LECTURES/REVIEWS
Written of history of the description of myopathies, modem classification of muscular dystrophies, clinical-genetic peculiarities of basic types of muscular dystrophies (dystrophinopathy, emerinopathy, facio-scapulo-limb type, limb-girdle types, distal types, ocylopharingeal and oculopharingodistal) and their diagnostic, pathogenesis and methods of treatment.
Low back pain (LBP) is one of the most common causes of temporary disability. The main causes of LBP are considered: 1) nonspecific (musculoskeletal) pain; 2) specific pain caused by a serious pathology (tumor, fracture, infection, etc.); 3) compressive radiculopathy, lumbar stenosis. Non-specific LBP is most common (90-95% of cases). It has a favorable course and can be caused by a diskal hernia (with or without compression of the root of spinal nerve), damage to the facet joints or sacroiliac symphysis, as well as muscles of the back (myofascial pain). Psychosocial characteristics of the chronic course of LBP are also considered.
CLINICAL RESEARCH AND SURVEILLANCE
Introduction. The long-term prognosis of multiple sclerosis (MS) is influenced by various factors, including comorbidity. The purpose of study is to investigate comorbidity in multiple sclerosis using the developed and validated specialized questionnaires.
Materials and methods. The subjects comprised two groups from the population of St. Petersburg: 885 patients with MS and 396 the control group, 154 patients provided outpatient card medical records. A specially designed self-report questionnaire included demographic, anthropometric, lifestyle data, and standard questions on comorbidities. To validate the questionnaire, extracts from individual outpatient cards were analyzed. Validation of questionnaires was performed by mean of kappa statistics. To assess the odds of the presence of comorbidity in each group, Fisher’s exact test was used.
Results. Satisfactory and high agreement of information (kappa> 0.41) revealed for arterial hypertension, ischemic heart disease, atherosclerosis, diabetes mellitus, uveitis, psoriasis, thyroid disease, bronchial asthma, migraine, and cancer. In MS patients, the prevalence of uveitis, autoimmune thyroiditis, anxiety and depression was higher, and chronic obstructive pulmonary disease and obesity prevalence was lower than in control group.
Discussion. The developed questionnaire has demonstrated high sensitivity and specificity for a number of comorbidities. The risk of comorbidities in MS patients In St. Petersburg is comparable to that in other regions of the world. Anxiety and depression are of higher risk in the subgroup of younger patients with MS, and autoimmune thyroid disorders are of higher risk in middle-aged and elderly MS patients compared with the general population.
Pre-Mild Cognitive Impairment (PreMCI) is characterized by patients’ complaints of memory impairment and/or other cognitive functions. In elderly and senile age, PreMCI can be a predictor of the development of more significant cognitive defects up to the development of dementia.
The study was aimed at the analysis of the neuropsychological characteristics of patients with PreMCI in dependence on their age.
Materials and methods. The results of the examination of 547 patients (159 men and 388 women) in the age groups of 45–59 years old, 60–74 years old and 75–89 years old were analyzed in comparison to 104 healthy subjects with the same age characteristics who did not complain of cognitive functions’ impairment. The study included clinical research and the use of scales and questionnaires set evaluating cognitive functions.
Results. Significant differences were detected between patients with PreMCI and healthy subjects of middle and old age in most neuropsychological tests. Patients over 75 years old differed from healthy ones only in the clock drawing test and immediate reproduction of 12 words test. Most significant difference in neuropsychological tests in different age groups of healthy subjects was impairment in executive functions and the index of the 12-word immediate reproduction test. Patients with PreMCI had a deterioration of divided-attention measure indicators, executive and language functions, and constructional praxis, while in memory tests no significant dynamics was detected.
Conclusion. A broader spectrum of cognitive functions’ defects is detected in patients with PreMCI compared to healthy ones. At the same time, memory impairment is a fixed condition, and is not essentially dependent on age.
Epstein–Barr virus (EBV) infection is considered as risk factor for multiple sclerosis (MS) and possibly a factor in the adverse course of the disease. Forty-three patients with remitting MS in remission with the onset of the disease before the age of 18 years and 49 volunteers without MS participated in the study. It was found that the majority of patients with MS, as well as the majority of the control were seropositive for EBV infection (100% and 91.8%, respectively, р = 0.120). All identified seropositive patterns are typical of EBV past-infection. The combination of anti-VCA IgG and anti-EBNA-1 IgG is more common in patients with MS (97.7% vs. 83.7% in control, p = 0.034). The anti-VCA IgG level above the median and the seropositive result for anti-EBNA-1 IgG are associated with MS (OR 5.29; 95СI 1.99–14.04; p < 0.001 and OR 15.36; 95СI 1.95–20.70; p = 0.009, respectively). Anti-VCA IgG levels above the median are associated with HLA-DRB1*1501 in patients with MS (OR 8.4; 95CI 0.87–80.92; p = 0.047). Anti-VCA IgG level, anti-EBNA-1 IgG are not correlated with the age of the MS onset, disability, frequency of exacerbations, as well as with serum concentrations of 25-hydroxy vitamin D. It can be assumed that the patients with remitting MS have features of the EBV past-infection immune status. Dynamic observation of the serological markers levels of EBV infection is important not only in remission, but also in exacerbation of MS. The results of these observations will assess the prospects of EBV infection control and treatment for the management of patients with MS.
Purpose of the study. Optimization of the diagnostic algorithm of neuropsychic development in the context of primary pediatric and specialized neurological care on the basis of analysis of the formation of psychomotor, speech and mental abilities in children with infantile cerebral paralysis, formed on the background of hypoxic-ischemic and hypoxic-hemorrhagic perinatal CNS damage.
Materials and methods. The article presents an assessment of psychomotor, speech and mental development of 81 children with infantile cerebral paralysis (ICP): 50 patients with hypoxic-ischemic central nervous system damage and 31 patients — with hypoxic-hemorrhagic central nervous system damage.
Results. PSlowing of psychomotor development (SPD) was diagnosed in 98.8% of cases. The greatest percentage of children with SPD was noted in children under 1 year of age — 40%. The majority of children with SPD are noted under age 2. The amount of children with the delay in speech development (DSD) was 93.8%. DSD was absent in 5 (6.2%) patients. In children under 1.5 years of age, DSD was noted in 34.2% of all cases, while in the group with hypoxic-ischemic damage (HID) of the central nervous system — 47.9% and only 10.7% — in children with hypoxic-hemorrhagic damage (HHD) of the central nervous system(p < 0.01). At the age of 1.6 up to 2 years, the proportion of children with DSD from the group with HID of the central nervous system was 35.4%, and in the group with HHD of the central nervous system — 82.1% (p < 0.01). Mental retardation (MR) was noted in 28.4% of children. The severe and deep forms of MR were 2 times higher in groups with HHD of the central nervous system (22.2 and 11.1%, respectively). Other mental disorders, caused by brain dysfunction, were diagnosed in 50.6% of patients. The absence of MR and other mental disorders was noted in 21% of cases.
Conclusion. Presumably, the cause of high detectability of SPD in children under 2 years of age is late evaluation of the child’s speech abilities by parents . The presence of MR in 1/3 of all children under study, and the presence of other mental disorders in 50.6% of patients, indicates a significant proportion of developmentally delayed children with infantile cerebral paralysis (ICP).
DRUGS IN NEUROLOGY
The standard for the treatment of dementia in Alzheimer's disease (AD) is the use of basic medicines affecting the cholinergic and glutamatergic systems.
The aim of the study was a comparative evaluation of combined treatment with an NMDA receptor antagonist akatinol memantine and donepezil — acetylcholinesterase inhibitor, compared with donepezil monotherapy in AD patients with mild dementia.
Material and methods. The study included 35 patients with AD (average age 78.51 years old ± 6.08) at the stage of mild dementia. 35% of patients received donepezil monotherapy (alzepil), 65% of patients received donepezil and akatinol memantine combined treatment. The observation period was 12 months. During this period, the patients underwent a neuropsychological examination every 3 months. It included a Mini Mental State Examination, Frontal assessment battery, memory test with an assessment of immediate and delayed reproduction of «12 words», verbal fluency (phonetic and semantic), clock drawing test, Trial making test, part A.
Results. In patients, who received the combined treatment, improvement was detected on almost all cognitive scales, in 3 months of the therapy. It continued to increase throughout the whole observation period. And, contrary-wise, in patients, who received monotherapy, only stabilization of cognitive status was manifested.
Conclusion. The administration of combined treatment with akatinol memantine and donepezil to patients with AD at the stage of mild dementia during the period of 12 months, is more effective than donepezil monotherapy.
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