Pseudo-Lennox syndrome (PLS), or atypical benign partial epilepsy of childhood, is a disease from a group of age-related epileptic encephalopathies with a phenomenon of continuous spike-wave activity during slow sleep, which manifests itself as frequent polymorphic focal motor and pseudogeneralized seizures, cognitive impairments, as well as regional and diffuse epileptiform activity on electroencephalogram (EEG) by the morphology identical to that of benign epileptiform patterns of childhood. The disease was first described by J. Aicardi and J. J. Chevrie in 1982, based on a study of 7 cases. Its diagnostic complexity is the polymorphism of both epileptic seizures and EEG data, as well as low awareness of the syndrome among physicians and its absence in the international classification of epilepsies. The typical triad of seizures, which occurs in nearly 100 % of patients, encompasses focal motor paroxysms (identical to those as observed in Rolandic epilepsy), atypical absences, and atonic seizures. Seizures in PLS in its active period (generally up to 7–8 years) are highly resistant to antiepileptic drugs. Only a few agents have been proven to be effective in PLS; these include valproates, succinimides, benzodiazepines, topiramate, and sulthiame. The frequency of seizures are noted to increase in patients with PLS treated with drugs, such as vigabatrin, gabapentin, lamotrigine, phenobarbital, or phenytoin. The author considers in detail the history of studies of the disease, clinical manifestations, diagnostic criteria, therapeutic approaches, and prognosis.

The article presents the clinical, electroencephalographic (EEG) and neuroimaging features, as well as the results of treatment of patients with focal epileptic myoclonus (FEM) with the Kozhevnikov–Rasmussen syndrome (KRS). FEM in KRS-patients was identified in 11 cases, accounting for 0.9 % of all the cases of epilepsy with the onset of seizures up to 18 years (n = 1261). The age at onset of KRS ranged from 3 to 21 years (average – 9.2 ± 5.7 years). In the active period of the disease of all patients in the clinical picture the active FEM appeared and increasing frequency of the secondary generalized seizures (SGS). In addition SGS and FEM, the clinical picture of the disease in most patients (91 %) the focal motor (clonic) and the somatosensory focal seizures were observed. As the disease progressed, the FEM became more pronounced in frequency and intensity, seized more muscle groups, localizing mainly in the muscles of the trunk and limbs. The typical EEG pattern of FEM patients with KRS was regional epileptiform activity that occurs in the structure of the continued regional slowing localizing maximum of the fronto-central-temporal region. During the magnetic resonance tomography of the brain in dynamics all the patients observed the increase in total cortical hemiatrophy. In all the cases, the appointment of antiepileptic therapy resulted in a slowing of the FEM, however, a complete remission was reached at none of the patients. Two patients were made surgical treatment of epilepsy. In one case remission of epileptic seizures was observed after right-side hemispherotomy. Our study showed that FEM is very resistant type of epileptic seizures. This fact calls for the identification of the FEM at the early stages of the disease with the purpose to improve the prognosis, as well as for an earlier surgical treatment.

Examinations were made in 11 female patients aged 3 to 23 years with Rett syndrome and typical mutation in the MECP2 gene, who had been followed up from 2006 to 2015. The investigators evaluated neurological and psychic status (systematically) and performed continuous video electroencephalographic (EEG) monitoring (if clinically indicated), magnetic resonance imaging, and molecular cytogenetic examination. Epilepsy was diagnosed in 6 (54.5 %) patients: symptomatic focal epilepsy in 5 cases and symptomatic generalized epilepsy in 1. The mean age at epilepsy onset was 3 years and 9 months. Generalized epileptic seizures were noted in 60 % of cases and focal ones in 40 %. EEG changes were revealed in 9 patients, 5 with slowing of background activity, 2 with regional epileptiform activity, and 1 with diffuse epileptiform activity that corresponded to that of the benign epileptiform patterns of childhood. Five patients were recorded to have multiregional epileptiform activity.

Ornithine transcarbamylase deficiency (type II hyperammonemia) – X-linked metabolic disorder of the urea cycle, caused by mutations of the gene encoding ornithine transcarbamylase (OTC). Changes to the nervous system caused by degenerative processes in the gray and white matter of the cerebral hemispheres. The authors describe 1-year-old boy with ornithine transcarbamylase deficiency as a clinical example, with the onset of the disease in the first year of life, with refusal of food, vomiting, weakness and tiredness progressing to lethargy and unconsciousness, convulsive seizures, refusal from meat in the interictal period, delayed of psychomotor development. The child was admitted to the children’s intensive care unit in serious condition, unconscious with severe neurological symptoms. The clinical picture, the results of instrumental and laboratory examination and the presence of family history were the basis for the assumption of the hereditary origin of the disease. Genetic further examination was planned. In the context of children’s intensive care unit, the patient was undergoing of intensive therapy, which had no effect. Death occurred on the 5th day of hospitalization. To verify the diagnosis post-mortem autopsy was conducted, based on which was installed the immediate cause of death. In confirming the diagnosis is considered as tandem mass spectrometry, and DNA diagnostics.

The article describes the phenomenon of dual pathology – a combination of structural changes in the brain and benign epileptiform discharge of childhood on electroencephalogram. The uniqueness lies in the observation that the child, since birth suffering from spastic form of cerebral palsy and severe epilepsy, demonstrated the development of Stevens–Johnson syndrome due to intolerance of one of the antiepileptic drugs. Therapeutic approaches to overcome a whole range of violations are discussed in the article.

Osteopathy is a young medical specialty that has found official acceptance in Russia since 2013. The paper deals with the history of osteo pathy and its founder Andrew Taylor Still (1828–1917). He left a few printed scientific works, including his biography reporting much of his life and activities and the creation of osteopathy. The emergence date of osteopathy is considered to be 22 June 1874. Historically, modern osteopathy has come into existence not so long. We are almost contemporaries of it and its author, but despite this, the international osteopathy community is romanticizing and mythologizing the personality of A.T. Still. In our opinion, the reason is his magnetic personality and brilliant life. Thanks to A.T. Still’s personality, osteopathy may not have been lost among many medical areas and discoveries and continues to develop and attract new supporters. On the other hand, osteopathy is, in the opinion of its founder, a science; therefore a historical rather than mythological approach to A.T. Still’s bibliography, which takes into account all factors for personality formation, is considered correct. Modern science tells that these factors are inheritance, environment, and education. The authors have applied just this scientific approach and, without demolishing the myths, set forth an aim to study the factors forming the world outlook of A.T. Still in order to predict a further way to develope osteopathy in our country.

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