[Pseudotumorous form of primary central nervous system vasculitis]. / Psevdotumoroznaya forma pervichnogo vaskulita tsentral'noi nervnoi sistemy.

A 23-year-old female patient with primary vasculitis of the central nervous system simulating a brain tumor is described. The clinical picture was represented by migraine-like headaches, ataxia, transient numbness of the right leg, the lips, double vision, a slight decrease of cognitive functions. MRI of the brain revealed a tumor-like focus in the cerebellum, intensively accumulating contrast, containing micro-hemorrhages (SWI mode). Small single ischemic foci in the brain hemispheres and brain stem were also found. MR angiography (3T) did not found any pathology. Examination of the cerebrospinal fluid revealed a small cytosis (mainly T-lymphocytes) and a slight increase in protein. The results of the analysis of cerebrospinal fluid for syphilis, tuberculosis and the herpetic group of viruses were negative, type 1 oligoclonal synthesis was found. Blood tests for toxoplasmosis, antibodies to aquaporin, anti-neutrophil antibodies, markers of systemic inflammation were within normal limits. Different diagnoses were assumed: demyelinating disease, encephalitis, multiple encephalomyelitis, lymphoma. The diagnosis was established only by a brain biopsy - lymphocytic vasculitis was revealed. According to the immunohistochemical study, T-helpers predominated in the infiltrates. After pulse therapy with Metylprednisolon (1000 mg intravenously drip №. 5), the patient's condition almost returned to normal. It was recommended to take prednisolone per os (starting dose 60 mg) for 7 months.

[Reversible cerebral vasoconstriction syndrome and ischemic stroke]. / Sindrom obratimogo tserebral'nogo vazospazma i ishemicheskii insul't.

The article describes a 45-year-old female patient with recurrent transient ischemic attacks and ischemic stroke due to spontaneous spasm of the middle cerebral artery, the anterior cerebral artery and distal part of the internal cerebral artery on the left, verified by MR angiography and CT angiography. It is assumed that the spasm caused damage to the vascular wall, an increase in its permeability, the development of edema, inflammation and subsequent fibrosis, therefore a complete restoration of the arterial lumen did not occur.

Individual Variability of Broca's Area of the Brain in Women.

In this article, we studied individual features of the macroscopic structure of Broca's area of the brains in 9 women (18 hemispheres) aged from 20 to 30 years, without any mental or neurological disorders. By using MRI, the structures of the sulci and gyri of the pars triangularis and pars opercularis of Broca's area were studied: the anterior and ascending rami of the lateral sulcus, the radial, diagonal, precentral, inferior frontal, and lateral sulci. We also studied the relationship between the pars triangularis and pars opercularis as well as their relationships with neighboring cortical structures. We measured the volume of the pars triangularis and pars opercularis and the thickness of their cortex. Significant individual variability in the location and relationships between the anterior ramus of the lateral sulcus and the ascending ramus of the lateral sulcus, as well as structural features of the pars triangularis and pars opercularis of Broca's area were demonstrated.

[Gender morphology of Broca's motor speech area]. / Gendernaya morfologiya rechedvigatel'noi zony Broka.

OBJECTIVE: To study the general patterns and differences in the macroscopic structure of Broca's motor speech area in the left and right hemispheres of male and female brains. MATERIAL AND METHODS: The study was conducted on MRI images of the brains of 9 men and 9 women (36 hemispheres in total). All the people were between the ages of 20 and 30, without any mental or neurological disorders. The localization and structure of the main sulci and gyri of Broca's area, namely the pars triangularis and pars opercularis, were studied. In addition, the topography of the main sulci in Broca's motor speech area, namely their shape, length, and relative position to the other sulci, was analyzed. RESULTS: The features of the localization of the sulci in Broca's area, the differences in the number of additional sulci in the pars triangularis and pars opercularis of male and female brains, as well as the degree of asymmetry of Broca's area in the left and right hemispheres of the brains of men and women were established.In modern neuroscience a new scientific direction of genderology, which studies the behavior and cognitive functions of males and females, is rapidly developing. CONCLUSION: Broca's motor speech area of the brain of men and women differs in macroscopic structure.

[C9orf72-associated frontotemporal dementia in the Russian population]. / C9orf72-assotsiirovannaya forma lobno-visochnoi dementsii v rossiiskoi populyatsii.

OBJECTIVE: To evaluate the frequency of C9orf72-associated frontotemporal dementia (FTD) in the Russian population and to study clinical features of GGGGCC-repeat expansion carriers. MATERIAL AND METHODS: Twenty-eight patients with FTD are included in the study: 15 with a behavioral variant of FTD (bvFTD) and 13 with a agrammatic/non-fluent variant of primary progressive aphasia (avPPA). The mean age was 62 years (34-80), the mean disease duration was 4 years (1-10). The positive family history was noted in 46% of cases. DNA diagnosis was performed using repeat-primed polymerase chain reaction. RESULTS: The frequency of the C9orf72 repeat expansion in patients with FTD was 14%, in patients with bvFTD 20%, in patients with avPPA 8%. The mean age of disease onset in the expansion carriers was 63 (55-75) years. The frequency of the C9orf72 repeats expansion in familial FTD cases was 31%, in sporadic cases 7%. bvFTD with parkinsonian syndrome was noted in two out of four cases, bvFTD with amyotrophic lateral sclerosis (ALS) was shown in one case, avPPA with ALS was shown in one case. One female patient with bvFTD with parkinsonian syndrome presented with cognitive fluctuations that required a differential diagnosis with Lewy body disease. CONCLUSION: This is the first study of the genetic structure of FTD in the Russian population. The prevalence and clinical characteristics of C9orf72-associated FTD were defined, in particular, the spectrum of motor symptoms was shown along with behavioral and aphasic disturbances. DNA diagnosis plays an important role in confirming the diagnosis and selection of patients for potential disease-modifying treatment.

[Intracerebral hemorrhage in the late period of internal carotid artery dissection]. / Vnutrimozgovye krovoizliianiia v pozdnem periode dissektsii vnutrennei sonnoi arterii.

Cervical artery dissection is the common cause of ischemic stroke in young and middle-age patients. According to our previous studies, dissection is related to arterial wall dysplastic changes, which in their turn are due to mitochondrial cytopathy. The authors describe three male patients who at the age of 53, 25 and 35 years underwent internal artery (ICA) dissection with occlusion of its lumen and subsequent recanalization in one of them. In 3.5 months, 13.5 years and 3 years respectively, patients developed intracerebral hemorrhage (IСH), which was not related to arterial hypertension, cerebral arterial aneurysms and anticoagulants. IСH were located on the side of ICA occluded after dissection (2 patients) or bilaterally in the territory of patent ICA (1 patient). Multivoxel 1H-MR spectroscopy performed in one patient on 40 and 48 days after ICH revealed a high lactate peak in the externally unchanged hemispheric white matter. It is assumed that mitochondrial cytopathy in patients with dissection may involve large as well as small intracerebral arteries (mitochondrial microangiopathy), which could be the cause of ICH.

[Clinical characteristics and diagnosis of disseminated encephalomyelitis in adults]. / Klinicheskie osobennosti i diagnostika ostrogo rasseiannogo éntsefalomielita u vzroslykh.

AIM: To analyze clinical, neuroimaging and laboratory characteristics of acute disseminated encephalomyelitis (ODEM) in adults with an analysis of the frequency of diagnostic errors at the initial examination stage. MATERIAL AND METHODS: The study included 23 patients hospitalized with a diagnosis of ODEM. The analysis of clinical characteristics, MRI results and cerebrospinal fluid (CSF) was performed. The diagnosis of the disease in each case was specified after a full examination and dynamic observation. RESULTS: The diagnosis of ODEM was confirmed only in 16 cases. In 6 cases, the final diagnosis of multiple sclerosis (MS) was made, and one case of Susak syndrome, primary CNS lymphoma and levamisole-associated multifocal inflammatory leukoencephalopathy was also detected. It has been shown that ODEM in adults is characterized by the acute development of multifocal brain lesions in combination with clinical manifestations of encephalopathy, which in half of cases is preceded by an infectious disease. There were no specific clinical, neuroimaging and laboratory features, which could allow differentiation of ODEM from onset of MS. CONCLUSION: To make a correct diagnosis in the first episode of acute multifocal brain lesion, a dynamic observation, including repeated MRI, is needed. The development of specific biomarkers may be of great importance for the early differential diagnosis of demyelinating diseases.

[Brain MRI-findings in Ph - negative myeloproliferative disorders].

Myeloproliferative disorders (MPD) are accompanied by a high proportion of thrombotic complications, which may lead to cerebrovascular disease (CVD). AIM: To describe MRI-findings in patients with Ph - negative MPD and evaluate any cerebrovascular disease. MATERIALS AND METHODS: We included 104 patients with Ph - negative MPD (age varied between 20 and 58) with clinical correlates of cerebrovascular pathology. RESULTS: Brain MRI showed post - stroke lesions in 20% of patients (7 hemispheric infarcts due to thrombotic occlusion of one of the large cerebral arteries, 14 - cortical infarcts). 37 patients (36%) had vascular cerebral lesions. Cerebral venous sinus thrombosis occurred in 5 patients - in 7% (n=3) of patients with polycythemia vera and 5% (n=2) - in patients with essential thrombocythemia. The incidence of vascular cerebral lesions was associated with higher levels of the following: erythrocyte, platelet count, fibrinogen, and with the decrease in fibrinolytic activity, as well. CONCLUSION: The pioneering results of the study include the description and analysis of brain MRI-findings in patients with Ph - negative MPD. The underlying mechanisms of cerebrovascular pathology in these patients are associated with certain blood alterations (particularly, hemorheology) which present a major risk factor.

[Multifocal central nervous system lymphoma misdiagnosed as acute disseminated encephalomyelitis]. / Pervichno-mnozhestvennaia limfoma tsentral'noi nervnoi sistemy, oshibochno diagnostirovannaia kak ostryi rasseiannyi éntsefalomielit.

Primary central nervous system lymphoma (PCNSL) is a rare aggressive extranodal non-Hodgkin lymphoma. Difficulties in diagnosing PCNSL are associated with the absence of pathognomonic clinical and neuroimaging findings of this disease. The article describes the clinical case of a female patient with autopsy-confirmed multifocal large-cell B-cell CNS lymphoma misdiagnosed as acute disseminated encephalomyelitis (ADEM). Clinical and neuroimaging characteristics of PCNSL and ADEM as well as the role of diagnostic methods in establishing the correct diagnosis are discussed.

Multimodal approach to treatment of neurological complications of chronic brain ischemia.

AIM: The study of Actovegin effectin clinical presentations and hemorheological characteristics in patients with chronic cerebrovascular pathology (CCVP) and mild cognitive impairment. MATERIALS AND METHODS: The study group included 47 patients (25 male and 22 female), aged 61-75 years (mean age 63.8±5.4) with CCVP who were treated with Actovegin. The control group comprised 28 patients matched by gender and age, without associated cerebrovascular pathology. All patients along with thorough neurological examination underwent laboratory analyses (platelet and erythrocyte rheology), neurovisualization studies (functional magnetic resonance imaging of the brain). Depending on the dosage all patients were divided in two groups: Actovegin 1000 mg and 160 mg daily. RESULTS: Overall, with Actovegin treatment in 81% of cases positive dynamics both in subjective symptoms, and somatic status was observed. A favorable effect on cognitive function in patients with CCVP was noted. The dose-dependent drug effect was demonstrated. The effect of Actovegin on blood cell functioning included the formation of smaller (Tf and Ts; p=0.0096 and p=0.016) and less solid (γ dis) erythrocyte aggregates (p=0.0034) both in the study and control group. The increase in erythrocyte deformability during therapy was significantly associated with cognitive improvement (via MoCA test, r=0.28). CONCLUSION: Complex (including neuropsychological and neurovisualizational) examination may not only help determine the cognitive status in patients with CCVP, but also assess the efficacy of neurometabolic therapy. New facts of Actovegin's influence on erythrocyte aggregation and deformability have been identified, which may enhance micro- and macrocirculation. The acquired data may prove the wide spectrum of Actovegin's pharmacological effect, which allows to use it in all forms of cerebrovascular pathology.

[Differential diagnosis of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy]. / Differentsial'naia diagnostika tserebral'noi autosomno-dominantnoi arteriopatii s podkorkovymi infarktami i leikoéntsefalopatiei.

Cerebral autosomal dominant arteriopathy with subcortical infarctions and leucoencephalopathy (CADASIL) is an inherited CNS disease, which is caused by mutations in the NOTCH3 gene. Selective disorders of small vessels underlie the disease pathogenesis. Clinically CADASIL is characterized by headaches, multiple stroke-like disorders (in most cases transient ischemic attacks and lacunar strokes), and different focal neurological symptoms and dementia. There are specific MRI signs of the disease: multiple lacunar infarctions located in the basal ganglia, brain steam and cerebellum, focal lesions of temporal poles, capsula externa, periventricular and subcortical areas; diffuse white matter changes and leukoaraiosis can be observed as well. The differential diagnosis of CADASIL is made with many diseases, which are manifested by multiple brain matter lesions, including demyelinating disorders. It should be taken into account that CADASIL is characterized by headaches as one of the initial symptoms, multiple lacunar and diffuse brain matter lesions based on MRI data with an absence of atherosclerosis and arterial hypertension. Family history and autosomal dominant mode of inheritance is also typical of CADASIL. Detection of the NOTCH3 gene mutation is necessary for the definite diagnosis of CADASIL.

[Area postrema lesion as a cause of intractable nausea, vomiting and hiccups in neuromyelitis optica spectrum disorders]. / Porazhenie area postrema kak prichina neukrotimoi ikoty, toshnoty i rvoty pri zabolevaniiakh optikomielitnogo spektra (nabliudenie iz praktiki).

Neuromyelitis optica (Devic's disease) is a chronic autoimmune disease associated with the production of anti-bodies to aquaporin-4 (AQP4). Area postrema lesions is the third, after optic neuritis and myelitis, syndrome of opticomyelitis-related disorders. Clinical symptoms of this disorder include intractable nausea, vomiting and hiccups. In many cases, area postrema syndrome manifests as the first clinical symptom of a neuromyelitis optica spectrum disorder that hampers the diagnosis. The authors present a case report of a female patient with area postrema lesions developed several months before the first disabling attack of myelitis.

[Prоgnosis of motor function recovery in ischemic stroke using diffusion tensor MRI]. / Otsenka prognoza vosstanovleniya dvigatel'noi funktsii u bol'nykh s ishemicheskim insul'tom s pomoshch'yu diffuzionno-tenzornoi magnitno-rezonansnoi tomografii.

AIM: To identify factors of motor recovery in ischemic stroke (IS) using diffusion tensor imaging (DTI). MATERIAL AND METHODS: Forty-seven patients with IS were studied within 1-2, 7-8, 14-15, 20-21 days after stroke. The fractional anisotropy (FA) values of the pyramidal tract were measured in the posterior limb of the internal capsule, cerebral peduncle and pons. Relative values of FA (rFA) ratio (rFA=FA affected side/FA unaffected side) were assessed as well. RESULTS: rFA≤0.7 in the cerebral peduncule and posterior limb of the internal capsule accurately predicted poor motor outcome in ischemic stroke. CONCLUSION: DTI can evaluate the motor deficit quantitatively and may predict the functional prognosis in patients with IS.

[Changes in the Functional Connectivity of Motor Zones in the Use of Multimodal Exoskeleton Complex "Regent" in the Neurorehabilitation of Post-Stroke Patients].

The article discusses the effect of a course of treatment with the use of multimodal complex exoskeleton (MCE) "Regent" on the reorganization of cortical locomotor zones in 14 patients with post-stroke hemiparesis, mainly atthe chronic stage of the disease. Before the course of treatment, we identified specific areas of activation in the primary sensorimotor and supplementary motor areas and the inferior parietal lobules in both affected and healthy hemispheres by means of functional MRI (fMRI) with the use of special passive sensorimotor paradigms. After the course of treatment with MCE, we observed an improvement of temporal characteristics of walking; it was accompanied by a decrease in the activation zones of inferior parietal lobules, especially in the healthy hemisphere, and by a significant increase in the activation zone of primary sensorimotor and supplementary motor areas. The analysis of the functional connectivity of studied zones before and after the course of treatment with MCE showed significant changes in intra- and interhemispheric interactions.

[Chronic cerebrovascular diseases and metabolic syndrome: approaches to pathogenic therapy of cognitive dysfunction]. / Khronicheskie tserebrovaskuliarnye zabolevaniia i metabolicheskii sindrom: podkhody k patogeneticheskoi terapii kognitivnykh narushenii.

Cognitive decline comprises one of the most important symptoms of chronic cerebral hypoperfusion. It leads not only to social disability of the patients with a subsequent decline in life quality but also to a decrease in adequate control of the course of both vascular pathology and comorbid states. Pathogenetic therapy and prevention of cerebrovascular disease progression, along with general medical measures, should include the drugs normalizing different symptoms of metabolic syndrome (arterial hypertension, diabetes mellitus etc) as well as complex drugs with multiple actions (hemangioma correction, antithrombotic, antioxidant and vasoactive) actions.

[Localization of brain electrical activity sources and hemodynamic activity foci during motor imagery].

Studied are sources of brain activity contributing to EEG patterns which correspond to motor imagery. The accuracy of their classification determines the efficiency of brain-computer interface (BCI) allowing for controlling external technical devices directly by brain signals without involving muscle activity. Sources of brain activity are identified by Independent Component Analysis. Those independent components for which the BCI classification accuracy are at maximum are treated as relevant for motor imagery task. Two of the most relevant sources demonstrate strictly exposed event related desynchronization and synchronization of mu--rhythm during imagery of contra--and ipsilateral hands. These sources are localized by solving inverse EEG problem taking into account individual geometry of brain and its covers provided by anatomical MRI images. The sources are shown to be localized in BA 3A relating to proprioceptive sensitivity of the contralateral hand. Their positions are closed to foci of BOLD activity obtained by fMRI.

[New approaches in the study of neuroplasticity process in patients with central nervous system lesion].

Methods which on one hand can ensure the patient's mobility and on other hand activate afferents inputs are the main in rehabilitation treatment. Recent studies has shown that plasticity is structural base of recovery after central nervous system injury. Reorganization of cortical areas and increase of preserved structures functional effectiveness (intensification afferent input) are an anatomical basis of plasticity. However, sensory correction methods, without accounting of functional condition of patients, can lead to the formation of pathological symptoms: spasticity, hyperreflexia, etc. So the main aim is to study adequate management of the neuroplasticity process. This problem cannot be solving without modern methods of neuroimaging and brain mapping. The new approach for study cortical mechanisms of neuroplasticity, responsible for locomotion, was developed in the present study. This approach is complex use of functional magnetic resonance imaging (fMRI) and navigation transcranial magnetic stimulation (nTMS). It was showed that vast fMRI activation area in the first and the second sensorimotor area emerges with passive sensorimotor paradigm using that imitate backing load during walking. The mechanical stimulator footsteps backing zones "Corvit" uses for create this paradigm, nTMS examination, which used after fMRI, help localize the motor representation of muscles which control locomotion more accurately. We guess that new approach can be used for neuroplasticity process study and assessment of neuroplasticity changes during rehabilitation for restore and correct the walking.

[Specifics of activation of cortex by stimulation of support receptors in healthy subjects and in patients with lesions of CNS].

Results of basic studies in space medicine revealed the mechanism of motor disorders and the importance of support input in regulation of the tone and posture system of the mammals. Progress in functional magnetic resonance tomography (fMRT) enabled in vivo estimation of activity of various parts of the brain during stimulation of support afferent pathways. The goal of our study was to detect specific activation of the cortex during stimulation of support input in healthy subjects and in patients with lesions of CNS. The study included 19 healthy volunteers (mean age of 38 ± 15, 13 years) and 23 patients with cortical-subcortical ischemic stroke (mean age 53 ± 9.07); all subjects underwent fMRT. During scanning of each subject, support areas of the foot soles were stimulated to imitate slow walking using the block design. In healthy volunteers, primary somatosensory cortex, premotor and dorsolateral cortex, and insula were significantly activated (corrected <0.05 at cluster level). In patients with stroke, pattern of activation of the supraspinal systems of locomotion control clearly depended on the stage of the disease. In patients with cortical-subcortical stroke undergoing motility rehabilitation, the sensomotor locomotion module was predominantly activated as the contralateral pattern.

[Two patterns of Ct-brain perfusion and Dc-potentials of the brain evoked cognitive task in discirculatory encephalopathy patients].

In patients with discirculatory encephalopathy the influence of verbal fluency test on the characteristics of cerebral perfusion, DC-potentials of the brain, as well as on blood pressure and heart rate was investigated. Two patterns of responses to the verbal fluency test were observed. The first one is the process of generalized activation, manifested by the reduction of the TTP (time to peak) parameters of brain perfusion, the rise of the DC-potentials in all areas of brain and the modulation of blood pressure and heart rate. The second process, directly connected with cognitive processing, was manifested by the shifts of local characteristics of brain perfusion and DC-potentials in the frontal, temporal and central cortex, especially in the left hemisphere. Correlations were found between the characteristics of cerebral perfusion and DC-potentials on the one hand and the number of words during the verbal fluency test performance on the other hand.