Vol 14, No 2 (2020)

Introduction. Development of the new approaches to the rehabilitation of patients with poststroke hemianopia is of great importance, given the high prevalence of that disorder and its negative impact on patients’ quality of life.

This study aimed to investigate the effect of transcranial direct current stimulation (tDCS) on the quality of life and the quality of visual perception in patients with poststroke hemianopia, as well as to study the safety and tolerability of this technique.

Materials and methods. The study included ten patients with poststroke hemianopia. Patients underwent ten tDCS sessions (2 mA, 20 min with anode placed over Oz, cathode — over Cz for a unilateral lesion, and anode — over O1 and O2, cathode — over Fp1 and Fp2 for bilateral lesions). The tolerability of tDCS was evaluated after each session using a standardized questionnaire. Clinical assessment before and after tDCS was performed using the Visual Function Questionnaire (VFQ-25) and the 36-item Short Form Survey (SF-36). Functional MRI using a visual paradigm was performed in 5 patients before and after the course of tDCS.

Results. Adverse effects were recorded during 9.9% of the sessions and had low severity in most cases. There was one withdrawal from the study because of increased pain in the hand and leg, in a patient with central post-stroke pain syndrome, which was likely unrelated to the biological effects of stimulation. Data of 9 patients were included in the analysis. There was a statistically significant increase in the overall score on the VFQ-25 (p = 0.02) after tDCS with improvements in the social activity (p = 0.02), mental health (p = 0.02), dependence on others (p = 0.04), and peripheral vision (p = 0.04) sections. A statistically significant improvement was also found in the psychological component of quality of life (p = 0.04), vitality (p = 0.03), social functioning (p = 0.02), role functioning due to physical health (p = 0.04), and general health (p = 0.008). In 3 cases, increased activation of the occipital cortex after tDCS was identified using functional MRI with a visual paradigm.

Conclusion. tDCS is a safe, well-tolerated, and potentially effective method in patients with poststroke hemianopia. Larger, controlled, and randomized studies are needed to clarify the efficacy of this method in hemianopia.

his study aimed to evaluate the epidemiological features ofHuntington disease (HD), based on data from the Centre for Extrapyramidal Pathology and Botulinum Toxin Therapy of theRepublic ofTatarstan.

Materials and methods. We examined 44 patients with HD (mean age 47.3 ± 12.5 years). The study design included determining and comparing clinical data, genealogical and epidemiological data, and laboratory parameters.

Results. The highest prevalence of HD was found in the north-western and north-eastern parts of theRepublic ofTatarstan being 1.1 per 100,000 people, regardless of nationality. The phenomenon of anticipation in HD inheritance was observed, considering the instability and increasing expansion in subsequent generations along the paternal line. Psychiatric manifestations (apathy, irritability, anxiety, depression) were present in 61.4% of cases at disease onset. One male patient was presumably diagnosed with the Westphal variant. Manifest carriers of a CAG-expansion in the range of 36–39 repeats in the mutant gene were found in 15.9% of cases and were predominantly represented by females (85.7%).

Introduction. Carpal tunnel syndrome (CTS) is the most frequent tunnel neuropathy. Local injection of corticosteroids (CS) and wrist splinting are used to treat CTS. The optimal frequency of local CS injections has not been determined.

The aim of this study was to carry out a comprehensive (clinical, electrophysiological, and ultrasound) evaluation of the effect duration of a single local injection of corticosteroid with lidocaine in patients with mild and moderate CTS, in combination with regular wrist splinting for 6 months.

Materials and methods. Changes in the clinical, neurophysiological, and ultrasound parameters were analyzed in 44 cases of mild and moderate CTS after a single local administration of CS and the use of regular wrist splinting. The results of Tinel’s test, Phalen’s test, and the Boston Questionnaire, median nerve conductivity based on electroneuromyography data, cross-sectional area of the nerve at the entrance to the carpal tunnel, and the degree of nerve flattening on ultrasound were assessed before treatment and within six months after injection.

Results. Within 6 months following injection, all patients noted a significant improvement in the clinical condition. The greatest clinical effect occurred in the first 2 months, after which the objective signs of improvement stabilized or gradually regressed. Clinical symptoms, nerve conduction values, and the cross-sectional area remained stably improved for 6 months, while the amplitude of motor and sensory responses remained stable for 2 months.

Conclusion. In mild and moderate CTS, local injection of CS and lidocaine plus splinting provides the maximum improvement in clinical, electrophysiological, and ultrasound parameters for the first 2 months. We hypothesize that a further improvement in objective results is possible with a repeat injection after 2 months.

Introduction. Hepatolenticular degeneration (HLD), or Wilson disease, is one of the severe progressive hereditary disorders of the nervous system. A number of questions regarding its pathogenesis and pathology are the subject of in-depth research.

The aim of the study was to examine cerebral pathology and determine the leading factors in its pathogenesis in the autopsy cases of HLD.

Materials and methods. A postmortem study was carried out in 15 deceased patients (14–35 years old) with HLD. In all cases, clinical diagnosis was based on the characteristic signs of CNS and liver damage, presence of the Kayser–Fleischer corneal ring, and specific biochemical abnormalities in copper and protein metabolism. A set of histological, neurohistological, and histochemical staining methods was used, including the histochemical study of copper after the fixation of brain slices in rubeanic acid.

Results. In all cases, severe microcirculatory changes were found in the brain, characteristic of the angiotoxic component of HLD, with impaired vessel permeability and the development of persistent edema and spongiform changes in brain tissue. Changes were more often found in the basal nuclei area, as well as in the white matter of the cerebral hemispheres, cerebellum, and pons. The cytotoxic component of HLD was evident in the same regions of the brain, represented by degenerative changes in astrocytes and neurons, often ending in their death. The most frequent form of pathology was the appearance of Alzheimer type II glia with ‘naked’ nuclei, and much less frequently, Alzheimer type I glia. The histochemical study showed deposits of copper granules in the endothelial cells of microvessels, Alzheimer type II glia, and neurons – predominantly in the globus pallidus and the caudate nucleus.

Conclusion. A set of pathogenic factors plays an essential role in the pathogenesis of the brain damage in HLD: the toxic effect of copper on the brain, the damage to and impaired permeability of the blood-brain barrier, severe metabolic disturbances caused by liver failure, and brain hypoxia.

Research Center of Neurology, Moscow, Russia

The novel coronavirus pandemic presents one of the most significant challenges to modern healthcare, which involves all medical specialties. The current review encompasses the neurologic manifestations of COVID-19 — a yet to be defined problem. L. Mao et al. have found a third of COVID-19 patients to exhibit neurological symptoms the latter divided into three categories: central nervous system involvement (vertigo, headache, altered consciousness, acute cerebrovascular pathology, ataxia, and seizures) in 24.8% of patients, peripheral nervous system involvement (smell and taste disorders, neuropathy) — 8.9%, and muscle pathology (muscle pain, associated with creatine kinase increase) — 10,7%. Cerebrovascular pathology in 221 patients, described by Y. Li et al. occurred in 5.9% of cases — the majority was comprised by ischemic stroke, and as a whole it was associated with a more severe disease course. T. Oxley et al. described 5 patients (less than 50 years of age) with a large-vessel stroke occurring as a result of COVID-19. It has been shown that meningoencephalitis may be linked to COVID-19 — this review addresses several described cases. A case series of Guillain-Barre syndrome n patients with SARAS-CoV-2 infection is also described. Apart from that, it is well established that SARS-CoV-2 may lead to deterioration of concurrent somatic and (or) neurological diseases, worsening the prognosis.

Dementia is a pathology that leads to disability and significantly affects the quality of life of patients and their relatives. The study and development of treatment methods for dementia is a priority in medicine, given the rate of growth in the number of patients. Vascular dementia is the second most common type of dementia in the world after Alzheimer disease. Poor sleep quality is a common feature of the neuropsychiatric profile in dementia patients. Sleep disorders are a modifiable and significant risk factor for cardiovascular disorders, in particular, vascular cognitive impairment. Several sleep disorders are more pronounced in patients with vascular dementia than in patients with dementia due to Alzheimer disease.

This review contains data on changes in the sleep-wake cycle, often found in vascular cognitive impairments. The likely mechanisms connecting sleep disorders and vascular cognitive impairments are described. The possibility of predicting the development of vascular cognitive impairment based on identified sleep disorders is shown, as well as the potential for studying and developing methods to treat sleep disturbances to prevent vascular cognitive impairment are presented.

Introduction. Frontotemporal dementia (FTD) is a neurodegenerative disease and is the second most frequent cause of early-onset dementia (before 65 years). Quantitative assessment of disease severity can be an essential tool for predicting and objectively assessing the rate of disease progression, which is vital not only for clinical practice but also for research. There are no specialized Russian language scales for evaluating the severity of dementia in FTD, and the use of functional assessment scales developed for patients with Alzheimer disease for this purpose leads to erroneous diagnosis and reduces the quality of research.

The aim of this work was to perform linguistic and cultural adaptation and validation of the Russian version of the Frontotemporal Dementia Rating Scale (FTD-FRS).

Materials and methods: Forward and back translations of the scale and its materials, pilot testing, development of the Russian language version with the help of a linguist and neurologists specializing in working with patients with cognitive impairments were performed.

Results. After completing the forward and back translations, the expert commission removed inaccuracies in the translation of the scale materials. Pilot testing was carried out on 10 patients with FTD. Researchers did not have any difficulties understanding and interpreting the instructions. After this stage, the final edition of the Russian version of the scale was adopted. The article contains the main text of the scale and a link to the full version of the scale with the main text and instructions.

Conclusion. A Russian language version of the Frontotemporal Dementia Rating Scale has been published for the first time. The first stage of validation (linguistic and cultural adaptation) was performed in the Research Center of Neurology. There is ongoing work to evaluate the psychometric properties of the Russian language version.

Glutaric aciduria type I is an autosomal recessive disease caused by mutations in the GCDH gene, which encodes the glutaryl-CoA-dehydrogenase enzyme. A lack of this enzyme leads to the accumulation of glutaric and 3-OH-glutaric (3-hydroxyglutaric) acids in biological fluids and tissues, which have a neurotoxic effect primarily on subcortical brain structures. We present two clinical cases of glutaric aciduria type I in children diagnosed after developing neurological symptoms. One patient had acute disease onset in the form of an encephalopathic crisis, which is typical for most clinical cases of the disease. The other patient developed subacute symptoms. Generalized dystonic hyperkinesia was a key sign of the disease in both patients. Information from the medical history and clinical presentation, together with the neuroimaging results, enabled us to suspect the right diagnosis and to confirm it using biochemical and then molecular genetic tests. In both cases, despite diet therapy using a specialized medicinal product, the patients continued to have motor disturbances.