REVIEWS
Progressive external ophthalmoplegia (PEO) is characterized by progressive bilateral ptosis and eye movement disturbances in horizontal and vertical directions and represents a typical symptom of many mitochondrial disorders, but not a separate disease. PEO often occurs together with other systemic manifestations of mitochondrial disfunction. Correct and early diagnosis of PEO is essential for optimal management of these patients. In this review we collected the last data about etiology, spectrum of clinical symptoms, differential diagnosis, diagnostical methods and treatment options for isolated chronic PEO and other PEO-associated mitochondrial syndromes.
Cognitive and behavioral factors are of great importance in the development of chronic insomnia, which explains the effectiveness of cognitive-behavioral therapy (CBT). CBT can be used both in combination with drug therapy and without it. This review gathers the existing information about conducting CBT, its various methods and effectiveness in comparison with drug therapy. CBT allows correcting erroneous thoughts (cognitive errors) and behavioral patterns that adversely affect the quality and duration of sleep. The issues of optimizing the use of CBT in clinical practice are discussed.
CLINICAL RESEARCH AND SURVEILLANCE
The purpose of the work is to study the state of the elastic properties of the common carotid artery wall (CCA) in patients with dissection of the internal carotid artery (ICA) and/or vertebral (VA) artery by means of the ultrasonography research.
Materials and methods 51 patients were examined (average age 38,4 ± 7,1 years old, 69% of them were women, undergone dissection of the ICA and/or VA. Control group: 51 healthy volunteers. Ultrasonography research with the assessment of the elastic properties of CCA (distensibility coefficient, arterial stiffness coefficient, Young’s modulus) was performed for all patients and healthy individuals.
Results. In patients with dissection, compared to the control group, the following characteristics were revealed: a decrease in CCA distensibility coefficient (23,08 [19,6; 26,2] 10–3 kPa–1 vs 29,6 [27,4; 31,7] 10–3 kPa–1, p < 0,0005), an increase in stiffness index β (6,5 [5,8; 7,4] vs 5,4 [4,9; 5,7], p < 0,0005), an increase in Young’s modulus, characterizing the elastic properties of the arterial wall’s material (49,9 [36,5; 56,4] kPa/mm vs 35,4 [30,0; 41,3] kPa/mm, p < 0,0004). It was higher in patients with FA, i.e. functional apoplexy (56 [38,9; 66,1] kPa/mm), compaired to patients with isolated neck pain/ headache (42,6 [36,3; 56,8] kPa/mm, p = 0,019); it increased with age (R = 0,352, p = 0,011) and due to the increase in cholesterol level (R = 0,330, p = 0,019). In patients with arterial hypotension, Young’s modulus was lower than that of normotonics (38,2 [32,5; 51,2] kPa/mm against (48,9 [42,5; 60,5] kPa/mm (p = 0,021). The stiffness index β was higher in patients examined during the first 3 months of the disease (7,1 [6,3; 7,6]) than in those examined later — (6,2 [5,4; 6,6], p = 0,036).
Conclusion. Change in biomechanical properties of the CCA wall in patients, undergone the ICA/VA dissection, indicates its increased rigidity. The presence of increased rigidity in the ICA/VA wall is also assumed, which predisposes to their dissection. Revealed correlations between parameters of biomechanical properties of CCA and clinical data explain some features of the clinical manifestations of dissection and its course.
Cerebral amyloid angiopathy (CAA) is a common disease in the older age group with high clinical and neurovisual heterogeneity. A literature review and two clinical cases reflecting different phenotypes of CAA are presented — amyloid angiitis (CAA-related inflammation) and “hemorrhagic phenotype” (CAA associated with intracerebral hemorrhage and progressive cortical superficial siderosis). In each case, in relation to the clinical and neuroimaging picture, the directions of the disputed vectors of secondary prevention and therapy of CAA are considered: antithrombotic, antihypertensive and lipid-lowering therapy. The issues of epidemiology, pathogenesis, diagnosis and treatment of CAA are discussed.
Multiple systemic atrophy.(MSA) is a progressive neurodegenerative disease with a poor prognosis and unknown etiology, which belongs to the “atypical parkinsonism” group of diseases. Currently, the diagnosis of MSA is difficult and at the present stage remains predominantly clinical. But, despite the fact that no effective treatment has been developed, timely diagnosis and symptomatic therapy can improve the patient’s quality of life. The article presents a clinical observation of a patient with MSA, a feature of which was the combination of MSA with vascular pathology, which greatly complicated and delayed the diagnosis of MSA.
FROM HISTORY OF NEUROLOGY
The article provides a brief outline on the history of the first clinic of the nervous system diseases in Russia that was organized in 1969 in Moscow New Catherine hospital, and also presents information about its leaders — A.Ya. Kozhevnikov, G.I. Rossolimo, A.A. Kornilov, L.O. Darkshevitch, I.Yu. Tarasevitch.
YUBILEE
ISSN 2686-7192 (Online)