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Neuromuscular Diseases

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Vol 8, No 4 (2018)
View or download the full issue PDF (Russian)
https://doi.org/10.17650/2222-8721-2018-8-4

LECTURES AND REVIEWS

12-18 2850
Abstract

Amyotrophic lateral sclerosis is a neurodegenerative disease, resulting in the loss of self-service and death of the middle-aged and elderly people. In the last 2 decades, significant progress has been made in the study of the pathogenesis of this disease. Two known drugs (riluzole and edaravone) have been approved by the Food and Drug Administration for treatment of amyotrophic lateral sclerosis. The efficacy of these drugs is extremely low, so clinical trials of new drugs are ongoing all over the world. This review discusses the current achievements and future directions of therapy of this disease.

ORIGINAL REPORTS

35-42 1840
Abstract

Background. Patients with the chronic migraine frequently present with memory and attention complaints. However, the prevalence and phenotype of such impairment in chronic migraine have not been studied.

Objective – to evaluate the prevalence of the objective cognitive deficit in patients with chronic migraine and factors underlying its etiology.

 Materials and methods. We recruited 62 subjects with chronic migraine and 36 genderand age-matched controls with low-frequency episodic migraine (not more, then 4 headache days per month) aged 18–59. All patients filled in the Hospital Anxiety and Depression Scale (HADS) and Sheehan Disability Scale. Cognitive function was assessed with the Montreal Cognitive Assessment (MoCA), Digital Symbol Substitution Test (DSST), Rey Auditory Verbal Learning Test (RAVLT), and the Perceived Deficits Questionnaire (PDQ-20).

Results. In this study 58 % of patients with chronic migraine complained of memory loss. Cognitive impairment was also found with PDQ-20. Objectively, we found a significant decrease in 90-second DSST results and RAVLT total recall and learning rate. In 40 % of subjects with chronic migraine scored lower than 26 points on MoCA. Patients with chronic migraine more frequently had lower DSST rates as compared to episodic migraine (odds ratio 5.07 (95 % confidence interval – 1.59–16.17); p = 0.003). Depression and anxiety did not correlate with performance on cognitive tests. Chronic migraine (frequent headache) and longer headache history, but not depression, anxiety or medication overuse were independent predictors of cognitive impairment.

Conclusion. Subjective and objective cognitive deficits are prevalent in the chronic migraine population. Most often memory and attention are impaired. Longer headache history and presence of chronic migraine are independent risk factors for cognitive impairment in patients with chronic migraine.

43-48 2414
Abstract

Background. Excessive daytime sleepiness is a very important symptom of a wide range of pathological conditions and has a significant impact on both the individual patient and the society as a whole. Estimation of the prevalence of excessive daytime sleepiness, identifying the cause, and also its elimination is an important socio-economic task.

Objective to estimate the prevalence of excessive daytime sleepiness in the population of the Russian Federation according to the Internet survey data and the distribution of the estimate of the daytime sleepiness by age and body mass index.

Materials and methods. One-time study according to the Internet survey data on the Epworth drowsiness scale (ESS).

Results. Excessive daytime sleepiness (more than 10 points on the ESS scale) was observed in 40,9% of respondents.

Conclusion. According to the results of the study, excessive daytime sleepiness is widespread in the Russian Federation, which speaks of its socio-economic significance, the need for measures to identify its causes and their elimination.

CLINICAL DISCUSSION

49-53 1810
Abstract

The cardiofaciocutaneous syndrome is a condition of sporadic occurrence, with patients showing multiple congenital anomalies and mental retardation. The syndrome is caused by molecular disturbances in the RAS/MAPK pathway. We report on the girl, 9 year-old, with the cardiofaciocutaneous syndrome presenting with typical craniofacial appearance, heart defects, ectodermal abnormalities, neglected orthopedic pathology, developmental delay and spasticity, which rare in this syndrome.

54-60 2775
Abstract

Christian–Weber disease refers to the group of panniculitis, which present an inflammation of the subcutaneous adipose tissue with the involvement of neutrophils, white blood cells and histiocytics, which leads to fibrosis in adipose tissue and sometimes to granulomatous changes. Clinically this pathology manifests from the formation of nodes in the subcutaneous adipose tissue, which appear suddenly, sporadically in some areas or multiple, sometimes forming large aggregations (plaques) or strands, shaped like a rosary. Consistency of nodes initially soft, then compacted, can form adhesions with the skin, the skin over them is not changed initially, followed by a color change from bright pink to brown-purple. Ends the process of atrophy of the affected area, sometimes with a depression in the center. Localization sites in the body are different; they are placed often on the buttocks, arms and legs. The duration of the process of the skin varies from a few days to a few months, after involution of old nodes may appear new ones, it are the cause of increasing the duration of the disease, having a relapsing course. It can be rather difficult to do differential diagnosis, which requires a great deal of experience of experts and a team approach to the study and treatment of this pathology. To confirm the diagnosis requires a biopsy of the skin. Prognosis depends on the nature of the disease and timely diagnosis.

61-70 3421
Abstract
The article is devoted to the etiology, prevalence, differential diagnosis and selection of therapy for pain in the lumbar spine. To determine the cause of lumbar pain, coordinated actions of a neurologist and an orthopedic surgeon are necessary, since chronic pain syndrome is often caused by systemic joint damage – osteoatrosis. Using the example of a clinical case, an algorithm for managing a patient with degenerative-dystrophic changes of the lumbar spine and osteoarthrosis using nonsteroidal anti-inflammatory drugs is considered. 


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ISSN 2222-8721 (Print)
ISSN 2413-0443 (Online)