LITERATURE REVIEW
Parkinson’s disease (PD) is one of the most common neurodegenerative diseases in the world. While until recently MRI was used exclusively for the diagnosis of symptomatic forms of parkinsonism, recent advances in neuroimaging allow the detection of signs of nigral degeneration (MR biomarkers of PD). The article discusses the possibilities of modern MRI modes sensitive to iron (SWI, T2*) and neuromelanin (neuromelanin-sensitive MRI); emphasis is placed on identifying false-negative and false-positive results of the study. The imaging of nigrosome-1 in the dorsal substantia nigra (SN) in MRI-SWI has been discussed since 2013. In the absence of nigral degeneration, this area is defi ned as a hyperintense ovoid area within the dorsolateral border of the hypointense SN (“swallow’s tail” sign). If the optimistic results of the fi rst studies testifi ed to the high sensitivity and specifi city of this technique in PD (the absence of the “swallow’s tail” sign), then in subsequent studies, similar changes were detected in patients with other neurodegenerations with parkinsonism. In addition, the diagnostic value of this technique occurs when using tomographs with a magnetic fi eld strength of at least 3 Tc. Similar conclusions can be drawn about the use of neuromelanin-sensitive MRI, since it is necessary to use high-fi eld magnetic tomographs of 3 Tc or more to detect nigral degeneration, and results similar to PD can also be found in atypical forms of parkinsonism. However, the search for signs of nigral degeneration may be useful in the diff erential diagnosis of PD and non-neurodegenerative disorders. In addition to MRI in the diagnosis of PD, the article discusses neuroimaging in various types of multisystem atrophy, progressive supranuclear palsy, and dementia with Lewy bodies. The article is illustrated with own MRI scans of the brains of patients with PD and other forms of parkinsonism.
CLINICAL RESEARCHES AND CASE REPORTS
Introduction. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is common variant of small vessel disease. Determination of neuroimaging markers have already contributed at an early stage to the more timely diagnosis of the CADASIL.
Objective. The aim of the study was to determine probable CADASIL magnetic resonance imaging (MRI) biomarkers for patients from the Russian population.
Material and methods. The study included 16 patients. Diagnosis of CADASIL was suspected based on clinical data and an MRI and confi rmed by a molecular genetic study of the NOTCH3 gene. Brain MRI data from 16 patients and spinal cord MRI data from 5 patients were collected and analyzed. Cerebrospinal fl uid (CSF) analysis was performed for diff erential diagnosis in 2 subjects.
Results. White matter hyperintensity was observed in 100% of cases. The involvement of the anterior temporal lobes and external capsules was revealed in 62 and 68% of subjects respectively. Multiple lacunar infarcts are characteristic for CADASIL. Microbleeds were detected in 27% of patients. Brain atrophy was observed in 56% of cases. Lesions in corpus callosum and cervical spinal cord were found in 2 patients.
Conclusion. Detection of white matters hyperintensities in frontal and parietal lobes, multiple lacunar infarcts with suspected CADASIL makes it possible to select the group of patients for further molecular genetic testing in order to detect mutations in NOTCH3 gene.
Muscular-tonic disorders (MTD) in prolonged disorders of consciousness (PDoC), including a vegetative state (VS) and a minimal consciousness state (MCS), are poorly understood.
Aim. To systematize MTD in PDoC, to highlight the features of their dynamics depending on the change in consciousness.
Material and methods. 87 patients in PDoC (VS — 52, MCS — 35) resulting from brain damage of diff erent etiology, lasting from 2 months up to 10 years. MTD, provoking hyperkinesis factors and consciousness were analyzed retrospectively in the dynamics and complex.
Results. MTD had 98% of patients in PDoC. The ratio of occurrence of spasticity: hyperkinesis: postural spasms corresponded to 11:11:10, and hyperkinesis — dystonia: myoclonus/myokymia: athetosis: stereotypes: ballism: choreiform hyperkinesis: tremor — 17:10:6:3:2:1:1. Their clinical variants and features are noted. The total dynamics of MTD on improved consciousness was as follows. In general, regardless of the initial VS or MCS, their “change” (especially decrease) prevailed over “no changes” (p < 0.001). Comparatively more often the decrease occurred in MCS “plus” (p < 0.05); appearance/increase/modifi cation — in VS (p < 0.05); “no changes” — in MCS “minus” (p < 0.01). When considered separately, spasticity, dystonia, spasms, hemiballismus and stereotypy in MCS “plus” correlated (p < 0.01) with the change of consciousness. The key provoking hyperkinesis factors in VS were pain and other sensory infl uences (p < 0.01), but their role from MCS “minus” to MCS “plus” decreased, while the role both conscious emotions and movements increased (p < 0.01).
Conclusion. Along with academic interest, the data are promising in developing the prognosis, pathogenesis and treatment of PDoC.
The association of predisposition to multiple sclerosis (MS) with HLA-DRB1 gene polymorphisms is the strongest. It is not clear whether the DRB1 alleles associated with the risk of this disease diff er in adult and pediatric populations living in the same environmental conditions.
Objective: comparative study of associations of HLA-DRB1 gene polymorphism with the risk of pediatric-onset MS and adult-onset MS in the Altai region.
Material and methods. Caucasian with relapsing-remitting MS, born and living in the Altai region of Russia in the southeast of Western Siberia, participated in the case–control study: 200 patients with adult-onset MS, 86 patients with pediatric-onset MS. The control group included 200 volunteers. Genotyping was performed by TaqMan probes. Results. Alleles 03, 13, 15 of the HLA-DRB1 gene are genetic risk factors for both adult-onset MS and pediatric-onset MS in Caucasians in the Altai region. Alleles 01 and 07 of the HLA-DRB1 gene may have a protective eff ect against pediatric-onset MS, alleles 01, 07, 11 and 16 against adult-onset MS.
Conclusion. It can be assumed that the diff erence in the age of MS onset is not associated with the diff erent infl uence of risk alleles of the HLA-DRB1 gene in populations under and over 18 years of age.
Background. Neuropsychiatric disorders are common after stroke. They decrease patients’ quality of life and have negative impact on the disease outcomes.
The objective: to analyze the reliability of the Russian Version of Emotional Behavior Index and assess the frequency and characteristics of the disorders detected by this scale in patients in acute phase of ischemic stroke.
Material and methods. Fifty-nine patients were recruited. The reliability of the tool was assessed. Along with the disorders of emotional behavior stroke severity, degree of disability, presence of delirium, symptoms of depression and cognitive functions were assessed.
Results. Changes in at least one category of the “Emotional Behavior Index” were observed in 40% of patients. Emotional behavior problems were associated with the severity of cognitive decline and depressive symptoms in acute stage of stroke.
Conclusion. “Emotional Behavior Index” is a reliable instrument for the assessment of the state of mood of the patients with acute stroke. The results of this scale could be used as predictors of the development of cognitive and aff ective disorders.
No multicenter randomized clinical trial has been conducted worldwide to date on indications, types of surgery and their comparison with conservative treatment in patients with PI BSA.
Aims: of the study is to improve the results of surgical treatment in patients with pathological tortuosity of the internal carotid artery
Material and methods. Тhe study included 115 patients (38 (33%) men and 77 (67%) women) with PI ICA aged 35 to 72 years (average age 54.2 ± 7.5 years) divided into 2 groups. 61 patients (53%) of group I underwent BSA resection with lower mouth and 54 patients (47%) of group II underwent BSA prosthetics. Depending on the degree of neurological disorders, patients were distributed according to the classifi cation of A.V. Pokrovsky.
Results. In patients with СМН1, the clinical eff ect was due to the absence of cerebrospinal and focal neurological symptoms. As a result of surgical treatment of PI BSA in group I, a clinical eff ect was achieved in 54 (89%) patients. In group II, regression of clinical-neurological symptoms was observed in 40 (74%) patients.
Conclusions. Resection and prosthetics of BSA PI in order to stop neurological symptoms and prevent ischemic stroke in patients with this pathology is a safe and eff ective method of surgical treatment.
Dabigatran etexilate (DE) is a direct thrombin inhibitor that has been shown to be eff ective and safe in preventing thrombotic events in a number of studies. Currently idarucizumab, which is a monoclonal antibody and a DE antagonist, is used to immediately inactivate the DE-induced eff ect.
Objective. Еvaluation of the effi ciency and safety of idarucizumab in patients receiving DE.
Material and methods. 6 patients (2 men, 4 women) aged 61 to 86 years (mean age 72.8 ± 10.6 years) receiving DE, who are expected to use idarucizumab in achieving the goal of sTLT or surgery.
Results. In none of the patients the use of idarucizumab was accompanied by a decrease in thrombin time of less than 11 seconds which could indicate a hypercoagulable phenomenon. Before inactivation of dabigatran etexilate thrombin time was signifi cantly higher (p < 0.05) than after the administration of the drug. There were no statistically signifi cant diff erences in the concentration of D-dimer before and after the administration of idarucizumab which indicates the absence of procoagulant properties of this drug. None of the patients developed clinically signifi cant arterial and/ or venous thrombotic events such as recurrent IS, myocardial infarction, deep vein thrombosis of the lower extremities and pulmonary embolism, during the entire period of hospitalization.
Conclusion. The use of idarucizumab is allowed for systemic thrombolytic therapy and emergency surgical treatment in patients taking DE. Idarucizumab quickly and safely neutralizes the anticoagulant eff ect of DE and doesn’t have a prothrombotic activity.
Clinical case of structural epilepsy in a patient with multiple sclerosis is presented, which shows the achievement of seizure’s stable remission, which is extremely rare in forms of epilepsy with a multi-focal lesion another etiology and a long history of the disease without combination with multiple sclerosis. Video-EEG monitoring, positron emission tomography of the brain with 18F-deoxyglucose, expand the possibilities of diagnosing form of epilepsy in patients with combined pathology, which allows prescribing adequate therapy.
LECTURE
Palliative care for neurological patients is an approach to patient care and assistance to their families facing chronic, progressive, life-limiting and disabling conditions. The peculiarity of palliative care in neurology is due to the signifi cant duration of the late stages of many neurological diseases and the distinctive features of some diseases (cognitive disorders, mental disorders, speech disorders, pelvic organ dysfunction, paresis, convulsions, contracture development, etc.), as well as the presence of concomitant common symptoms (eating disorders, pain, bedsores, respiratory failure, etc.). The symptoms developing in the advanced stages of neurological diseases requiring palliative care are described. Drug and non-drug methods of treatment are described, measures aimed at improving the quality of life and preventing complications are considered. The features of pharmacotherapy, taking into account their interaction with drugs for the treatment of concomitant pathology, are considered.
ISSN 2686-7192 (Online)