[Long-term follow-up of adult patients with serial and status course of epileptic seizures]. / Dolgosrochnyi katamnez vzroslykh patsientov s epilepsiei s epilepticheskim statusom ili seriinymi pristupami.

OBJECTIVE: To study the follow-up of adult patients with status epilepticus or a history of serial seizures, assessing the likelihood of achieving long-term remission and identifying predictors of treatment effectiveness. MATERIAL AND METHODS: The study included 280 patients divided into 137 patients with epilepsy with a series of seizures or a history of status epilepticus (group 1) and 143 patients, who had not previously received therapy and did not have a series of seizures or a history of status epilepticus (group 2). A clinical and neurological examination, analysis of medical documentation data, electroencephalography, and MRI were performed. RESULTS: After correction of therapy, remission in patients in group 1 was achieved in 21.9%, improvement in 30%, no effect was observed in 48.1%; in group 2 the indicators were 51%, 28.7%, 20.3%, respectively. The onset of epilepsy in childhood, frequent seizures, and regional epileptiform activity were associated with the lack of treatment effect. CONCLUSION: The results confirm the main role of the clinical examination in determining the prognosis of epilepsy in a particular patient. Currently available instrumental techniques have limited predictive value.

[The effect of the start date of cognitive rehabilitation after ischemic stroke on the level of recovery]. / Vliyanie sroka nachala kognitivnoi reabilitatsii posle perenesennogo ishemicheskogo insul'ta na uroven' vosstanovleniya.

OBJECTIVE: The dependence of result of cognitive training in patients who have suffered an ischemic stroke (IS) on the timing of their onset continues to be discussed. The aim was to study the results of cognitive rehabilitation of patients after IS during various periods after it. MATERIAL AND METHODS: 140 patients were examined during complex rehabilitation in terms up to 1, 2-3, 4-6 and 7-12 months after IS, 78 of them received drug support (DS) of rehabilitation with intravenous injections of ampasse. The Montreal Cognitive Assessment (MoCA) and Hospital Anxiety and Depression Scale (HADS) were used to monitor the effectiveness of rehabilitation. RESULTS: In all subgroups, there was a statistically significant increase in the MoCA score after the course, but number of people with an increase in the score by 1 or more points was highest among those who started the course 3 or more months after the development of IS (p=0.015). Among those who received DS, an increase in the MoCA was noted in 87.2%, in those who did not receive it - 38.7% (p<0.001). There was no statistically significant increase in the severity of anxiety and depression after the course of treatment in any of the subgroups. CONCLUSION: The used approach of a combination of cognitive, physical rehabilitation and DS proved to be justified for achieving results during a two-week course of inpatient rehabilitation of patients both in the early and late recovery period after IS.

[Assessment of disorders of neurodynamic processes in patients with acute ischemic stroke]. / Otsenka narushenii neirodinamicheskikh protsessov u bol'nykh v ostrom periode ishemicheskogo insul'ta.

OBJECTIVE: To develop a method for assessing neurodynamic disorders in the acute period of stroke. MATERIAL AND METHODS: Sixty-three right-handed patients in the acute period of ischemic stroke (IS) were examined, including 28 patients with IS in the basin of the left middle cerebral artery (LMCA) (group 1), 19 patients with IS in the basin of the right middle cerebral artery (group 2) and 16 patients with IS in the vertebrobasilar system (group 3). The control group consisted of 37 healthy individuals. The «Tapping Test¼ technique developed by E.P. Ilyin was used to assess the strength of nervous processes. The mobility of nervous processes was determined by a complex sensorimotor reaction of discrimination. To assess the balance of the processes of excitation and inhibition, a complex sensorimotor reaction to a moving object was used. RESULTS: There was a significant decrease in the strength of nervous processes in the patients compared to the control group (p<0.0001), more pronounced in IS in LMCA. When checking the mobility of nervous processes in patients of groups 1 and 2, the number of errors was statistically significantly higher than in the control group (inertness of nervous processes). These indicators were the highest in patients with IS in LMCA while in patients of group 3 the lowest values, approaching the data of healthy individuals, were noted. When checking the balance of nervous processes, torpidity of reactions was noted in patients of group 1, torpidity when performing the test with a paretic hand was noted in group 2. In group 3 these indicators approach the data of healthy individuals, which reflected the approximate balance of the processes of excitation and inhibition. CONCLUSION: In the study of neurodynamic processes in patients in the acute period of IS, there was a decrease in strength, inertness, and torpidity of the mobility of nervous processes, more pronounced in the damage of the dominant hemisphere. It can be assumed that in the origin of these shifts in patients in the acute period of IS, a diaschisis plays an important role, and with left-sided localization of IS, not only the focal, but also its connective, transcallosal form.

[Ampakines - a promising approach to neuroprotection]. / Ampakiny ­ novyi podkhod k neiroprotektsii.

Increased interest in glutamatergic neurotransmission emerged in the second half of the twentieth century. Later, the role of glutamate neurotransmission in learning and memory processes became clear. AMPA receptors (AMPR) and NMDA receptors (NMDAR) turned out to be important links in the mechanism of long-term potentiation (LTP) involved in memory processes, which was expressed in an increase in the excitatory postsynaptic potential in response to repeated stimuli. The data obtained in recent decades indicate that AMPR is the main regulators of synaptic plasticity, learning and memory. In clinical terms, the greatest interest is not the formation of memory traces in various parts of the brain, but its restoration in various pathological processes, including reactivation of connections between neurons activated by learning in various areas of the brain. AMPAR synaptic plasticity disorder has been detected in several neurodegenerative diseases accompanied by cognitive disorders. Ampakines, a heterogeneous class of numerous small molecules that bind to the allosteric site on the AMPAR receptor, which slows down the kinetics of AMPAR deactivation, enhances excitatory synaptic current and enhances LTP, have become increasingly attracting the attention of researchers.

[Dynamics of recovery of cognitive deficit in patients in the early recovery period of ischemic stroke]. / Dinamika vosstanovleniya kognitivnogo defitsita u patsientov v rannem vosstanovitel'nom periode ishemicheskogo insul'ta.

OBJECTIVE: Study of the effectiveness of the use of the drug Ampasse in the process of complex rehabilitation in patients in the early recovery period of ischemic stroke at the second (stationary) stage. MATERIAL AND METHODS: The study included 60 patients, 28 women and 32 men, aged 43 to 76 years (mean - 58.4±9.1 years), in the recovery period after suffering a stroke in the period from 1 to 12 months (on average - 4.7±3.5 months). All patients received complex rehabilitation, patients of the 1st group received additional intravenous injections of the drug Ampasse 25 mg (5.0 ml), 15 injections. Patients of the 2nd group (n 0) did not receive Ampasse. To assess cognitive functions, the following tests were used: the Montreal Cognitive Assessment Scale (MoCA), Stroop's test, subtest 9 of the Wechsler test, Koos cubes (CC), the severity of anxiety and depression was assessed, and motor recovery was assessed by the hand motor activity test (ARAT). The assessment was carried out before the start of treatment and on the 21st day. RESULTS: There was a statistically significant increase in the score on the MoCA scale, in patients of the 1st group by an average of 2 points, in the 2nd group there was no significant dynamics, a statistically significant difference was found in the proportion of patients who had an increase in the MoCA index after the course of treatment in the 1st group. compared with the 2nd (χ2 - 22.528, p<0.001). Decreased the level of rigidity according to the Stroop test in patients of the 1st group compared with the 2nd (χ2 - 8.297, p=0.004). The number of patients who showed positive dynamics in the Koos cubes test in the 1st group was statistically significantly higher (χ2- 4.344, p=0.038). A statistically significant decrease in the level of depression was revealed in patients of the 1st group. The number of patients with improved motor function of the hand was greater in the 1st group of MG (χ2 - 4.286, p<0.039). CONCLUSION: In patients in complex therapy receiving intravenous administration of the drug Ampasse at a dose of 25 mg (5.0 ml) 15 administrations, a statistically significant improvement in cognitive functions was revealed according to MoCA tests, Stroop test, Koos Cubes, when compared with the comparison group. The use of Ampasse increased the effectiveness of cognitive and motor rehabilitation in patients with post-stroke disorders.

[Cerebral circulation disorders in COVID-19]. / Narusheniya mozgovogo krovoobrashcheniya pri COVID-19.

Present two clinical cases of cerebral circulation disorders in COVID-19. Cerebrovascular disorders in patients have been associated with COVID-19. Despite the similarity of symptoms, the pathogenesis of neurological damage in these patients was different due to damage to the arterial system in the first case and the venous system in the second case. It is concluded that during the COVID-19 pandemic, doctors need to be alert to all patients with new-onset neurological symptoms.

[Independence of ischemic stroke patients after alteplase systemic thrombolytic therapy]. / Pokazateli nezavisimosti u bol'nykh s ishemicheskim insul'tom posle sistemnoi tromboliticheskoi terapii preparatami alteplazy.

OBJECTIVE: Was to determine the proportion of patients with ischemic stroke (IS) who achieved functional independence by the end of the hospitalization, among those who underwent systemic thrombolytic therapy (STT) with two different alteplase preparations (Actilyse and Revelisa). MATERIAL AND METHODS: An open, prospective observational non-interventional cohort study of the results of STT in patients with IS was carried out. The study included 195 patients, 123 men and 72 women aged 32 to 86 years, who received STT in the acute period of IS. Of these, 120 patients received Actilyse, 75 - Revelisa. RESULTS: Patients who achieved independence in movement (0-2 points on the modified Rankin scale (MRS)) among those who received STT with Actilyse and Revelisa, the value of the χ2 criterion was 0.014 (p=0.905), which showed that there was no statistical significance of the difference in the frequency of recovery of functional disorders on the MRS scale, in patients receiving STT with two different alteplase preparations. CONCLUSION: After STT with alteplase preparations, patients with IS experienced a significant improvement, which was expressed in a statistically significant decrease in the severity of neurological deficit and a decrease in the degree of disability. When comparing the results of STT with two different alteplase preparations, no statistically significant differences were found in terms of mortality, survival, and independence during the acute period of IS.

[Remissions and relapses in epilepsy]. / Remissii i retsidivy u patsientov s epilepsiei.

OBJECTIVE: To study a cohort of adult patients with various forms of epilepsy to determine various clinical patterns of remission with subsequent relapse of seizures. MATERIALS AND METHODS: The study included 1384 patients. Two hundred and forty-nine patients with one or more remissions in the history resulted in relapse. Patients were stratified into 6 groups by clinical features of the disease. For each group, a long-term outcome of the disease was evaluated. RESULTS AND CONCLUSIONS: Return to a basic therapy after a relapse due to dose reduction or AED discontinuation does not guarantee the remission. One or more «honeymoons¼ in medical history are predictors of the low probability of achieving remission. Patients with focal epilepsies often have one or more long-term remissions that may not correspond with AED treatment. In these patients, the relapse often happen in the second decade of life with the following development of intractable epilepsy.

[Clinical polymorphism of demyelinating diseases associated with MOG-antibodies]. / Klinicheskii polimorfizm demieliniziruyushchikh zabolevanii, assotsiirovannykh s antitelami k MOG.

The authors present three clinical cases of demyelinating diseases associated with MOG-antibodies. In the first case of a young adult male patient, the disease first manifested with stem encephalitis and high titer of plasma MOG-antibodies. In the second case of a male adolescent, the disease proceeded as neuromyelitis optica, aquaporin-4 antibody titer was normal. In the third case, the female child the disease presented with unilateral multifocal encephalitis and focal epileptic seizures. The article emphasizes the relationship of MOG-antibody titers with the severity and prognosis of the disease.

[Pathomorphosis of idiopathic generalized epilepsies]. / Patomorfoz idiopaticheskikh generalizovannykh épilepsii.

AIM: To study the pathomorphosis of idiopathic generalized epilepsies (IGE) in a large population of adult patients with long-term follow-up. MATERIAL AND METHODS: The study involved 1480 patients with epilepsy, including 281 patients with IGE. RESULTS: In 26 patients with childhood absence epilepsy (CAE), age-related changes in disease course have been particularly pronounced. None of the patients had frequent typical absences ('pyknolepsy') at the time of the survey. Sixteen of 26 patients had predominantly rare generalized tonic-clonic seizures in combination with occasional absences or myoclonic jerks, while in 2 patients there was the transition from the DAE to juvenile myoclonic epilepsy (JME). The EEG recorded in the 'adult' age showed the level of epileptiform activity in only 12 patients out of 26 (in 2 patients only during nighttime video-EEG-monitoring). EEG changes resembled a 'juvenile' form of epilepsy. At the time of completion of the study, 13 patients were free of seizures and only 5 of 26 patients with DAE did not require treatment with antiepileptic drugs. In patients with Jeavons (n=14) and Tassinari (n=1) syndromes, the disease did not change over time. CONCLUSION: The pathomorphosis of IGE with childhood onset depends on the certain epileptic syndrome.

[Balo's concentric sclerosis]. / Kontsentricheskii skleroz Balo.

Balo's concentric sclerosis (BCS) is a rare demyelinating disease, first described by Hungarian neurologist Josef Balo in 1928. BCS occurs predominantly in young adults, the average age of onset of the disease - 34 years (range from 3 to 62 years). Our case report describes a 27-year woman with acute onset progressive right-side pyramidal weakness, MRI results showed a variant of demyelination as Balo's concentric sclerosis.

[Genetic causes of stroke in young patients]. / Geneticheski determinirovannye prichiny insul'ta u molodykh patsientov.

The paper addresses genetic causes of stroke: MELAS, antiphospholipid syndrome, CADASIL, Fabry disease. The etiology and pathogenesis, symptoms, diagnosis, treatment methods of these diseases are described.

[A retrospective analysis of using zonisamide and retention in monotherapy among patients with epilepsy in routine clinical practice]. / Retrospektivnyi analiz primeneniia i uderzhaniia na monoterapii zonisamidom bol'nykh épilepsiei v usloviiakh povsednevnoi klinicheskoi praktiki.

INTRODUCTION: Zonisamide is one of the first antiepileptic drugs of new generation with a wide spectrum of action. It is successfully used in treatment of epilepsy for 30 years. The study aims at analyzing the results of the multicenter Russian trial on the efficacy and tolerability of zonisamide and retention in monotherapy. MATERIAL AND METHODS: The analysis included 92 patients with focal epilepsy, aged 18-78 years, from 20 epileptology centers in different regions of Russia. The patients were treated with zonisamide in initial and subsequent treatment. RESULTS AND CONCLUSION: The efficacy (the reduction of seizure frequency by >50%) was achieved in 81 (91%) out of 89 patients (95% CI 83.6-95.7%). Fifty-two patients received zonisamide for more than one year. The efficacy was achieved in 50 (96.2%) (95% CI 88.2-99.2%), including 39 (79.6%) patients with remission out of 49 patients with assessment of seizure free periods (95% CI was 66.8-89% for the frequency of remissions). The adverse effects were recorded in 27 (29.3%) patients. Seven (7.6%) patients were withdrawn due to these effects. The maximal duration of observation period was 50 month. Mean time of retention in treatment was 42.4 month (95% CI 38.7-46.2 month). The total frequency of retention in treatment was 82.1% (95% CI 73.1-91%) during the whole observation period.

[Pathomorphosis of idiopathic generalized epilepsy. Juvenile forms]. / Patomorfoz idiopaticheskoi generalizovannoi épilepsii. Iuvenil'nye formy.

AIM: To investigate the pathomorphosis of idiopathic generalized epilepsy (IGE) in the aspect of prognosis. MATERIAL AND METHODS: The study involved 1480 patients with epilepsy including 281 patients with IGE. RESULTS AND CONCLUSION: 'Juvenile' forms were diagnosed in 228 patients. Relapse of seizures in anamnesis occurred in 19% out of 105 patients with juvenile myoclonic epilepsy (JME). Remission was achieved in 59.2% out of 76 patients with long-term follow-up. Out of 35 patients with juvenile absence epilepsy (JAE), relapse of seizures in anamnesis occurred in 25.7%. Out of 24 patients with long-term follow-up, remission was achieved in 70.8%. Out of 88 patients with IGE with generalized tonic clonic-seizures, relapse of seizures in anamnesis occurred in 15%. Out of 60 patients with long-term follow-up, remission was achieved in 86.7%. The main features of epilepsy pathomorphosis in all groups of patients were the decrease of informativeness of routine EEG, reduction in the frequency and duration of seizures, occurrence of seizures in the discontinuation of therapy and/or under the influence of provoking factors.

[Marburg's variant of multiple sclerosis: a case report]. / Rasseiannyi skleroz - variant Marburga (nabliudenie iz praktiki).

Marburg's variant of multiple sclerosis, first described by the Austrian neurologist Otto Marburg in 1906, is a rare demyelinating disease that usually leads to severe disability or death within weeks to months. Our case report describes a young 24-year old woman with Marburg's variant of multiple sclerosis who became totally disabled over a period of some months. Clinical features and MRI-characteristics of two demyelinating diseases: acute disseminated encephalomyelitis (ADEM) and Marburg's variant of multiple sclerosis are discussed. The use of intense immunosuppression in the case described is considered.

[Niemann-Pick type C disease in a child]. / Bolezn' Nimanna-Pika tipa S u rebenka.

The authors consider a clinical case of Niemann-Pick disease type C, an orphan hereditary autosomal recessive neurodegenerative disease belonging to the group of lysosomal storage disease, in an 11-year female patient with the late infantile form of the disease. The combination of psychomotor retardation, polymorphic neurological symptoms and physical changes in the form of isolated splenomegaly suggested the diagnosis of Niemann-Pick type C disease. DNA testing was carried out using direct automated sequencing. The patient was treated with miglustat.

[Leber's hereditary optic neuropathy and epilepsy: a case report]. / Nasledstvennaya atrofiya zritel'nykh nervov Lebera i epilepsiya: opisanie klinicheskogo nablyudeniya.

A clinical case of a young man with rapid bilateral visual impairment developed 2 months after the onset of generalized convulsive seizures is presented. The replacement of valproate to levetiracetam led to the remission of seizures, but the prognosis for vision remains poor, particularly given the pessimistic results of optical coherence tomography (OCT).

[The neurological manifestations of Fabry disease. A review]. / Nevrologicheskie proyavleniya pri bolezni Fabri.

Fabry disease (Anderson-Fabry disease) is an X-linked recessive lysosomal storage disorder resulting from deficient activity of lysosomal hydrolase, alpha-galactosidase A (alpha-Gal A), which leads to progressive accumulation of globotriaosylceramide (Gb3) in various cells, predominantly endothelial and vascular smooth muscle cells, with clinical manifestations affecting major organs including the central nervous system. Manifestations of Fabry disease include progressive renal and cardiac insufficiency, neuropathic pain, stroke and cerebral disease, skin and gastrointestinal symptoms. Clinical onset usually occurs in childhood, but many severe patients are diagnosed in adulthood. Females may be severely affected as males and both may die prematurely due to stroke, heart disease and renal failure. Early recognition of symptoms, enzyme activity levels, concentration of Gb3 levels in the blood, urine and skin biopsies, as well as genetic testing (GLA gene) enable establishment of early diagnosis and therapeutic intervention with enzyme replacement therapy. Enzyme replacement therapy can stabilize or reduce the progression of the disease. Early therapy may prevent complications of the disease.

[Antiepileptic drugs and seizure aggravation]. / Protivoépilepticheskie preparaty i aggravatsiia pristupov.

Objective. To analyze epileptic seizure aggravation associated with antiepileptic drugs (AED) in adult patients. Material and methods. We examined 1407 patients aged 18-89 years. Different patterns of seizure aggravation were identified in 103 patients. Results. Aggravated seizures due the generic substitution were found in 32 patients. First was topiramate (TPM) (n=12), followed by valproates (VPA) (n=8), carbamazepine (CBZ) (n=5), lamotrigine (LTG) (n=1) and levetiracetam (LEV) (n=1). Patients with idiopathic generalized epilepsies (IGE) suffered aggravation with CBZ in 17 cases, VPA - in 6, TPM - in 6, LTG - in 1 and LEV - in 1. CBZ aggravated absences in patients with juvenile absence epilepsy (JAE) were found in 5 cases of 17 (29.4%), with childhood absence epilepsy (CAE) - in 1 of 24 (4.2%), absences and myoclonic jerks in juvenile myoclonic epilepsy (JME) - in 9 of 47 (19.1%), absences with eyelid myoclonus (Jeavons syndrome) - in 2 of 11 (18.2%). Seizure aggravations with different AEDs were observed in 13 patients. This pattern of aggravation was associated with resistant epilepsy and poor prognosis. Seizure aggravation due to increasing the dose of AEDs was found in 10 cases and associated with resistant epilepsy and poor outcome. "True" aggravation was associated with CBZ in 34 patients, including 16 IGE patients, with TPM - in 13, VPA - 5, LTG - 5, LEV - 2 and with clonazepam in 1 patient. Most often seizure aggravation was associated with CBZ in IGE patients (8.3%), and with LTG, TPM and CBZ in patients with other epileptic syndromes (non IGE): 4.9%, 4% and 3.7%, respectively. Conclusions. Different AEDs can cause aggravation. Seizures aggravation after generic substitution was characteristic of TPM most frequently. Seizure aggravation with different AEDs and due to increasing the dose of AEDs was associated with poor outcome. In all cases, seizure aggravation was more frequently caused by LTG (4.7%), CBZ (3.7%) and TPM (3.3%). To reduce the risk of aggravation, slow AEDs titration and keeping a diary of seizures are required.