Vol 14, No 2 (2020)

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Full Issue

Original articles

Transcranial Direct Current Stimulation in Poststroke Hemianopia

Bakulin I.S., Lagoda D.Y., Poydasheva A.G., Kremneva E.I., Suponeva N.A., Piradov M.A.


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.

Annals of Clinical and Experimental Neurology. 2020;14(2):5-14
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The Diagnostic Significance of Intrathecal Synthesis of Antibodies Against Measles, Rubella, and Varicella Zoster Viruses in the Differential Diagnosis of Demyelinating Diseases of the Central Nervous System

Moshnikova A.N., Lapin S.V., Nazarov V.D., Krasnov V.S., Dovidenko K.S., Surkova E.A., Savostyanova V.N.


Introduction. The basis of autoimmune inflammation in multiple sclerosis (MS) is the polyclonal activation of B lymphocytes in the central nervous system, followed by intrathecal immunoglobulin production, caused by the synthesis of antibodies to measles, rubella, and varicella zoster viruses (MRZ reaction).

The study aimed to assess the possibility of using the IgG index, oligoclonal immunoglobulin synthesis, the permeability coefficient of the blood-brain barrier, specific indices of antibodies against measles, rubella, and varicella zoster viruses with the calculation of the MRZ reaction in the diagnosis of MS, neuromyelitis optica, and acute disseminated encephalomyelitis.

Materials and methods. The study included 40 patients with the following diagnoses: MS (n = 19), acute disseminated encephalomyelitis (n = 11), and neuromyelitis optica (n = 10). Intrathecal synthesis indicators were measured in all patients in paired samples of cerebrospinal fluid and serum.

Study results. In the study group, the MRZ reaction was a specific test for the diagnosis of MS and was not found in neuromyelitis optica or acute disseminated encephalomyelitis. An increase in the IgG synthesis index was observed in only 90.5% of patients with MS, and oligoclonal IgG was detected in only 71.4% of cases. Elevated albumin quotient values are not typical for MS, which characterizes the degree of permeability of the blood-brain barrier.

Conclusion. The MRZ reaction is a highly specific laboratory marker for verifying MS. It also enables differential diagnosis of MS and other demyelinating diseases.

Annals of Clinical and Experimental Neurology. 2020;14(2):15-22
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Clinical and Epidemiological Aspects of Huntington Disease in the Republic of Tatarstan

Munasipova S.E., Zalyalova Z.A.


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%).

Annals of Clinical and Experimental Neurology. 2020;14(2):23-28
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Surgical Treatment of Trigeminal Neuralgia Combined with Tumors of the Cerebellopontine Angle

Grigoryan G.Y., Dreval О.N., Maslova N.N., Sitnikov А.R.


Introduction. Trigeminal neuralgia (TN) develops because of demyelination in the entry zone of the trigeminal nerve root (TNR) into the brainstem, which is accompanied by ectopic and cross-excitation of the nerve impulses with paroxysms of facial pain. One of the causes of TN are tumors of the cerebellopontine angle (CPA), which have a direct or indirect effect on the TNR. The optimal surgical approach in such cases depends on the anatomical relationship between the TNR and the tumor and vascular structures.

The aim of the study was to evaluate the relationship between the anatomical variants of the TNR and the outcomes of using various surgical methods to treat TN in CPA tumors.

Materials and methods. We performed a retrospective analysis of 52 patients (38 women and 14 men aged 22–82 years) with TN and ipsilateral tumors of the CPA: 29 with apical petrous meningiomas, 11 with epidermoid tumors, 10 with vestibular schwannomas, 1 with haemangioma, and 1 with cavernoma.

Results. Six variants of the anatomical relationships of the TNR to CPA tumors and the adjacent vascular structures were identified: where the TNR is completely surrounded by the tumor; where the tumor compresses and displaces the TNR; where the tumor is located inside the TNR; where the tumor, together with the blood vessel, compresses the TNR; where the tumor displaces the TNR towards the vessel; and where the tumor is not in contact with the TNR, but the TNR is compressed by the blood vessel. Displacement and deformation of the TNR by CPA tumors was found in 50 patients, and no direct contact between the TNR nerve fibers and the neoplasm was observed in 2 cases. The neurovascular conflict caused by arterial and venous vessels was identified in 16 subjects. The type of a vascular decompression technique depended on the degree of stretching and deformation of the TNR. Postoperative MRI confirmed the complete removal of CPA tumors in 51 patients, while minor epidermoid residue in the contralateral cerebellopontine angle cistern was found in only one subject. There were no fatalities after surgical interventions, and the postsurgical cranial nerve neuropathies were transient and regressed within 2–3 months. In 15 cases, hypoesthesia developed or worsened in the trigeminal nerve innervation zone, then disappeared within several weeks, and was not accompanied by a recurrence of TN. Facial sensitivity improved in 6 cases, who had not previously undergone any surgical manipulations of the TNR and its peripheral branches. Complete regression of TN in the immediate postoperative period was observed in 51 patients. A percutaneous radiofrequency trigeminal rhizotomy was performed in 1 subject with an epidermoid tumor, to eliminate the intense pain paroxysms that persisted for a week. No recurrent growth of CPA tumors or relapses of TN were observed during the subsequent follow-up period of 2–10 years.

Conclusion. TN can be caused by direct compression and deformation of the TNR and brainstem by CPA tumors and vascular structures. A detailed examination of the entire length of the TNR after tumor removal is necessary to evaluate the neurovascular relationships. In cases of vascular compression of the TNR, various methods of microvascular decompression can be used to treat TN.

Annals of Clinical and Experimental Neurology. 2020;14(2):29-36
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A Comprehensive Evaluation of the Effect Duration of a Single Local Corticosteroid Injection in Combination with Regular Wrist Joint Splinting in Carpal Tunnel Syndrome

Kiselev V.N., Alexandrov N.Y., Alexeeva T.M.


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.

Annals of Clinical and Experimental Neurology. 2020;14(2):37-42
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Gene Expression of Lysosomal Membrane Proteins in Parkinson Disease, Associated with Mutations in the Glucocerebrosidase Gene (GBA)

Usenko T.S., Bezrukova A.I., Bogdanova D.A., Nikolaev M.A., Miliukhina I.V., Gracheva E.V., Senkevich K.A., Zakharova E.Y., Emelyanov A.K., Pchelina S.N.


Introduction. In carriers of a mutation in the lysosomal enzyme glucocerebrosidase (GBA) gene, the risk of Parkinson disease (PD) is increased by 7–8 times. However, not all carriers of the GBA gene mutations develop PD during their lifetime. We hypothesize that the dysfunction in the lysosomal membrane proteins involved in autophagy and transport of GBA into the lysosomes can contribute to the development of PD in carriers of mutations in the GBA gene.

The aim of the study was to assess the contribution of LAMP2 and SCARB2 genes expression in CD45+ peripheral blood cells to the development of GBA-PD.

Materials and methods. We examined 9 patients with GBA-PD, 9 asymptomatic GBA gene mutations carriers, 37 patients with PD, and 56 people in the control group. The relative mRNA level of LAMP2 and SCARB2 genes in CD45+ blood cells, obtained using magnetic sorting, was measured by quantitative real-time polymerase chain reaction using fluorescent probes.

Results. The relative mRNA level of LAMP2 and SCARB2 genes in CD45+ blood cells was reduced in patients with GBA-PD in comparison to patients with PD and to controls (LAMP2: p<0.0001, p = 0.01 respectively; SCARB2: p = 0.01, p<0.05, respectively) and in asymptomatic carriers of GBA gene mutations compared to patients with PD (LAMP2: p = 0.021; SCARB2: p<0.05) and controls (LAMP2: p = 0.029). We also found decreased mRNA levels of the LAMP2 gene (p = 0.024) and the absence of differences in the mRNA levels of the SCARB2 gene (р<0.05) in CD45+ blood cells in patients with GBA-PD when compared to the group of asymptomatic carriers of GBA gene mutations.

Conclusion. GBA-PD is characterized by a pronounced expression of the LAMP2 gene in the CD45+ peripheral blood cells, which may indicate a role of the decreased LAMP2 gene expression in the pathogenesis of GBA-PD.

Annals of Clinical and Experimental Neurology. 2020;14(2):43-49
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Cerebral Pathology in Hepatolenticular Degeneration (Wilson Disease)

Gulevskaya T.S., Chaykovskaya R.P., Anufriev P.L.


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.

Annals of Clinical and Experimental Neurology. 2020;14(2):50-61
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Neurological aspects of COVID-19

Tanashyan M.M., Kuznetsova P.I., Raskurazhev A.A.


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.

Annals of Clinical and Experimental Neurology. 2020;14(2):62-69
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Legal Aspects of Medical Care in Hospital-acquired Ischaemic Stroke

Voznyuk I.A., Kolomentsev S.V., Nikitin Y.N.


Generally, the main pathogenetic mechanisms of hospital-acquired ischaemic stroke (HAIS) do not differ from classical ones, except in cases of iatrogenic origin. In certain cases, HAIS may be caused by insufficient vascular risk prevention or develop as a complication of medical intervention. At the current stage of the healthcare development, there is an increased legal responsibility of doctors, associated with the broad legal capabilities of a patient to protect their rights and legitimate interests when obtaining medical care. The very fact of a stroke — a disease with a high rate of adverse outcomes — contradicts the prevailing opinion in the society that a patient in a hospital is guaranteed ‘protection’ from the possibility of developing another, especially a more serious illness, and this may give rise to judicial proceedings. Currently, many difficulties arise in the treatment of patients with HAIS, based on the lack of a unified approach to providing medical care for this pathology, including at the legislative level. Data from the world literature and our own studies demonstrate delays in performing diagnostic measures in cases of inpatient stroke and lower indicators of the quality of medical care. At the same time, the human factor is critical and affects the promptness of HAIS diagnosis and treatment. Proper organization of medical care for HAIS is a prerequisite for ensuring that patients receive timely care in accordance with the standards of treatment, and that medical employees of all levels receive legal protection.

Annals of Clinical and Experimental Neurology. 2020;14(2):70-75
pages 70-75 views

Sleep and Wakefulness Disorders in Vascular Cognitive Impairment

Merkulova T.B., Kovrov G.V., Yakhno N.N.


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.

Annals of Clinical and Experimental Neurology. 2020;14(2):76-81
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Frontotemporal Dementia Rating Scale (FTD-FRS): Linguistic and Cultural Adaptation in Russia

Shpilyukova Y.A., Yusupova D.G., Akmadullina D.R., Polekhina N.V., Naminov A.V., Sinelnikov M.E., Zaytsev A.B., Zimin A.A., Belopasova A.V., Fedotova E.Y., Suponeva N.A., Illarioshkin S.N., Piradov M.A.


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.

Annals of Clinical and Experimental Neurology. 2020;14(2):82-87
pages 82-87 views

Clinical analysis

Two clinical cases of glutaric aciduria type I in the Republic of Bashkortostan

Saifullina E.V., Magzhanov R.V., Davletova A.I., Mardanova A.K., Timofeyeva E.A., Kurkina M.V., Zakharova E.Y.


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.

Annals of Clinical and Experimental Neurology. 2020;14(2):88-92
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Leukoencephalopathy with Brainstem and Spinal Cord Involvement and Lactate Elevation (LBSL) on Magnetic Resonance Spectroscopy: A Clinical Observation

Kazantsev A.Y., Matveeva T.V.


Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) on magnetic resonance spectroscopy is a rare and little-known form of hereditary leukoencephalopathy, a significant portion of which is probably obscured by the label of primary progressive multiple sclerosis. The article presents a clinical case of an adult form of LBSL and a review of the literature on this disease, discusses the particular pyramidal syndrome lesions in LBSL and multiple sclerosis, and presents the MRI criteria for LBSL. The difficulty in differential diagnosis with Krabbe disease is highlighted. Clinical observation confirms the difficulty of diagnosing LBSL and the lack of medical awareness about this disease.

Annals of Clinical and Experimental Neurology. 2020;14(2):93-98
pages 93-98 views

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