases.
Patients and methods. Fifteen female patients aged 21 to 62 years with refractory SE were comprehensively examined using long-term electroencephalography monitoring. The investigators evaluated the efficiency of treatment regimens with intravenous antiepileptic drugs (AEDs), such as diazepam (DZP); valproic acid (VPA); levetiracetam; and lacosamide and their combinations, at the prehospital and hospital stages, as well as SE therapy complications noted only in the intravenous administration of narcotics (propofol, sodium thiopental).
Results and discussion. A fetal outcome due to multiple organ dysfunction indirectly related to SE was recorded in 2 (13.3%) patients with acute symptomatic status. SE was abolished in the other 13 cases. The preliminary findings may suggest that it is appropriate to prescribe VPA just at the prehospital stage. The co-administration of VPA and DZP substantially enhances the efficiency of SE therapy. The maximum acceptable doses of AEDs using the whole available therapeutic arsenal should be administered within the first hours of acute symptomatic SE.
38-40 223
Abstract
Objective: to study the presence of comorbidities and to investigate quality of life in patients with main types of epilepsy in relation to age.
Patients and methods. The investigation enrolled 150 patients aged 17 to 64 years with idiopathic, cryptogenic, and symptomatic types of epilepsy. The investigators studied the presence of sleep disorders using the questionnaires designed by the Somnology Center, Ministry of Health of Russia, and that of daytime sleepiness by the Epworth Sleepiness Scale, determined the level of anxiety and depression by the Hospital Anxiety and Depression Scale, and assessed the severity of seizures by the National Hospital Seizure Severity Scale. Quality of life was investigated using the QOLIE-89 questionnaire. A cluster analysis was carried out to divide all the patients into three age groups (mean age, 27.3; 30.7; and 37.7 years.
Results and discussion. A positive relationship was obtained between some indicators of sleep disorders, level of anxiety, and age. There were significant differences between the above three age groups in the following indicators: sleep disorders; daytime sleepiness; level of anxiety, severity of seizures, and scores of the QOLIE-89 questionnaire (p<0.001). Since the patients suffer from different basic types of epilepsy (cryptogenic, idiopathic, and symptomatic), the findings testify to age as an independent factor that modifies quality of life in epileptic patients.
41-46 230
Abstract
Objective: to study clinical symptoms and brain activity in new forms of infantile epilepsy, such as malignant migrating partial seizures in infancy (MMPSI) and Markand-Blume-Ohtahara syndrome (MBOS) and to elaborate their differential diagnostic criteria for the timely choice of treatment policy and for the prediction of the disease.
Patients and methods. Thirty-eight children aged 1.5 months to 3 years with MMPSI and MBOS were examined. Their medical history and neurological examination data, electroencephalographic and neuroimaging characteristics, and the efficiency of chosen anticonvulsant therapy were investigated.
Results and discussion. The investigators revealed the following characteristics of these forms: the causes of these syndromes were highly diverse and unspecific; the neurological status was characterized by a variety of symptoms with an obvious delay in psycho-prespeech development concurrent with a high rate of partial focal (in MMPSI) and generalized (in MBOS) status epilepticus; The EEC characteristic sign was MISF and
«lafa» patterns in the children with MBOS and continuous migrating partial ictal status epilepticus patterns in those with MMPSI. These forms of epileptic encephalopathies are extremely drug-resistant and characterized by a high risk for a fatal outcome.
47-53 339
Abstract
Crowdsourcing and crowdfunding in public health should be considered as an untraditional process of gathering new ideas and assets. Theirefficiency is associated with that a large number of people may be involved in using their specific knowledge to solve complex problems and projects. According to statistics, the annual incidence rate for epilepsy averages 70 per 100,000 population and the disease starts in childhood in
nearly half of the cases. So the early differential diagnosis of paroxysmal states in children at the early stage of the disease is a necessary condition for adequate drug therapy.
Patients and methods. Only in the period from 2007 to 2009, the Center for Diagnosis and Treatment of Epilepsy and Sleep Disorders in Children and Adolescents, Department of Nervous System Diseases, Saint Petersburg State Pediatric Medical University, examined 259 children aged several weeks to 18 years with paroxysmal disorders of consciousness. Among them, there were 156 (60.23%) boys and 103 (39.77%) girls.
Results and discussion. Based on the results obtained during the children's  comprehensive examinations using video-EEG monitoring, the investigators specified diagnoses in all the examinees and changed treatment in the vast majority of cases. Many nonepileptic paroxysms with external manifestations resemble epileptic seizures; these are rather frequently qualified as erroneous and treated as such. Only the comprehensive examination involving video-EEG monitoring may avoid misdiagnosis in children with different epilepsy types and nonepileptic paroxysms. Video-EEG combines the video monitoring recording of EEG readings and makes it possible to reveal epileptic activity during a seizure, to compare the clinical presentation of the latter with EEG changes, to locate an epileptogenic focus, and to differentiate epileptic seizures from
nonepileptic ones. The effective diagnosis of paroxysmal states in childhood is a complex scientific and social problem, the solution of which requires that the government should invest a great deal of assets and may be rational only when crowd technologies are applied.
54-58 247
Abstract
Objective: to study the epidemiology of afebrile seizures (ASs) in the pediatric  population of the Trans-Baikal Territory in 2004 to 2013.
Patients and methods. The data available in the registry of patients with epilepsy and convulsive syndromes in childhood (febrile seizures and isolated convulsion) from the Trans-Baikal Territorial Antiepileptic Center (TAEC) over 2004–2015 were retrospectively
analyzed.
Results and discussion. Since the time of setting up the TAEC, the incidence of ASs in the Trans-Baikal Territory ranged from 0.23% (64 cases in 2005) to 0.49 (128 cases in 2008). The prevalence rate for ASs increased from 1.37 (431 cases in 2004) to 4.71 (1232 cases
in 2013) per 1,000 pediatric population. The prevalence and incidence of ASs did not substantially differ among the children living in Chita and in the areas of the Trans-Baikal Territory. The rate of AS cases in the structure of the registry of children with epilepsy and
convulsive syndromes decreased from 94.31% in 2004 to 83.19% in 2013. ASs were prevalent in children aged 3 years and 1 month to 7 years. The AS rate ratio between the boys and girls varied from 1:1.1 to 1.1:1.
11-17 250
Abstract
Objective: to generalize the Russian experience with perampanel (PER) in routine clinical practice; to do this, the results of its use as an adjuvant partial epilepsy medication were retrospectively assessed. The study is still in progress now; therefore, the paper gives its preliminary results. Patients and methods. The investigation included 52 patients with drug-resistant partial epilepsy. Their mean age was 28.92±14.02 years (a
small number of the patients had not attained the age of 12 years); the proportion of men was 56%; the disease duration was over 10 years (69.2%); symptomatic epilepsy was in 76.9% with an epileptic focus being in the frontal (46.2%) and temporal (44.2%) regions. PER was prescribed to the majority (71.2%) of patients after three previous therapy lines. The baseline monthly rates of all types of seizures were 127.29±82.29; those of generalized seizures were 6.72±1.90.
Results and discussion. After addition of PER to therapy just within the first month, there was a significant reduction in the frequency of all types of seizures to 52.06±29.26 per month (Sign test; p = 0.00001) and in that of secondary generalized seizures to 3.71±1.71 (Sign test; p=0.00001). The duration of PER administration was more than 6 months in the overwhelming majority of cases. In 58% of the patients, the frequency of seizures decreased by more than 50% (respondents). The lack of all types of seizures was noted in 8%; that of only secondary generalized seizures was in 31%. Adverse  events were observed in 30.1% of the patients (aggression in 11.5% and somnolence in 9.6%; others were seen more rarely). The dose of PER was decreased because of side effects in 7 (13.5%) patients; the drug was discontinued in 4 (7.7%). The mean dose of PER for adults was as high as 6 mg.

REVIEWS

59-65 263
Abstract
Antiepileptic drugs (AEDs) may have a negative effect on bone tissue, by increasing the risk of fractures in epileptic patients compared to the general population. Many investigations have shown lower bone mineral density and a higher risk for osteopenia and osteoporosis in patients taking traditional and novel AEDs. Multidrug therapy and the duration of AED intake are associated with the most significant risk for lower bone mineral density. Nevertheless, the molecular mechanisms of action of different AEDs on bone tissue remain little studied.

CLINICAL OBSERVATIONS

66-68 353
Abstract
Epilepsy belongs to the diseases that show the high rate of comorbidity with another abnormality. The complexities of management of patients with two chronic diseases who need continuous treatment are related to the possible impact of therapy for one disease on the course of the other, to worse overall prognosis, and to poorer quality of life in the patients. This clinical case demonstrates the concurrence of the two independent neurological diseases – epilepsy and myasthenia. The treatment of both diseases in this case has proven successful. The treatment performed for one disease has failed to aggravate the course of the other. The performed therapy could achieve a complete remission in both epilepsy and myasthenia.

LONG-TERM MANAGEMENT OF PATIENTS WITH EPILEPSY

69-73 288
Abstract
Epilepsy is a chronic brain disease that requires a long-term or lifelong follow-up of patients. The factors that influence the outcome of therapy are various; moreover, the key factors of them are the organization of care and the mode of patient follow-up. The necessity of optimizing the specialized health care to this category of patients stems from the poorer quality of life in the patients and their families and from the more frequent
development of mental disorders and drug-resistant forms of the disease during ineffective therapy. These and other issues are considered in this paper with emphasis on the specific area – the south of the Tyumen Region. This underlines the importance of setting up not only highlevel epileptologic centers, but also a whole network that provides access to specialized epileptologic care. It seems today that it is more appropriate to solve problems in the organization of management for epileptic patients at the level of an administrative region, for this state structure has all necessary conditions for rapidly and rationally implementing the most successful developments of specialized care to the population.


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ISSN 2074-2711 (Print)
ISSN 2310-1342 (Online)