Vol 13, No 1 (2019)

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Full Issue

Original articles

Intravenous thrombolysis in ischemic stroke: clinical predictors of efficacy and safety

Domashenko M.A., Maksimova M.Y., Tanashyan M.M.


Introduction. Systemic thrombolysis with recombinant tissue plasminogen activator is the “gold standard” of reperfusion therapy, having the maximum level of evidence in European and North American guidelines for the treatment of patients with acute ischemic stroke (IS).
Objective: to determine factors of individual efficacy and safety of systemic thrombolysis in patients with IS aiming to establish personalized approach to its optimization.
Materials and methods. The study included 396 patients with IS, of whom 196 patients underwent systemic thrombolysis with recombinant tissue plasminogen activator and 200 patients formed the control group. A prospective non-randomized study was conducted in parallel groups to estimate efficacy and safety of systemic thrombolysis with recombinant tissue plasminogen activator. The primary endpoint of the study was functional independence of patients measured with a modified Rankin scale 3 months after the stroke.

Results. We confirmed improvement of the functional outcome in patients with IS who underwent systemic thrombolysis. We also showed no effect of thrombolytic therapy on the mortality rates. Decreased wakefulness, presence of aphasia, hemiplegia, congestive heart failure, type 2 diabetes, postinfarction cardiosclerosis were shown to be clinical factors associated with an unfavorable prognosis of the disease after systemic thrombolysis. We found higher likelihood of death in patients with decreased level of consciousness (OR 3.1 (1.1–8.8); p=0.03), as well as with paresis (OR 6.8 (2.2–20.9); p<0.001), hemiplegia (OR 6.5 (2.0–21.4); p=0.002), and chronic heart failure (OR 2.4 (1.1–5.3); p=0.03).

Conclusion. Adequate analysis of neurological symptoms and clinical and anamnestic data upon admission of a patient with IS allows to predict the effectiveness of thrombolysis and may be important in treatment planning and in choosing reperfusion methods.

Annals of Clinical and Experimental Neurology. 2019;13(1):5-14
pages 5-14 views

Myotonic dystrophy: genetics and clinical polymorphism

Ivanova E.O., Moskalenko A.N., Fedotova E.Y., Kurbatov S.A., Illarioshkin S.N.


Myotonic dystrophy is the most common form of hereditary progressive muscular dystrophy in adults. The disorder is characterized by progressive course, autosomal
dominant inheritance and multisystem involvement (skeletal muscles, myocardium, endocrine system, eyes, etc.). The paper highlights a huge heterogeneity of
clinical manifestations of myotonic type 1 and type 2, and reviews genetic aspects and current approaches to the diagnosis of myotonic dystrophy. We present our
own clinical observation of myotonic dystrophy in a family, which demonstrates a rare combination of a classical form of myotonic dystrophy type 1, syringomyelia
and proximal muscle weakness, and provides an classical example of the phenomenon of anticipation.

Annals of Clinical and Experimental Neurology. 2019;13(1):15-25
pages 15-25 views

Cervical arthroplasty: 5-year follow-up

Guscha A.O., Dreval M.D., Petrosyan D.V., Arestov S.O., Kascheyev A.A., Vershinin A.V., Poltorako Y.N.


Research objective: analysis of the early and long-term outcomes of cervical intervertebral disc prosthetics, assessment of the amount of movement and of the regression of radicular symptoms within 5 years.

Materials and methods. Thirty patients (19 women and 11 men, average age 35.8 years) were operated on for a single-level degenerative lesion of the intervertebral disk. The amount of movement was assessed based on functional X-ray data. Regression of radicular pain symptoms was assessed using visual analogue scale (VAS).

Results. Good and excellent results were obtained. In the long-term period (60 months), local cervical pain syndrome ranged from 0 to 3 VAS points, with initial estimation at 6–8 points. Radicular pain was estimated at 0–2 VAS points, initially 5–8 points.

Conclusion. Arthroplasty is a highly effective method for the treatment of degenerative lesions of cervical intervertebral discs. The technique can definitely compete with rigid stabilization systems and proves its efficacy in treatment of the disease of the adjacent segment.

Annals of Clinical and Experimental Neurology. 2019;13(1):26-30
pages 26-30 views

Current situation of diagnostics and management of patients with primary headaches in the Republic of Armenia

Vekilyan H.R., Sahakyan A.E., Manvelyan H.M.


Introduction. Headache is one of the most common complaints of patients seeking for medical care. Headaches affect people of all ages, races, income levels, and geographical areas. They form a substantial burden on personal and social lives of patients, diminish their quality of life, and cause financial losses. The problem of headaches (primary ones, in particular) has not been previously studied in Armenia. We aimed to focus on several types of primary headaches (tension-type headache, migraine, trigeminal autonomic cephalalgias (particularly, cluster headaches). The prevalence of headaches in the adult population is 47%, however, many people do not receive proper treatment.

Objective of the study was to investigate the prevalence of different types of primary headaches (tension type, migraine, trigeminal autonomic cephalalgias), and assess quality of life of patients with headaches who sought for medical care.

Materials and methods. According to study inclusion and exclusion criteria, 150 patients with headache, who visited the Neurology Department of YSMU during the past 4 years, were selected for further analysis. All patients were asked to complete three questionnaires: (1) A questionnaire which was compiled by us based on diagnostic criteria of International Headache Society (2-nd edition and 3-rd edition beta versions), (2) Headache Impact Test, and (3) SF-36. All studied patients were examined by a neurologist.

Results. In the analyzed population, tension-type headache was found in 31% of patients, migraine — in 60% of patients, and trigeminal autonomic cephalalgias — in 9% of participants. More than the half of the analyzed patients have seen physicians before (75% of them were examined by a neurologist), and in 37% of cases there were multiple referrals to a physician.

Conclusion. Data analysis showed significant underestimation (both by patients and physicians) and misdiagnosis of primary headaches in our region, especially in respect to cluster headache. That leads to chronification of headaches, ineffective treatment, and development of medication-overuse headaches. It is necessary to increase public awareness about the possibilities of headache treatment, as well as to increase the competence of the medical specialists.

Annals of Clinical and Experimental Neurology. 2019;13(1):31-35
pages 31-35 views

Daily profile of arterial pressure and brain microstructural changes in patients with hypertension-related cerebral small vessel disease

Dobrynina L.A., Shamtieva K.V., Kremneva Е.I., Kalashnikova L.A., Krotenkova M.V., Gnedovskaya E.V., Berdalin A.B.


Introduction. Application of modern antihypertensive medications has improved the course of arterial hypertension (AH), but has not led to the expected decrease
in small vessel disease (SVD) incidence and its complications. This fact encourages further investigation of hypertension-related mechanisms of brain damage.
Objective: to study the relation between daily profile of blood pressure and brain microstructure changes in patients with both SVD and AH.
Material and methods. The study included 64 patients (38 – 59.4%) female, mean age 59.4±5.4 years) with both SVD and AH. Ambulatory blood pressure
monitoring and diffusion-tensor MRI were obtained for all participants. The relation between studied parameters was estimated using the multivariate statistical
analysis method – linear regression analysis.
Results. Сhanges in daily profile of blood pressure according to ambulatory blood pressure monitoring data was associated with microstructure abnormalities of
the juxtacortical white matter hyperintensities (jWMH) of anterior frontal lobes, temporal-parietal regions and left posterior cingulate cortex. An increase and
variability of diastolic blood pressure were of primary importance in brain microstructural damage in mentioned areas leading to mean diffusivity and radial
diffusivity increase.
Conclusion. The revealed relation between daily profile of blood pressure and brain microstructural changes indicating increase of free water diffusivity and
myelin damage in jWMH and posterior cingulate cortex corresponds to the experimental data on the breakdown of the autoregulation reaction in cortex arteries
and further increase of brain-blood barrier permeability with descending vasogenic edema in brain damage in hypertensive patients. An increase and variability of
diastolic blood pressure have primary importance to microstructural damage of white matter in patients with SVD receiving antihypertensive treatment.

Annals of Clinical and Experimental Neurology. 2019;13(1):36-46
pages 36-46 views

Integrative functions of the retrosplenial cortex in rats: anatomy, connectomics, and cellular electrophysiology

Mineeva O.A., Bezryadnov D.V., Chekhov S.A., Svarnik О.E., Anokhin K.V.


Current review is focused on the integrative functions of the retrosplenial cortex, which neurons are largely involved in spatial orientation and ambulation of an organism. We discuss anatomy and connectivity of the retrosplenial cortex in rats as well as the most recent findings concerning the behavioral specialization of its neurons observed using multielectrode recordings. Pattern of connections of the retrosplenial cortex allows to consider its interfacing role in linking brain regions specifically involved in spatial navigation and memory with areas of the associative cortex which lack spatial tuning. In this paper, we touch upon that unique anatomical connectivity which is reflected in the peculiar behavioral specialization of the retrosplenial cortex neurons. Complex spatial tuning of retrosplenial neurons is likely to represent the association of spatial and nonspatial information, and provides a clue to principles of information integration in the cerebral cortex.

Annals of Clinical and Experimental Neurology. 2019;13(1):47-54
pages 47-54 views


Current view on phenotypic and genetic features of autosomal recessive inherited peripheral neuropathies

Murtazina A.F., Shchagina O.A., Nikitin S.S., Dadali E.L., Polyakov A.V.


Inherited peripheral neuropathies (IPNs) are a heterogeneous group of hereditary motor and sensory neuropathies (HMSN), hereditary motor neuropathies, and hereditary sensory neuropathies. IPNs can be inherited in autosomal dominant, autosomal recessive or X-linked manner. In clinical practice, isolated cases are more common, and the absence of genealogical data significantly complicates differential diagnosis. About 45% of HMSN cases lack genetic confirmation.

For a number of autosomal recessive IPNs, peculiar clinical, electrophysiological and histological features can be distinguished, however, recent publications show IPNs often to have pronounced clinical and genetic heterogeneity. That complicates the differential diagnosis of hereditary neuropathies. Prevalence of autosomal recessive pathology increases in remote populations and isolates due to a local founder effects. As the literature review showed, there are many such examples among autosomal recessive IPNs. Detection of the founder effect and major mutations in the population and development of diagnostic algorithms based on these data can significantly improve the diagnostic process.

Annals of Clinical and Experimental Neurology. 2019;13(1):55-69
pages 55-69 views


Current neurosurgical approaches to treatment of structural epilepsies

Sitnikov A.R., Skorobogatova V.A., Maslova N.N.


The drug-resistant epilepsy remains one of the major problems in contemporary neurology. However, the new surgical approaches showed their great efficacy in treatment of patients with intractable seizures. The relevant neurosurgical methods of epilepsy treatment, as well as the issues of preoperative diagnostics and indications for operative treatment are described in this review. We also present a clinical case which demonstrates the successful treatment of patient with drug-resistant epilepsy who underwent stereotaxic implantation of electrodes in the anterior thalamic nuclei followed by significant clinical improvement.

Annals of Clinical and Experimental Neurology. 2019;13(1):70-77
pages 70-77 views

Clinical analysis

Difficulties of clinical diagnosis in primary progressive aphasia. Clinical observation

Barantsevich Y.P., Koval’chuk Y.P., Mel’nik Y.V., Emanuel’ V.S., Emanuel’ Y.V.


Primary progressive aphasia is a syndrome characterized by progressive speech dysfunction. There are three types of this condition. The first — agrammatic — type of primary progressive aphasia is typical for frontotemporal dementia and characterized by progressive worsening of speech during several years without other neurological symptoms. For this type, alexia is typical (can be present before the onset of other clinical symptoms) along with agraphia and oral apraxia.

Second type of primary progressive aphasia, semantic aphasia, may also be present in frontotemporal dementia.

The third type of primary progressive aphasia is a logopenic variant which is a manifestation of the atypical variant of Alzheimer’s disease. In this type, the speech disorders are prevailing over the mnestic manifestations. Differential diagnosis in that case is difficult given lack of specific markers in patients with Alzheimer’s disease.

As a clinical illustration, we present a case of a 50-year-old patient. She has been considering herself ill from the age of 45 years, when her speech impairment emerged and started to progress; she also started to experience difficulties in recalling and correct pronunciation of the words. No significant pathologies in internal organs were identified. Her neurological examination showed pyramidal insufficiency more on the right, slight muscle rigidity in the limbs, acheirokinesis, and mild ataxia. Examination of patient’s higher cerebral functions showed mixed aphasia (amnestic and motor), frequent agrammatisms, paraphasias, anomias, apraxia more in the left arm, simultaneous agnosia, partial hemispatial neglect of the left side, alexia, agraphia, acalculia, and finger agnosia. Frontal Assessment Battery test score was 8 and Mini Mental State Examination score was 16. Vascular, infectious, metabolic, autoimmune, tumorous, and iatrogenic causes of dementia have been ruled out. Speech disturbances were typical for agrammatic variant of primary progressive aphasia, but didn’t contradict with logopenic type of Alzheimer’s disease. The PET scan showed no hemispheric asymmetry. We suggested that the most probable diagnosis could be primary progressive aphasia, which can be a manifestation of both frontotemporal dementia and Alzheimer’s disease. Moreover, the differential diagnosis based on clinical manifestations only, without specific PET investigation or specific CSF study is rather difficult.

Annals of Clinical and Experimental Neurology. 2019;13(1):78-83
pages 78-83 views

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