AUTHENTIC ARTICLES
Typical absences (TA) are brief primary generalized epileptic seizures characterized by sudden onset and termination. According to their definition, absences consist of impairment of consciousness that is synchronously accompanied by electroencephalographic (EEG) changes as generalized spike–slow wave discharges of 3 or more Hz.
The authors conducted an investigation of 1261 patients with different forms of epilepsy with onset of seizures from the first days of life to the age of 18 years. The patients were followed up from 1990 to 2010. Absence seizures were detected in 231 patients, which accounts for 18.3 % of all the epileptic patients. TA were found in 102 patients, which constitutes 8.1 % of all cases of epilepsy with onset of seizures beyond the age of 18 years. The paper provides a detailed analysis of a group of patients with TA in terms of anamnestic, clinical, electroencephalographic, and neuroimaging features and the results of therapy with antiepileptic drugs (AEDs).
The age of onset of TA-associated epilepsy was from 9 months to 17 years (mean 9.4 ± 4.06 years). The disease occurred most frequently in young school-age children (41.2 %). Isolated TA as the only type of seizures were observed in the clinical picture of 28 (27.5 %) patients. TA were concurrent with other types of seizures in other cases. The investigators have identified 4 types of seizures which TA (generalized convulsions, myoclonic seizures, febrile seizures, and eyelid myoclonia) may be concurrent with. Neuroimaging stated there were no brain changes in 85.3 % of TA-associated epilepsy cases. Moderate diffuse subatrophic changes were detected in other cases (14.7 %). Local cerebral structural abnormalities were absent. The use of antiepileptic therapy as both monotherapy and polytherapy using different combinations showed the high efficacy of AEDs. Complete remission was achieved in 84.3 % of TA-associated epilepsy cases. An AED-induced reduction in the frequency of seizures was observed in 15.7 % of the patients. No one case – without effect. The greatest success in relieving seizures was seen in juvenile myoclonic epilepsy; childhood absence epilepsy was intermediate; the lowest percentage of remission was noted in juvenile absence epilepsy. The most effective drugs were valproic acid and ethosuximide used both alone and in combination. Lamotrigine, topiramate, and levetiracetam were used as part of combination therapy.
Juvenile myoclonic epilepsy (JME) (Janz syndrome) is a form of idiopathic generalized epilepsy (IGE) of adolescence, which is characterized by massive bilateral myoclonic seizures occurring predominantly in the arms on awakening from sleep. A Proposed Diagnostic Scheme for People with Epileptic Seizures and with Epilepsy (2001) refers JME to a group of IGE with a variable phenotype. JME is one of the most com- mon forms of epilepsy (7 % among all forms of epilepsy and 27 % among its all idiopathic forms). The authors describe the history of epilepsy, the specific features of the course of the disease, its clinical manifestations, the characteristic types of seizures, and approaches to diagnosis and therapy. They also proposed the results of their investigation, the aim of study were epidemiological pattern of JME and the specific features of its therapy and remission in the Volgograd Region. The investigation showed that 83 % of the patents with JME in the Volgograd Region could achieve remission with medical treatment, as shown by the EpiCenter. Valproates are the drugs of choice (remission was achieved in 92 %). The most common combination was levetiracetam and valproate (71 % remission). Complete clinical and electroencephalographic remission of JME was achieved in 41 % of cases, valproates therewith remain to have benefits too (58 % remission).
The Lennox–Gastaut syndrome (LGS) is an epileptic encephalopathy, starting in childhood and showing in often polymorphic seizures (including tonic axial ones), severe cognitive deficite, slow activity of the acute–slow wave in the interictal period at the electroencephalography (EEG), runs of fast activity of 10–20 Hz, often associated with tonic seizures, as well as with the resistance to therapy. According ILAE Classification of epilepsy syndromes and epilepsies 1989 LGS was referred to generalized cryptogenic or symptomatic forms of the epilepsy. According to Proposed Diagnostic Scheme for People with Epileptic Seizures and with Epilepsy (2001) LGS is a classic representative of the group of childhood epileptic encephalopathies. LGS is a rather rare form of the epilepsy. The syndrome frequency makes from 1–4 to 6.6 % among all forms of the childhood epilepsy. LGS is subdivided into the cryptogenic and the symptomatic variants. From our point of view the latter it will be more correct to refer to the symptomatic focal epilepsy with the secondary bilateral synchrony phenomena at EEG. The LGS can be caused by cortical development defects, by perinatal encephalopathies, by brain tumors, by inherited metabolism diseases, by chromosomal anomalies, as well as by other factors. In case of the classic cryptogenic variant the ethiology of the LGS remains unknown. The disease onset is at the age of 2–8 y. o. In 20–40 % of cases LGS is transformed from the West syndrome. The LGS attribute is the polymorphism of seizures. The syndrome structure can combine tonic seizures, epileptic drop seizures, atypical absences, generalized tonic-clonic seizures. Focal seizures at LGS are a matter of argument.
The article gives details on the clinical EEG criteria of LGS, the semiology of epileptic seizures in the syndrome structure, diagnostic and treatment approaches. The main accent is made on EEG peculiarities of the disease. The author presents the detailed survey of the modern literature on this matter, as well as the own research data.
REVIEWS AND LECTURES
Lennox–Gastaut syndrome (LGS) is childhood-onset epileptic encephalopathy characterized by frequent polymorphic seizures, including tonic, axial, obvious cognitive impairments, interictal EEG slow spike-wave discharges with fast activity at 10–20 Hz, which are frequently associated with tonic seizures and resistance to therapy. Treatment for LG presents great challenges due to the fact that its clinical picture is characterized by a concurrence of a few types of seizures and their resistance to drug therapy. In this connection, there are great expectations for the design of new antiepileptic drugs that have radically other mechanisms of action and must aim specially for the treatment of this severe form of epilepsy. The authors review the foreign literature on the use of the new in Russia antiepileptic drug rufinamide (Inovelon) registered just for the treatment of adult patients and in children who are at least 4 years old. Rufinamide (Inovelon) was registered in Russia in January 2015 as adjunctive treatment of seizures associated with LGS in patients from 4 years of age. The paper describes the mechanism of action, pharmacokinetics, efficacy, and tolerability of rufinamide in LGS. Numerous trials have demonstrated the efficacy and good tolerability of rufinamide in the treatment of LGS.
In spite of substantial progress in epileptology, complete seizure control cannot be achieved in a considerable proportion of patients (about 30 %); in this regard, the synthesis of new antiepileptic drugs (AEDs) and rational combinations of available AEDs in view of their mechanisms of action remains relevant. Of particular interest are drugs with different mechanisms of action in relation to other AEDs (these drugs include levetiracetam, lacosamide, perampanel, etc.). The authors provide a literature review dealing with one of the new drugs – levetiracetam (Keppra) that has a different mechanism of action, as compared with other AEDs. Levetiracetam has been approved for use as initial monotherapy for partial-onset epilepsy with or without secondary generalization in patients who are at least 16 years of age, as adjunctive treatment of partial-onset seizures with and without secondary generalization in babies who are at least 1 month old (oral solution) or in children who are at least 6 years old (tablets), and in patients who are at least 12 years of age as adjunctive treatment of myoclonic seizures in juvenile myoclonic epilepsy and as therapy for generalized tonic-clonic seizures in idiopathic generalized epilepsy. The review details the mechanism of action of levetiracetam, its pharmacokinetics, and data on its efficacy and tolerability in the treatment of epilepsy. The findings have led to the conclusion that levetiracetam is a well-studied and promising drug used in the monoand polytherapy of different forms of epilepsy.
Attention deficit hyperactivity disorder (ADHD) is a serious problem to pediatric neurologists. The prevalence of ADHD in developed countries ranges from 1 to 20 %. ADHD is characterized by a triad of symptoms: inattention, hyperactivity, and impulsivity (the International Statistical Classification of Diseases, 10th revision, codes it as F90) and it is the most common conduct disorder in children. The etiology of ADHD remains disсutable to the present day; there are a few basic concepts of the origin of this disorder. Its manifestations may be a reason for family conflicts, poor peer relationships, social and school maladjustment, learning problems, lower academic performance, accidents and injuries, smoking, psychoactive substance abuse (toxicomania, narcomania), delinquencies, deviant social behavior, thus having a negative impact on all spheres of a patient’s life. The manifestations of ADHD may continue in adulthood, resulting in work and family life problems, low self-evaluation, alcohol and psychoactive substance abuse, and other unfavorable consequences. The authors describe the etiology, pathogenesis, diagnostic principles (diagnostic scales and tests), differential diagnosis (by setting out a large group of different diseases, the manifestations of which can mimic ADHD), treatment, and prognosis of the disorder. Within its therapeutic correction framework, the authors present the definition and general principles of Montessori therapy, including recommendations for parents and relatives to deal with children with ADHD.
Attention deficit hyperactivity disorder (ADHD) is the most common cause of behavioral disorders and learning problems at preschool and school ages.
Patients with ADHD are frequently found to have comorbidities that present additional diagnostic and therapeutic problems and result in an even greater decrease in quality of life in patients. Thus, ADHD is frequently concurrent with epilepsy; in this case, not only the manifestations of ADHD are more common in epilepsy, but the latter is more often encountered in patients with ADHD than in those as a whole. The authors consider the epidemiological factors and causes, which may lead to a concurrence of these two diseases in the same patient, the principles of diagnosis and differential diagnosis of ADHD in epileptic patients, the specific features of treatment in this patient category. At the same time, the authors place emphasis on the fact that some antiepileptic drugs, such as barbiturates, may cause symptoms that mimic ADHD (in these cases, inattention and hyperactivity are adverse reactions of therapy and lessen or disappear after drug discontinuation) or enhance the manifestations of ADHD in patients with a concurrence of these two diseases. On the other hand, care should be also taken to choose drugs for the treatment of ADHD due to the possible higher frequency of seizures. So preference should be given to the drugs having no negative effect on the course of comorbidities or those having a positive therapeutic effect against both diseases.
ADVANCED TRAINING
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