[Surgical treatment of focal drug-resistant epilepsy associated with temporal lobe encephalocele]. / Khirurgicheskoe lechenie patsientov so strukturnoi fokal'noi farmakorezistentnoi epilepsiei, assotsiirovannoi s visochnymi entsefalotsele.

BACKGROUND: In recent years, temporal lobe encephalocele has become more common in patients with focal drug-resistant epilepsy. Despite available experience, there are still no clear recommendations for choosing the extent of surgery in these patients. OBJECTIVE: To evaluate the effectiveness of diagnosis and surgical treatment of focal drug-resistant epilepsy associated with temporal lobe encephalocele. MATERIAL AND METHODS: The study included 21 patients with focal temporal lobe epilepsy and temporal lobe encephalocele. All patients underwent continuous video-EEG monitoring and MRI of the brain. There were 12 (57.4%) selective encephalocele resections and 9 (42.6%) anterior temporal lobectomies. The median follow-up period was 31 months. RESULTS: The overall effectiveness of surgical treatment with postoperative Engel class I was 76% (16 cases). Selective encephalocele resection was followed by postoperative Engel class I in 10 patients (83%). There were 6 (67%) patients with similar outcomes after temporal lobectomy. Mean volume of resected tissue adjacent to encephalocele was 8.3 cm3. CONCLUSION: Surgery is a highly effective treatment for patients with epileptic seizures following temporal lobe encephalocele. In our sample, favorable postoperative outcomes were achieved in 76% of patients (Engel class I). There were no significant differences in effectiveness between selective resection and temporal lobectomy. Further research is necessary for a clear protocol of surgical treatment of focal drug-resistant epilepsy associated with encephalocele.

[Long-term epilepsy-associated tumors]. / Glioneironal'nye opukholi, assotsiirovannye s epilepsiei.

OBJECTIVE: The goal of this review is to summarize data concerning radiological and histological diagnosis of epileptogenic tumors, as well as to discuss their surgical treatment. MATERIAL AND METHODS: PubMed literature database was searched for relevant articles, we also used our own clinical experience. RESULTS: Benign glioneuronal tumors are the second most common cause of drug resistant epilepsy in adults after hippocampal sclerosis. Exceptionally slow growth (more than 2 years), localization in the brain cortex, presence of differentiated neuronal tissue in tumoral body and long history of epilepsy are the key features of these tumors called LEAT (long-term epilepsy-associated tumors). Management of LEAT requires epileptological as well as neurooncological approach. The epileptogenic zone commonly spreads beyond the tumor borders and simple lesionectomy alone may not be sufficient for achieving seizure freedom. CONCLUSION: LEAT typically exhibit low proliferative activity, however they should be thoroughly differentiated from more aggressive glial tumors; while this task is sometimes quite challenging, it's achievable by means of histological and immunohistochemical examination.

[Pre-surgical Diagnosties in Patients with Intractable epilepsy]. / Predkhirurgicheskaia diagnostika u patsientov s farmakorezistentnoi épilepsiei.

PURPOSE: To conduct a systematic assessment of scientific publications devoted to pre-surgical examination of patients with intactable epilepsy. MATERIAL AND METHODS: We found, using PubMed and available Internet search tools, and analyzed 1.414 articles on pre-surgical diagnostics in patients with intractable epilepsy. RESULTS: Epilepsy is a chronic disorder caused by brain injury, which manifests as repeated epileptic seizures and is accompanied by a variety of personality changes. Mortality risks in the population of patients with uncontrolled intractable epilepsy significantly exceed those in the general population. Early onset of comprehensive treatment prevents pathological personality changes and reduces the risks of mortality. However, complete seizure control is not achieved in 30% of patients, and they develop pharmacoresistance later, which is the reason for considering these patients as candidates for surgical treatment. In the literature, many approaches to pre-surgical examination are described as each clinic has its own concept of pre-surgical diagnostics and its own approaches to surgical management. Based on the conducted analysis, we tried to summarize the received information and describe current ideas about pre-surgical examination of patients with intactable epilepsy. CONCLUSION: On the basis of analyzed literature, we performed a systematic assessment and the evaluated effectiveness of various approaches in the pre-surgical diagnostics of patients with intactable epilepsy.

Ictal and interictal MEG in pediatric patients with tuberous sclerosis and drug resistant epilepsy.

PURPOSE: Drug resistant epilepsy (DRE) is common in patients with tuberous sclerosis (TS). Interictal MEG has been shown as a valuable instrument in the presurgical workup. The goal of our study was to evaluate the role of ictal MEG in epileptogenic tuber selection, especially in patients with multiple irritative zones. METHODS: The clinical and MEG data of 23 patients with TS and DRE from two medical/research centers were reviewed. Seven pediatric patients, who had seizures during MEG recording and underwent resection or disconnection surgery, were included into the study. Cortical sources of ictal and interictal epileptiform MEG discharges were compared with epileptogenic zone location in six patients with favorable surgery outcome. RESULTS: In patients who improved substantially after surgery all resected and several other tubers demonstrated epileptiform activity on interictal MEG. Ictal MEG provided crucial information about lobar location of the seizure onset zone (SOZ) in two cases, and in the other four it confirmed the SOZ location derived from the interictal data. In one case, ictal MEG findings were unreliable. In one patient, who did not benefit from surgical treatment, the resected tubers did not overlap with interictal and ictal MEG sources. CONCLUSION: The combination of interictal and ictal MEG is a valuable tool for identification of the epileptogenic tuber/tubers in presurgical work-up in patients with TS.

[Surgery for intractable epilepsy in a patient with encephalocele of the temporal lobe: a case report]. / Khirurgicheskoe lechenie farmakorezistentnoi épilepsii u patsientki s éntsefalotsele visochnoi doli (klinicheskoe nabliudenie i obzor literatury).

We describe a case of surgical treatment of intractable temporal epilepsy in a female patient with congenital middle cranial fossa encephalocele. We present clinical-anamnestic and neuroimaging data as well as the microscopic and macroscopic pictures of encephalocele. We analyze outcomes of surgery for this pathology, which have been reported in the literature. To date, there have been a few articles on this subject in the domestic literature. The development of neuroimaging techniques and a growing number of verified encephalocele cases promote the widespread use of surgery for treatment of intractable epilepsy. Congenital encephalocele should be considered in the differential diagnosis of intractable temporal epilepsy, and, if verified, surgical treatment is the method of choice in most cases.

[Hippocampal sclerosis: pathogenesis, clinical features, diagnosis, and treatment]. / Skleroz gippokampa: patogenez, klinika, diagnostika, lechenie.

Hippocampal sclerosis, also known as Ammon horn sclerosis or mesial temporal sclerosis, is usually associated with intractable epilepsy and characterized by specific patterns of neuronal loss and gliosis in the medial temporal lobe structures. Hippocampal sclerosis manifests clinically as epilepsy, often intractable epilepsy; in most cases, this condition is surgically treatable. As the most common histological diagnosis in adult patients subjected to epilepsy surgery, hippocampal sclerosis is characterized by complex pathogenesis and requires a multidisciplinary approach to its diagnosis and treatment. This article reviews the pathologic features, natural history, pathogenesis, and electroclinical and MRI signs of hippocampal sclerosis.

[Supracerebellar transtentorial approach to tumors of the posterior portions of the medial temporal region].

OBJECTIVE: Despite the advances in microsurgery, the choice of the most adequate approach to the posterior part of the medial temporal region (MTR) remains a very controversial issue. The supracerebellar transtentorial approach (STA) is considered as the most preferable one, since it provides the optimal balance between retraction, incision, and resection of the brain tissue. Here, we present our consecutive series of 20 patients who underwent STA surgery. MATERIAL AND METHODS: Twenty patients with glial tumors affecting the posterior MTR underwent STA surgery between 2006 and 2014. The mean age of the patients was 20 years. Benign tumors were predominant (18 out of 20 cases). RESULTS: Resection of the posterior and middle MTRs was conducted in 16 cases. The anterior MTR was accessed through STA in 1 patient only; in 2 patients, STA was combined with the infraoccipital approach. Cerebellar edema occurred in 4 patients, with hemiparesis persisting in one of the cases for 1 year after surgery. Of 8 patients with drug resistant epilepsy, the Engel class 1 or 2 outcome was achieved in 6 cases within 1 year after surgery. CONCLUSION: STA provides an excellent surgical route to the posterior and middle MTR portions; however, the anterior MTR portions cannot be reached safely. The operative risks of STA increase as the surgeon proceeds with resection of the anterior MTR portions. Anterior MTR structures can be removed using a combination of the supracerebellar and infraoccipital transtentorial approaches or two-stage resection.

[Possibilities of using intravenous forms of antiepileptic drugs in epileptic seizures]. / Vozmozhnosti primeneniia vnutrivennykh form protivoépilepticheskikh preparatov pri épilepticheskikh pristupakh.

The literature data on using intravenous forms of AEDs that expand treatment possibilities for patients with epilepsy are presented. AEDs can be used in different situations, when patients are not able to take AEDs in per os. These situations can include seizure emergencies (recurrent seizures, clusters, status epilepticus), caused by disease decompensation; acute symptomatic seizures; perioperative preparation in the case of surgery; dysphagia; gastrointestinal problems; psychiatric disorders, and others. This article is based on a review of International and Russian expert consensus practice guidelines for management of clinical situations mentioned above. The authors present data on available Russian parenteral forms of drugs used today (valproats, levetiracetam, lacosamide, diazepam, midazolam, propofol, thiopental potassium). It has been concluded that an ideal intravenous AED should be highly effective, acts fast, has no severe adverse effects, has low drug-drug interactions, does not require frequent plasma concentration monitoring, and should be easily switched to a per os form without any additional titration. The importance of separate treatment approaches on different stages of medical help is emphasized. The choice of drug used can be influenced by a particular clinical situation, indications/contraindications, the recommended infusion speed, side effects, possible drug-drug interactions with other AEDs and other drugs used for the treatment of concomitant diseases, availability of a particular drug at the moment and the level of a physician's knowledge of usage of intravenous forms of AEDs.

[Magnetoencephalography in the presurgical evaluation of patients with drug-resistant epilepsy].

Magnetoencephalography (MEG) in combination with structural MRI (magnetic source imaging, MSI) plays an increasingly important role as one of the tools for presurgical evaluation of medically intractable focal epilepsy. The aim of the study was to compare the MSI and commonly used video EEG monitoring method (vEEG) in their sensitivity to interictal epileptic discharges (IED) in 22 patients with drug resistant epilepsy. Furthermore, the detection and localization results obtained by both methods were verified using the data of electrocorticography (ECoG) and postsurgical outcome in 13 patients who underwent invasive EEG monitoring and surgery. The results showed that MSI was superior to vEEC in terms of sensitivity to IED with difference in sensitivity of 22%. The data also suggested that MSI superiority to vEEG in detecting epileptic discharges might, at least partly, arise from better MEG responsiveness to epileptic events coming from the medial, opercular and basal aspects of cortical lobes. MSI localization estimates were in the same cortical lobe and at the same lobar aspects as the epileptic foci detected by ECoG in all patients. Thus, magnetic source imaging can provide critical localization information that is not available when other noninvasive methods, such as vEEG and MRI, are used.

[Topical features of speech disorders in children with focal cortical dysplasia and symptomatic epilepsy].

We studied speech function in 11 patients with focal cortical dysplasia (FCD) of different localization. The character of speech disorders was strongly correlated with the time of seizure onset and their frequency, while the localization of dysplasia did not play a significant role. The speech remained intact during seizures in the school age and during the relatively rare (weekly or monthly) seizures. The complete disappearance of speech after the period of normal development occurred in the daily multiple secondary-generalized seizures and resembled the previously described Landau-Kleffner syndrome ("aphasia-epilepsy"). Based on the literature and own data, the authors suggest that such global speech disorders can not be considered as aphasia, i.e., the disorder due to the focal lesions of speech brain areas. The authors did not reject the possibility of the development of aphasia in epileptic patients but only in those cases when the epileptic activity confined to mostly speech areas. Global speech disorders (up to the complete disappearance of impressive and expressive speech) at any localization of epileptic focus developed during the negative rearrangement of the speech functional system as a whole. These speech disorders are often accompanied by the acquired epileptic frontal syndrome. That is, in the first place are the most phylo- and ontogenetically young functional systems, namely, speech and regulatory functions, the damage of which emerges as a frontal syndrome. At the same time, the old consolidated pre-speech connections remain. Presumably, in this view, in cases of global speech disorders and speech disappearance, it is more correctly to substitute the terms "aphasia-epilepsy" and "total aphasia" for the familiar term "regression of speech development".

[Hemostatic disorders caused by chronic use of valproic acid in neurosurgical patients. Thromboelastographic monitoring].

The paper gives the results of analyzing the hemostatic system in 26 patients with various neurosurgical diseases on the basis of routine laboratory biochemical tests and thromboelastographic indicators. In all the patients, the pattern of the disease contained an epilepsy syndrome that required mono- or combination therapy with valproic acid. Laboratory indicators of clinical hypocoagulation were found to develop during the use of valproic acid, and its monotherapy in particular. Hemorrhagic complications were also analyzed in not only the immediate, but also late postoperative period (for as long as 6 months after surgery). Two cases of severe late complications, such as formation of chronic subdural hematomas requiring surgical intervention, were diagnosed in the valproate monotherapy group. A tactic using a thromboelastographic technique is proposed to prepare these patients for further neurosurgical intervention.

[Comatose state caused by seizure-free status epilepticus in postoperative period].

We present a rare clinical observation of progressive development of neurological symptoms up to comatose state in a child with extra-intraventricular craniopharyngioma in delayed postoperative period due to seizure-free status epilepticus which was diagnosed only by EEG-monitoring. Concerning this case report, we discuss genesis of seizure-free status epilepticus and its possible role in development of severe condition in patients with different kinds of neurosurgical pathology in postoperative period.

[Successful treatment of moya-moya disease using encephalo-duro-arteriosynangiosis].

Aim of the study was to demonstrate the possibility of effective surgical revascularization of the brain in children with moya-moya disease. 3 year old girl with moya-moya disease underwent encephalo-duro-arteriosynangiosis. At 14 months follow-up signs and symptoms of cerebral ischemia totally resolved. Angiography confirmed brain supply from external carotid artery via the newly formed anastomotic network. Authors emphasize the necessity to seek moyamoya disease in children with symptoms of brain ischemia to allow timely and effective surgical treatment.

[Noninvasive video-EEG-monitoring in the diagnosis of focal epilepsy in children].

UNLABELLED: Aims of the study include diagnosis of focal epilepsy, determination of localization of interictal and ictal epileptiform activity. 785 patients (age range -- 1 month-21 year) with admission diagnosis "epilepsy" were analyzed. Video-EEG-monitoring using a 21-chanel EEG system with duration 9-96 hours (mean -- 10 hours) was applied. The diagnosis of epilepsy was confirmed in 634 patients (80.8%) while in 151 patient (19.2%) non-epileptic paroxysms were diagnosed. Generalized epilepsy (GE) was diagnoses in 47 cases (7.4%), focal (FE) -- in 587 (92.6%) cases. According to localization, distribution was the following: frontal -- 48.2%, temporal -- 30.8%, occipital -- 10%, parietal -- 5.5%, multifocal -- 5.5%. 45% of patients with FE younger than 3 years had marked generalized seizures known as infantile spasms. Among 587 patients with FE discrepancy of localization of interictal focus and zone of seizure onset was found in 41 cases (7%), mainly in younger patients. In 14 cases (2.4%) we observed more than one zone of seizure onset: 2 -- in 13, 3 -- in 1, both in frontal and left temporal areas (all specified patients are from the younger group). CONCLUSIONS: 1) every fifth patients with primarily diagnosed epilepsy does not suffer from this disease; 2) 92.6% of patients in pediatric epileptologic clinic have FE, about half of them have frontal localization of the focus; 3) presence of generalized types of seizures mimicking GE is an important feature of FE in younger patients; 4) In some patients with FE, especially in younger ones, discrepancy of localization of interictal epileptiform activity and zone of seizure onset is found, we also observed that more than one zone was producing attacks. The specified features are necessary for consideration during preoperative assessment.

[The electrophysiological phenomenon of prolonged epileptiform peak-wave activity to the slow sleep phase].

Thirty-six cases of epilepsy with the phenomenon of prolonged epileptiform peak-wave activity in slow sleep (PESS) are presented. The peculiarities of this phenomenon are singled out. Basing on the obtained results, the authors describe a modified variant of PESS pattern which is characterized by the presence of the stable regional amplitude prevalence of peak-wave discharges and/or stable regional epileptiform activity in the "classic" PESS pattern.

[Epilepsy with electrical status epilepticus during slow sleep: diagnostic criteria and approaches to therapy].

Fourteen patients, aged from 5 to 14 years, with syndrome of electrical status epilepticus during slow sleep (ESESS) have been studied. The absence of epileptic attacks was observed in 21.5% of patients and diagnosis was established by a combination of continuous diffuse epileptiform activity with marked cognitive disturbances. In 78.5% patients, epileptic attacks presented as follows: pharyngeal and oral, hemicephalgia (100% patients with attacks), hemiclonic (18%), atypical absences (27%), negative myoclonus (18%), automotor (18%), focal adverse with vomiting (18%), secondary generalized (36%). Drugs of choice were valproate (depakine) and topamax in patients with attacks; suxilep and frisium in the absence of attacks and in case of continuous diffuse epileptiform activity on the sleep EEG. Two variants have been singled out by the character of ESESS syndrome. The first one, a "symptom variant", was featured by mostly hemiclonic, secondary generalized and automotor epileptic attacks, presence of continuous regional or lateral, less frequent diffuse epileptiform activity, detected by the local structural lesions in MRI, marked cognitive disturbances persisting after stopping of the attacks. The second ("idiopathic") variant is characterized by normal development of children before attack manifestation, appearance of mostly "rolandic" attacks, atypical absences and negative myoclonus, presence of exclusively continuous diffuse epileptiform activity in the phase of slow sleep, standard MRI results (moderate cortical subatrophy in single cases), cessation or substantial decrease of cognitive disturbances after attack stopping. It is emphasized that prognosis of ESESS syndrome should be considered separately with regard to attacks and cognitive disturbances. A prognosis for attacks is always favorable. Cognitive disturbances despite the therapy can persist for a long time.