Idiopathic childhood focal epilepsy with centrotemporal spikes, which is more known as rolandic epilepsy (RE), is age- and localization-related epilepsy with childhood onset, which is characterized mainly by short-lasting hemifacial and oropharyngeal seizures generally occurring when awakening or falling asleep, by the normal neurological status of patients, by specific electroencephalographic changes and complete arrest of seizures during therapy or when achieving puberty.

RE is the most common epilepsy in childhood. Its prevalence is 21 per 100,000 healthy children. It is characterized by an onset that is clearly related to age. In 85 % of cases, RE occurs at 4–10 years of age with its peak at about 9 years. The clinical manifestations of this form of epilepsy are several types of seizures, such as oropharyngolaryngeal, hemifacial, faciobrachial, secondarily generalized convulsive, unilateral seizures with the possible development of short duration Todd’s paresis. Other types of seizures (absence, atonic, and myoclonic ones) are uncharacteristic of RE. They may occur occasionally on aggravation resulting from the use of carbamazepine or oxcarbazepine or permanently on transformation into pseudo-Lennox syndrome. According to the definition, focal neurological symptoms and behavioral and intellectual disorders are absent in patients with RE. However, there have been recent observations suggesting that speech, cognitive, and behavioral disorders may occur (at the same time rarely) in children with RE. Magnetic resonance imaging (MRI) fails to reveal the abnormality in the vast majority of cases. The described MRI changes in nearly 10 % of the patients with RE are an incidental finding and irrelevant to the course of the disease. Valproate in small doses is a first-choice drug; if it is inefficient, levetiracetam, topiramate, or a combination of valproate and ethosuximide are administered. Sulthiame (ospolot) as monotherapy is used in Germany.

Based on their findings and the data available in the literature, the authors consider the etiology, pathogenesis, clinical manifestations of RE and its diagnosis, treatment principles, and prognosis. The specific features of the electroencephalographic pattern in this disease are detailed.

In accordance with current views, giftedness are considered as the systemic psychic property developing throughout a lifetime, which determines the human possibility of achieving higher (unusual, outstanding) results in one or several kinds of activities as compared to other people. People who are endowed with extraordinary abilities may be infrequently called absolutely somatically and mentally healthy. There are data that giftedness in childhood are frequently concurrent with both somatic diseases and different neurological disorders. Many gifted children are diagnosed as having left-handedness, speech disorders, and autoimmune diseases. There are scientific works on the association of giftedness with neurological and psychiatric disorders, including attention deficit/hyperactivity disorder (ADHD), learning problems (dyslexia in particular), autism (including Asperger’s syndrome), bipolar disorder, and migraine. According to the available data, approximately 3–5 % of children fall into a category of intellectually gifted ones; some children (2–5 to 20 % or more of all gifted children according to different findings) may have learning problems. The terms “twice-gifted” or “twice-exceptional” are proposed to characterize children with giftedness concurrent with learning problems, attention deficit (including ADHD), or other impairments of cognitive functions and behavior (including oppositional disorder and obsessive-compulsive disorder). These children need the more attention of teachers and other correction approaches and adaptation methods to be elaborated as compared to the procedures used for gifted children, on the one hand, and for those with learning problems, behavioral and attention disorders, on the other hand. There is a need for the development and further strengthening of strong suits (gift) and correction, adaptation of deficits in children with “twice exceptionality”. The review presents the history of studying the “twice exceptionality” phenomenon, ideas on its causes, approaches to differential diagnosis, and the specific features of correction methods.

Although epileptologists have a lot of antiepileptic drugs (AEDs) at their disposal now and there are more than 10 novel AEDs; until the present time, two groups of drugs (valproic acid and carbamazepine, which were designed in the 1970s) have remained the gold standard for the treatment of epilepsy. These 2 AEDs are called traditional or standard and the European literature on epileptology describes them as first-choice anticonvulsants. Carbamazepine has a narrower spectrum of therapeutic action and may aggravate a few types of seizures. On the contrary, valproates are effective in all forms of epilepsy, both focal and generalized ones. The anticonvulsant properties of valproic acid were discovered in 1962. Now valproates are successfully used in various age groups of patients with different forms of epilepsy and types of seizures during both mono- and polytherapy in more than 100 countries of Europe and the world, by firmly holding the leading position. A lot of cheaper valproic acid generic drugs have emerged in clinical practice in the past decades. By and large, almost 20 valproates that have one common active ingredient (valproic acid) and are commonly used to treat epilepsy are now available on the global pharmaceutical market.

The authors give the results of trials and describe their own experience with Valparin XR, an extended-release valproic acid drug. The results of the trials and clinical experience show the efficacy and good tolerability of Valparin XR in the treatment of epilepsy.

Kleine–Levin syndrome (KLS) is characterized by sleep attacks lasting several hours or days with imperative onset and awakening difficulty. The syndrome belongs to rare and little studied diseases not only in our country, but also throughout the world. It was first described in 1786. The diagnostic criteria for KLS include: 1) complaints of excessive somnolence; 2) sleepiness episodes lasting at least 18 hours daily; 3) at least 1–2 annual hypersomnia episodes lasting 3 days to 3 weeks; 4) predominance generally in adolescent males; 5) characteristic changes at polysomnographic study; 6) no relationship of hypersomnia to other somatic or mental disorders, such as epilepsy or depression; 7) no association with other sleep disorders, such as narcolepsy, sleep apnea, or nocturnal myoclonus. The etiology of the syndrome remains unknown. The disease may occur in the presence of trauma, metabolic disturbances, and other diseases. The likely etiology is considered to be viral or postinfection autoimmune encephalitis involving chiefly the hypothalamus. No specific treatment for KLS has been developed at present.

The authors consider the historical description of the syndrome, its presumed etiology, pathogenesis, and clinical manifestations, including the results of additional studies. They describe their observed clinical case of KLS that manifested itself as hypersomnia, hyperphagia, and psychopathic disorders and developed when using amphetamine and cannabinoids for the treatment of traumatic brain injury

The introduction of the latest genetic techniques into practice could discover a basis for the comorbidity of genetic epilepsies and behavioral disturbances with cognitive impairments. Some chromosomal syndromes are characterized by a specific electroencephalogram (EEG) pattern, the type of seizures, and the variant of the course of epilepsy. This paper describes a case of synaptic RAS GTP-ase-activating protein 1 (SYNGAP1) gene mutation in a 9-year-old female patient with eyelid myoclonic epilepsy, atypical absences, and atypical autism with mental retardation. The patient’s parents visited a physician for epilepsy (myoclonic absences), markedly delayed psycho-speech development, and specific communication problems in the child. The characteristics of autistic behavior were manifested from birth; routine EEG recorded epileptiform activity at the age of 2 years; epileptic seizures appeared at 5 years. Valproic acid and levetiracetam in this patient exerted a good effect on seizures; however, a clinical and encephalographic remission was achieved by a combination of levetiracetam and ethosuximide. The clinical case including the neurological and psychic statuses, logopedic characteristics, the result of psychological testing, and video-EEG monitoring findings are analyzed in detail.

The SYNGAP1 gene is located on chromosome 6p21.3. About 50 cases of SYNGAP1 syndrome are now known worldwide. After normal maternal pregnancy and delivery, the patients show delayed psychomotor development with pronounced regression at 1 to 3 years of age. At this age, there are diffuse polyspike discharges on the EEG or an onset of generalized epileptic seizures (atonic, myoclonic, eyelid myoclonic, and absence seizures), commonly photosensitivity and autoinduction, mental development stops, speech regresses, behavioral disorders that are typical of autism develop. Drug-resistant epilepsy is noted in approximately half of the described cases. There is a correlation between the severity of epilepsy and cognitive deficit.

В 1914 г. в Санкт-Петербурге по инициативе академика В. М. Бехтерева при Психоневрологическом институте была построена первая в России «Клиника для эпилептиков». На сегодняшний день ФГБУ «Санкт-Петербургский научно-исследовательский психоневрологический институт им. В. М. Бехтерева» – это крупный научно-исследовательский и клинический центр со специалистами мирового уровня в области психиатрии, психотерапии, медицинской психологии, наркологии, неврологии и нейрохирургии. В 2007 г. Институт отметил свой 100-летний юбилей. В этом году исполняется 105 лет со дня основания Российской противоэпилептической лиги и 140 лет со дня рождения Людвига Мартиновича Пуссепа – основателя Клиники нейрохирургии Психоневрологического института.