[A model for assessing and correcting the risk of developing pressure ulcers in the system of long-term care for geriatric patients with limited mobility.]

The article is devoted to the results of the third stage of the scientific and practical project on the introduction of advanced social practices based on innovative gerontotechnologies into the system of long-term care for geriatric patients. It has been established that senile xerosis or skin «frailty¼ occurs in 75% of the population over 65 years old, when increased dryness of the skin leads to peeling, the formation of microcracks, the appearance of senile itching, sleep disturbances, the development of pressure ulcers and, as a result, a decrease in motor activity. During the project, a tactic was developed for the care of «frail¼ skin in patients with senile xerosis and the risk of pressure ulcers. The article presents information about the current problems of diagnosis, correction and prevention of pressure ulcers in patients with limited mobility of elderly and senile age. The results of a comparative analysis of the severity of pressure ulcers and their impact on the quality of life in the system of long-term care for geriatric patients with limited mobility before and after the use of Cicalfate+ Eau Thermale Avène («Pierre Fabre¼, France) emollient were presented. A model was built and practical recommendations were given on the use of the most informative and adequate scales for predicting and assessing the risk of developing pressure ulcers in patients of older age groups.

[Current problems of polypharmacy in geriatric patients when taking drugs with a risk of photosensitivity.]

The article presents an overview of the current problems of polypharmacy in geriatric patients when taking drugs with a risk of photosensitivity. The article contains information about emerging adverse drug reactions, as well as methods for diagnosing, correcting and preventing phototoxic and photoallergic reactions in patients of older age groups. The main aspects of dermatological support in the system of long-term care for geriatric patients when taking drugs with a risk of photosensitivity are outlined. Clinical signs of senile xerosis and skin manifestations of adverse drug reactions were studied when taking drugs with the risk of photosensitization before and after the use of a photoprotector in elderly patients.

[Implementation of advanced social practices based on innovative gerontotechnologies in the system of long-term care for elderly and senile patients.]

From 2017 to the present a scientific project «The use of dermatoprotectors to improve the quality of life of geriatric patients with age-associated xerosis (AAX)¼ has been realized as part of a typical model of a long-term care system in order to introduce the innovative gerontotechnologies as advanced social practices in further optimization of social and medical care for elderly and senile patients in Leningrad Region. The article presents the results of a comparative analysis of the effectiveness of the dermatoprotectors application in the improvement of the quality of life, prevention and correction of AAX. The AAX clinical manifestations have been studied before and after the emollients use. The cohort under study has included geriatric patients with senile asthenia from seven institutions of social protection of the Leningrad Region. In the foreign literature, such patients are called «fragile¼. The data thus obtained indicate that AAX as a manifestation of skin «fragility¼ during aging can be considered as a component of the geriatric syndrome of senile asthenia in patients of older age groups. The development of skin «fragility¼ significantly reduces the quality of life of geriatric patients and requires timely prevention and correction with adequately selected dermatoprotectors.

Ambroxol increases glucocerebrosidase (GCase) activity and restores GCase translocation in primary patient-derived macrophages in Gaucher disease and Parkinsonism.

Mutations in the glucocerebrosidase gene (GBA) encoding the lysosomal enzyme glucocerebrosidase (GCase) cause Gaucher disease (GD) and are the most commonly known genetic risk factor for Parkinson disease (PD). Ambroxol is one of the most effective pharmacological chaperones of GCase. Fourteen GD patients, six PD patients with mutations in the GBA gene (GBA-PD), and thirty controls were enrolled. GCase activity and hexosylsphingosine (HexSph) concentration were measured in dried blood and macrophage spots using liquid chromatography coupled with tandem mass spectrometry. The effect of ambroxol on GCase translocation to lysosomes was assessed using confocal microscopy. The results showed that ambroxol treatment significantly increased GCase activity in cultured macrophages derived from patient blood monocytic cell (PBMC) of GD (by 3.3-fold) and GBA-PD patients (by 3.5-fold) compared to untreated cells (p < 0.0001 and p < 0.0001, respectively) four days after cultivation. Ambroxol treatment significantly reduced HexSph concentration in GD (by 2.1-fold) and GBA-PD patients (by 1.6-fold) (p < 0.0001 and p < 0.0001, respectively). GD macrophage treatment resulted in increased GCase level and increased enzyme colocalization with the lysosomal marker LAMP2. The possible binding modes of ambroxol to mutant GCase carrying N370S amino acid substitution at pH 4.7 were examined using molecular docking and molecular dynamics simulations. The ambroxol position characterized by minimal binding free energy was observed in close vicinity to the residue, at position 370. Taken together, these data showed that PBMC-derived macrophages could be used for assessing ambroxol therapy response for GD patients and also for GBA-PD patients.

Genetics variants and expression of the SCARB2 gene in the pathogenesis of Parkinson's disease in Russia.

Lysosomal integral membrane protein-2 (LIMP-2), encoded by the SCARB2 gene, is the specific lysosomal receptor for glucocerebrosidase enzyme. Association between rs6812193 and rs68250047 of SCARB2 with PD has been shown in genetic studies, including large genome-wide association studies. The aim of the current study was to determine whether rs6812193 and rs8475 are associated with PD in Russia. rs6812193 and rs8475 were genotyped in a total of 604 PD patients (65 PD patients with positive (fPD) and 539 PD patients with negative family history (sPD)) and 413 controls and also in 17 patients with PD associated with GBA mutations (PD-GBA) and 18 asymptomatic GBA mutation carriers (GBA-Carriers). SCARB2 expression was measured by real-time PCR in CD45+ blood cells in part of individuals in the studied groups. No linkage disequilibrium was shown between rs6812193 and rs8475 in Russian population. Increased PD risk for TT variant of rs8475 (OR = 2.02; p < 0.001) was found in sPD patients but not in fPD. rs6812193 and rs8475 were not associated with age at onset (AAO) of PD. SCARB2 expression level was decreased in GBA-PD patients and GBA-Carriers compared to PD patients (padjusted = 0.02, padjusted = 0.003, respectively) and GBA-Carriers compared to controls (padjusted = 0.013) with no significant difference in PD patients and controls. SCARB2 expression was not modified with rs6812193 and rs8475. In conclusion, rs8475 was associated with PD status. rs6812193 and rs8475 are not genetic modifier of AAO of PD and do not influence on SCARB2 mRNA level in CD45+ blood cells in studied groups. SCARB2 expression could be modified with GBA mutations and is independent of PD status.

[Efficacy and safety of neuroprotection in patients with primary open-angle glaucoma]. / Issledovanie éffektivnosti i bezopasnosti neiroprotektsii pri pervichnoi otkrytougol'noi glaukome.

Elevated intraocular pressure (IOP) is considered one of the main factors in the development and progression of primary open-angle glaucoma (POAG). The main schemes of drug treatment and cell protection from glaucomatous damage are aimed particularly at reducing IOP. However, it is possible for the disease to progress with normalized IOP. This determines the need to search for drugs that would affect other pathogenetic links of the progression of POAG. PURPOSE: To study the effect of 'Cytoflavin' drug on the stabilization of glaucomatous optic neuropathy in patients with POAG. MATERIAL AND METHODS: The study included 103 patients with initial or developed POAG. Patients were randomized into two groups using random numbers: the control group patients (n=50) receiving standard conservative treatment, and the main group (n=53), where patients received the combined drug with metabolic action 'Cytoflavin'. In addition to standard ophthalmological studies, all patients underwent biological, electrophysiological, perimetric, structural topographic, laboratory and sociological examinations before and after the treatment. RESULTS: By the end of the 3rd month of the treatment, the patients of the main group showed positive dynamics of the amplitude and phase characteristics of bioelectric activity of the retina, improvement in the total sensitivity of the retina for central field of view. Cytoflavin contributed to reducing the rate of progression of glaucoma according to optical coherence tomography: stabilization of the thickness of the nerve fiber layer, reduction of the rate of global and focal loss of retinal ganglion cells. In patients of the main group, by the end of the 3rd month, the severity of oxidative stress has significantly reduced and the antioxidant defense improved. On the background of stabilized glaucomatous process, a subjective improvement in general and psycho-emotional health, increased confidence in the success of the therapy and thus the improved the quality of life was observed in comorbid patients of the main group. CONCLUSION: Cytoflavin acts on the mechanisms that enhance the protection of the retina against the effects of reperfusion injury and oxidative stress, contributes to the metabolic adaptation of neurons and leads to a more favorable variant of POAG.

[Quality of life and physical activity among elderly and old people.]

This article presents the results of the identification of the main causes of physical inactivity among the elderly and the assessment of the effectiveness of the inclusion of regular Nordic walking into the preventive system for the elderly in order to improve the quality of life. A comparative analysis of the quality of life was used among elderly and old people, regularly engaged in Nordic walking (intervention group) and leading a physically passive lifestyle (control group). It was established that regular physical activity allows the improvement of the quality of life and positively affects health in groups under study, but it is the elderly people who represent the most physically inactive part of the population. The main reasons for the physical inactivity among older people are the presence of diseases, fear of injury and falls, lack of energy and weakness, low level of motivation, lack of partners or friends for joint activities, etc. The above studies have shown that regular practice of Nordic walking improves the quality of life for both elderly and old people. It was evidenced by the results of the SF 36 questionnaire analysis when we compared not only intervention and control groups but the age differences in the quality of life in all groups under study. After 12 months of regular Nordic walking it was registered an increase of the quality of life estimated on the scales of role-playing physical functioning, overall health, role emotional functioning, vitality, psychological health, social functioning, which correlated both with the physical and psychological components of health. The results thus obtained make it possible to recommend Nordic walking as the most simple, accessible and at the same time effective type of physical activity for the elderly population, that helps to overcome the main causes of physical inactivity, restore the functional capabilities of the aging organism. Gerontotechnologies based on the regular activities of Nordic walking are one of the most effective ways to improve and maintain the quality of life and, as a result, the activity, mobility, self-care ability among elderly and old people.

Reduced habituation of trigeminal reflexes in patients with episodic cluster headache during cluster period.

A growing body of evidence supports the pivotal role of the hypothalamus in the pathophysiology of cluster headache (CH). On the basis of animal studies, it has been suggested that a hypothalamic dysfunction can lead to a habituation deficit of brainstem reflex responses, as result of a stress-like condition. Taking into account these findings, we tested the hypothesis that habituation of brainstem reflexes may be impaired in CH patients. The habituation phenomenon of the late components (R2 and R3) of the blink reflex was studied in 27 CH patients during the cluster period, in 22 migraine patients interictally and in 20 control subjects. A significant habituation deficit in the R2 and R3 components was found in CH compared with both controls and migraineurs. The lack of habituation in CH, more pronounced than in migraine, points to abnormal processing of sensory stimuli at the trigeminal level that could be driven by hypothalamic dysfunction during the cluster period.

Trigemino-cervical reflex in patients with headache.

Neurophysiological studies have shown abnormal activity of some brainstem nuclei in headache patients. The trigemino-cervical reflex is an anti-nociceptive reflex that gives an opportunity for evaluation of the brainstem interneurone activity. It has not been previously examined in headache patients. We studied 15 patients with predominantly unilateral chronic tension-type headache, 15 patients with migraine without aura and 32 healthy subjects. The trigemino-cervical reflex was recorded bilaterally from the resting sternocleidomastoid muscle using surface electromyographic recordings. In all headache patients the trigemino-cervical reflex on the painful side was with shortened latency compared with the non-painful side and with healthy persons. The results suggest decreased activity of the brainstem inhibitory interneurones. We suggest that although the pathophysiological mechanisms of tension-type headache and migraine are different, they share common mechanisms of abnormal pain control.

The trigemino-cervical reflex in normal subjects.

There are only few reports on the trigemino-cervical reflex in humans and there is debate over the best method of reflex examination. The aim of this study was, comparing different methods, to provide a reproducible method for evaluating the trigemino-cervical reflex. The trigemino-cervical reflex was studied in 32 healthy volunteers. The stimulation was applied to the supraorbital, infraorbital or mental nerve. Recordings were performed bilaterally from the sternocleidomastoid and trapezius muscles at rest. The reflex was also examined during maximal voluntary contraction of the sternocleidomastoid muscle after supraorbital nerve stimulation. It presented as a two-component reflex if recorded from a tonically active muscle and as a one-component reflex if recorded from a relaxed muscle. The most reproducible reflex responses were obtained from the resting sternocleidomastoid muscle after stimulation of the supraorbital nerve. In conclusion, the trigemino-cervical reflex may be most easily obtained from the relaxed sternocleidomastoid muscle after supraorbital nerve stimulation.

Antiphospholipid syndrome and dystonia-parkinsonism. A case report.

Background: Although clinically evident and MRI confirmed, basal ganglia involvement, is usual in primary antiphospholipid syndrome, extrapyramidal disorders such as parkinsonism and dystonia are very rare. We were unable to find any report in the literature on dystonia-parkinsonism in patients with primary antiphospholipid syndrome. Here we report an adult patient with dystonia-parkinsonism and primary antiphospholipid syndrome.Case report: A 60 year old, right-handed man came to our attention due to writer's cramp, bradykinesia and stiffness of his right hand. Neurological examination revealed constant, marked dystonic posturing, rigidity and bradykinesia of the right hand. Hyper-gammaglobulinemia was demonstrated on electrophoresis-serum IgG was increased. Anticardiolipin antibodies were examined by counterimmunoelectrophoresis (ELISA): IgG was negative, while IgM was positive. There was also slight thrombocytopenia. Magnetic resonance imaging brain scan axial T2W/UTSE revealed several hyperintense lesions in the basal ganglia and in the periventricular white matter and diffuse hyperintensity of the subcortical white matter bilaterally in the parietal regions. There was asymmetric parenchimal atrophy, more prominent in the left hemisphere. No clinical improvement was achieved by levodopa, dopamine agonists or anticholinergics. According to the criteria for primary antiphospholipid syndrome our patient had thrombocytopenia and high levels of IgG and IgM anticardiolipin antibodies so he was presumed to have a primary antiphospholipid syndrome.Conclusion: Various movement disorders may appear secondary to stroke, antiphospholipid syndrome, Behcet's disease or brain tumor. These cases may help in the understanding of pathophysiology of movement disorders. Dystonia and parkinsonism as well as other movement disorders may be associated with primary antiphospholipid syndrome.

Parkinsonian syndrome as a neurological manifestation of Behçet's disease.

BACKGROUND: The central nervous system is often involved in Behcet's disease. Most common are meningoencephalitic and brain stem syndromes. Although basal ganglia involvement is not an uncommon finding on necropsy, there are only single reports on extrapyramidal syndromes-dyskinesia, chorea and Parkinsonism in patients with Behcet's disease. CASE STUDY: We report a patient fulfilling the criteria of the International Study Group for Behcet's disease. He had recurrent oral ulcerations, bilateral posterior uveitis and retinal vasculitis, skin papules and pustules, and recurrent monoarthritis. Neurologic examination revealed pseudobulbar palsy, slight and asymmetric bilateral pyramidal syndrome, muscle rigidity involving the four limbs, bradykinesia, masked face, and impaired postural reflexes. There was postural tremor in the extremities and myoclonic jerks involving the tongue and face muscles. Magnetic resonance imaging demonstrated small bilateral multifocal hyperintense lesions, with right predilection, involving the periventricular white matter, brain stem and basal ganglia. CONCLUSIONS: The Parkinsonian syndrome found in our patient might be due to involvement of both substantia nigra and basal ganglia. This case further emphasizes the wide spectrum of the neurological manifestations of Behcet's disease.

Contralateral hemi-parkinsonism and atypical tremor after stroke (a case report).

Extrapyraramidal parkinsonian-like syndromes have been observed as manifestations of different central nervous system lesions. A 45-year-old-man gradually developed tremor and slowing of movements 2 years after a stroke. Neurological examination revealed slight hemiparesis, muscle rigidity and bradykinesia in the left limbs. A tendency for hypomimia and impaired postural reflexes were found. There were resting, more pronounced postural and kinetic and most pronounced intentional tremors involving the left limbs. On electromyographic examination the static tremor was of 4Hz frequency, while the postural, kinetic and intention tremors were of 7Hz frequency. All tremors were with alternating activity in antagonist muscles. In conclusion our findings confirm the common anatomical basis of parkinsonism and essential like tremor. Both can appear after disruption of the pathways within and adjacent to the basal ganglia.