Vol 10, No 2 (2016)

Original articles

The main pathogenetic mechanisms of vascular cerebral pathology associated with atherosclerosis and metabolic syndrome: the search for correction approaches

Tanashyan M.M., Lagoda O.V., Antonova K.V., Raskurazhev A.A.

Abstract

Metabolic syndrome (MS) is a risk factor for the development of acute and chronic vascular diseases of the brain. Oxidative stress developing under these conditions promotes the progression of atherosclerotic disease of the brachiocephalic arteries, which necessitates correction of the identified pathobiochemical abnormalities. The use of antioxidant drugs in patients with cerebrovascular diseases and MS reduces manifestations of the latter, including insulin resistance, hypertriglyceridemia, and hyperglycemia. The article presents the results of investigation of the drug mexidol in patients with chronic cerebrovascular diseases associated with atherosclerosis and MS. The findings demonstrate that drugs of this group have good prospects to be used for comprehensive correction of various pathogenic stages of the developing pathology.
Annals of Clinical and Experimental Neurology. 2016;10(2):
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The relationship between visuospatial disorders in Parkinson’s disease and thickness of retina and cortex

Litvinenko I.V., Boyko E.V., Kulikov A.N., Dynin P.S., Trufanov A.G., Mal’tsev D.S., Yurin A.A.

Abstract

This paper presents the current data on the mechanisms of formation of visuospatial disorders in Parkinson’s disease (PD). Optical coherence tomography method was used to study the retina, using the protocols designed to assess the thickness of ganglionic layer, retinal nerve fiber layer, and macular map. Central structures were assessed using MRI of the brain followed by evaluation of the cortical thickness. Patients underwent neuropsychological tests, including tests designed to assess the severity of the disease, visuospatial perception, and cognitive functions. The results revealed the retinal and prestriate areas with the most significant changes in patients with different stages of PD, and allowed us to hypothesize the role of changes in the peripheral and central structures of the visual analyzer in formation of symptom complex of visuospatial disorders in PD.
Annals of Clinical and Experimental Neurology. 2016;10(2):
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Dystonic camptocormia: clinical presentation, diagnosis, and treatment results

Likhachev S.A., Chernukha T.N., Zabrodets G.V., Gleb O.V., Alekseevets V.V., Terekhov V.S.

Abstract

This study was aimed at assessing clinical features of camptocormia as one of the symptoms of dystonia and evaluating the effectiveness of various treatments for this condition. The study involved 39 patients with dystonia and camptocormia symptoms. We analyzed the clinical features of dystonic camptocormia, evaluated the results of pharmacotherapy and botulinum therapy, as well as neuromodulation methods, namely transcranial magnetic stimulation and deep brain stimulation. It was shown that phased and integrated approach is required to manage patients with camptocormia caused by dystonic hyperkinesis. Local injection of botulinum toxin and deep brain stimulation are the most effective treatments. More extensive use of low-frequency transcranial magnetic stimulation may be advisable for everyday practical use in patients with dystonic prosternation.
Annals of Clinical and Experimental Neurology. 2016;10(2):
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Antibodies to myelin lipids in multiple sclerosis

Ivanova M.V., Zakharova M.N.

Abstract

Development of immunopathological reactions in the central nervous system (CNS) is one of the key events in the pathogenesis of multiple sclerosis (MS). Humoral immune responses with formation of antibodies to the components of the myelin sheath play an important role. However, the targets for antibodies, their contribution to the development of pathologic process, and stages of the disease, where they play the most important role, are still not quite clear. In this study, we investigated the frequency of detection of antibodies to myelin glycolipids in MS and their relationship with clinical features of the disease. The results of the study showed that patients with MS demonstrate a trend towards more frequent detection of antibodies to glycolipids and sulfatide in blood serum, which is especially pronounced in patients with secondary progressive course. Antibodies to GM1 ganglioside were significantly more frequently detected in patients with secondary progressive MS as compared to patients with remitting course and healthy volunteers. These results are indicative of the fact that antibodies to lipids may participate in the development of demyelinating and neurodegenerative processes in MS and be the markers of disease progression. Further development of the concept of the mechanisms of humoral response to myelin lipids in MS and identification of the most significant antibody targets will facilitate the development of new approaches to prediction of disease course and discovery of new targets for immunomodulating therapy.
Annals of Clinical and Experimental Neurology. 2016;10(2):
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Epidemiology of Huntington’s disease in the Khabarovsk Territory

Proskokova T.N., Skretnev A.S.

Abstract

We conducted the first population-based study of Huntington’s disease (HD) in the Khabarovsk Territory (Khabarovsk, Komsomolsk-on-Amur, and 17 districts of the region). A total of 96 patients were identified, including 77 cases of familial disease (35 families), 15 cases – sporadic, and 4 – with unknown history. HD prevalence in the Khabarovsk Territory was 7.1:100 000 population. The article presents the clinical cases with rare forms of HD, confirmed by DNA diagnosis, namely Westphal juvenile form and primary akinetic-rigid adult-onset form. The role ofthorough collection of family history and molecular genetic testing to establish the correct diagnosis was emphasized.
Annals of Clinical and Experimental Neurology. 2016;10(2):
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Evaluation of the effects of new peptide compounds in experimental animals with a toxic model of Alzheimer’s disease

Stavrovskaya A.V., Yamshchikova N.G., Ольшанский A.S., Babkin G.A., Illarioshkin S.N.

Abstract

Development of new approaches to the treatment of Alzheimer’s disease (AD) is an actual problem of modern neurology owing to high prevalence of AD in the population and severe irreversible disability resulting from this disease. We investigated the effects of new peptide compounds, β-casomorphin-7 and colostrinin, in two complementary AD models in rats (with injection of β-amyloid into the nucleus basalis of Meynert and injection of streptozocin in the ventricles of the brain). Peptides were injected intranasally within 10 days after the onset of AD symptoms induced by neurotoxins. Injection of β-casomorphin-7 and colostrinin had similar effect on the behavior and cognitive function of rats with both amyloid and streptozocin AD model: there was statistically significant increase in locomotor activity and orienting responses, as well as improvement of animals’ cognitive functions. Colostrinin had stronger positive effect on the behavior of rats with induced AD, whereas β-casomorphin-7 had an effect on both sham-operated and model animals, which is indicative of the general neurotropic potential of this peptide. The proposed experimental approaches improve the capabilitiesof investigation of novel biologically active compounds aimed at prevention and treatment of neurodegenerative diseases.
Annals of Clinical and Experimental Neurology. 2016;10(2):
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Microelectrode recording of neuronal activity in the surgery for Parkinson’s disease

Nizametdinova D.M., Tyurnikov V.M., Fedorenko I.I., Gushcha A.O., Illarioshkin S.N.

Abstract

Microelectrode recording of neuronal activity is a modern and safe tool for neurophysiological mapping of subcortical brain structures that serve as targets for stereotactic functional neurosurgery. The article discusses the main technical and clinical aspects of this method and focuses on Parkinson’s disease, a classical object of functional neurosurgery. Microelectrode analysis improves the accuracy of electrode positioning and the effectiveness of surgical neuromodulation in Parkinson’s disease, enables investigation of the pathophysiological features of extrapyramidal disorders, mechanisms of action of drugs and various functional neurosurgery techniques, and also facilitates the search for new potential targets for deep brain stimulation.
Annals of Clinical and Experimental Neurology. 2016;10(2):
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Adult-onset leukoencephalopathy with vanishing white matter

Rudenskaya G.E., Zakharova E.Y.

Abstract

Leukoencephalopathy with vanishing white matter (LEVWM) is one of the most common hereditary leukoencephalopathies with characteristic MRI picture of diffuse white matter lesions with cystic degeneration. The disease is associated with EIF2B1-5 genes, encoding five subunits of EIF2B translation initiation factor. There are infantile, children’s (the most frequent one), and adult-onset forms. Adult-onset LE-VWM accounts for 15—20% of all cases and is characterized by significant clinical variability. In addition to neurological and cognitive disorders, this disease is characterized by ovarian failure. The review presents the clinical and molecular genetic aspects of adult-onset LE-VWM.
Annals of Clinical and Experimental Neurology. 2016;10(2):
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DYT6 form of idiopathic dystonia

Krasnov M.Y., Timerbaeva S.L., Abramycheva N.Y., Stepanova M.S., Shpilyukova Y.A., Ershova M.V.

Abstract

DYT6 is a recently described autosomal dominant form of primary dystonia with early onset of symptoms caused by mutations in THAP1 gene in chromosome 8. The incidence of this form in various populations is extremely variable and ranges from 1% to 25%. Knowledge of the molecular defect underlying the disease largely determines its prognosis and treatment approaches. The article presents the first in the Russian population case of DYT6 dystonia, which was confirmed by detection of c.424A> G (p.T142A) mutation in THAP1 gene. Clinical presentation included acute manifestation of symptoms at the age of 27 years with the development of left-directed latero-retrocollis. The incidence of this form of dystonia in our population of dystonic syndromes was 0.7%. We emphasize phenotypic polymorphism of DYT6 dystonia and the role of genetic testing in its diagnosis.
Annals of Clinical and Experimental Neurology. 2016;10(2):
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