[Chronic inflammatory demyelinating polyneuropathy with an acute onset. Clinical case].

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous treatable dysimmune neuropathy. The variety of clinical forms and course of the disease can be challenging for proper diagnosis and early treatment. In a quarter of cases CIDP starts acutely, mimicking GuillainBarr syndrome. The early diagnosis is especially important regarding differences in treatment and prognosis of these conditions. In this article, we present a clinical case of acute onset CIDP with a detailed analysis of the differential diagnosis between acute and chronic immune-mediated neuropathies.

[The possibilities of differential diagnosis of primary progressive aphasia]. / Differentsial'naya diagnostika pervichnoi progressiruyushchei afazii.

The authors describe a clinical case demonstrating the difficulties of diagnosing primary progressive aphasia, the need for a differential diagnosis with a logopenic variant of Alzheimer's disease. The data of a clinical, neuropsychological study with a detailed instrumental and diagnostic search, including data from cerebrospinal fluid, positron emission tomography-CT, MRI, electroencephalography with cognitive loads, are presented. The article focuses on the widespread use of various diagnostic scales based on the international diagnostic criteria.

[The use of vitamin B12 in cancer patients]. / Primenenie vitamina B12 u onkologicheskikh bol'nykh.

Based on the data of foreign and domestic literature, the question of the possible carcinogenesis of vitamin B12 and the expediency of its administration to patients with cancer who have neurological complications due to chemotherapy is analyzed. The absence of convincing direct evidence of the carcinogenic effect of cyanocobalamin has been demonstrated, as well as the reasonableness of drug correction of vitamin B12 deficiency in cancer patients in order to prevent the development of characteristic deficient neurological disorders. The question of the effectiveness of oral administration of cyanocobalamin at a dose of 1 mg per day is considered.

[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.

[Cavinton Comforte in the correction of vascular cognitive disorders]. / Kavinton komforte v korrektsii kognitivnykh narushenii pri khronicheskoi ishemii golovnogo mozga.

AIM: To study the effect of cavinton comforte (CC) on the severity of cognitive impairment and motor awkwardness in patients with chronic brain ischemia (HIGM). MATERIAL AND METHODS: There were 30 patients (8 men, 22 women) aged 50-65 years (61.6±4.9 years on average) with AHU with cognitive impairment. We used tests of MINI-Cog, Munsterberg, TMT), ADL). QC appointed 10 mg 3 times a day for 3 months. RESULTS: Against the background of therapy, the improvement of constructive praxis and short-term memory, an increase in the speed of visual search and visual-motor function, an increase in the level of selectivity of attention, an improvement in the reproduction of visual images and perception, an increase in the speed of the motor task (drawing strokes). CONCLUSION: The obtained data can testify about the improvement of indicators of function of a small motility of brushes on the background of improvement of cognitive functions.

[Emotional, affective and behavioral disorders in a behavioral variant of frontotemporal dementia]. / Émotsional'nye, affektivnye i povedencheskie narusheniia pri povedencheskoi forme lobno-visochnoi dementsii.

AIM: To determine the prevalence and severity of non-cognitive nervous and psychiatric disorders (NNPD) in a behavioral variant of frontotemporal dementia. MATERIAL AND METHODS: Twenty-nine patients with BVFTD, aged from 41 to 73 years (mean 60.7±8.1 years), were studied. All patients underwent neurological and neuropsychological examinations. NNPD were assessed using the Neuropsychiatric Inventory (J. Cummings et al). Twenty-seven patients underwent brain MRI with T1, T2 and FLAIR sequences. RESULTS: The most clinically significant symptoms of NNPD were apathy, behavioral disinhibition, eating disorders, abnormal motor activity and euphoria. Irritability, sleep disorders and excitement were less frequent. Anxiety and depression were identified in 13.8 and 20.7% of the patients, respectively. The severity of NNPD can increase and their spectrum can be qualitatively changed with the disease progression that indicates the spread of the neurodegenerative process. CONCLUSION: Patients with BVFTD had all NNPD with the exception of delusion and hallucinations. The character and degree of severity of some emotional, affective and behavioral disorders are associated with the predominant localization of the pathological process in frontal and temporal brain regions.

[Age dynamics of cognitive functions in persons 50-85 years].

The paper presents original study results of cognitive changes associated with aging in the absence of neurodegenerative, vascular and other significant for cognition disorders in period from 50 to 85 years. It was shown that aging is associated with moderate memory decrease predominantly because of retrieval deficit but not acquisition insufficiency. It was also shown that aging is associated with non-severe executive dysfunction (lack of planning and control). According usual neuropsychological approaches pattern of cognitive changes described above reflects anterior cortical dysfunction or/and impaired interaction between frontal lobes and subcortical basal ganglia. Shows what physiological changes occur with age in the field of memory and executive functions, which is of great clinical importance for the differential diagnosis of normal aging and early stages of common in the elderly cerebral diseases.

Method for making a single-step etch mask for 3D monolithic nanostructures.

Current nanostructure fabrication by etching is usually limited to planar structures as they are defined by a planar mask. The realization of three-dimensional (3D) nanostructures by etching requires technologies beyond planar masks. We present a method for fabricating a 3D mask that allows one to etch three-dimensional monolithic nanostructures using only CMOS-compatible processes. The mask is written in a hard-mask layer that is deposited on two adjacent inclined surfaces of a Si wafer. By projecting in a single step two different 2D patterns within one 3D mask on the two inclined surfaces, the mutual alignment between the patterns is ensured. Thereby after the mask pattern is defined, the etching of deep pores in two oblique directions yields a three-dimensional structure in Si. As a proof of concept we demonstrate 3D mask fabrication for three-dimensional diamond-like photonic band gap crystals in silicon. The fabricated crystals reveal a broad stop gap in optical reflectivity measurements. We propose how 3D nanostructures with five different Bravais lattices can be realized, namely cubic, tetragonal, orthorhombic, monoclinic and hexagonal, and demonstrate a mask for a 3D hexagonal crystal. We also demonstrate the mask for a diamond-structure crystal with a 3D array of cavities. In general, the 2D patterns on the different surfaces can be completely independently structured and still be in perfect mutual alignment. Indeed, we observe an alignment accuracy of better than 3.0 nm between the 2D mask patterns on the inclined surfaces, which permits one to etch well-defined monolithic 3D nanostructures.