Background: Duchenne muscular dystrophy (DMD) patients are at a high risk of developing sleep-related breathing disorders (SRBD) such as obstructive sleep apnea (OSA). This study aimed to determine the risk of developing OSA among DMD patients.
Methods: This cross-sectional study was conducted from February 2022 to July 2022 in a tertiary healthcare facility. As a screening tool for OSA, we used the Pediatric Sleep Questionnaire (PSQ).
Results: Subjects included 60 boys with DMD, mean age 10.15 ± 3.54 years. The mean BMI for all subjects was 18.9 ± 4.08 kg/m2. Of these, 22 (36.7%) children were at high risk of OSA. Children who were overweight, and on steroids tended to be at higher risk of developing OSA (P < 0.043) and (P < 0.029) respectively.
Conclusion: Our study shows a significant risk of OSA in DMD patients. Therefore, Sleep studies should be part of the standard of care for DMD patients.
Purpose: Sexual motives are major determinants of sexual behaviour. It has been known that sexual motives may vary according to circumstances. Multiple sclerosis (MS) is a chronic disease causing a broad range of symptoms and disabilities, that often interfere with sexual activities. We aimed to investigate the sexual motives in persons with MS.
Patients and methods: Cross-sectional study in 157 persons with MS and 157 controls matched for age, gender, relationship, duration of relationship and educational status via propensity score matching. The Reasons for Having Sex (YSEX) questionnaire assessed the proportion with which a person had engaged in sexual intercourse for each of 140 distinct motives to have sex. Estimated mean differences in scores for four primary factors (Physical, Goal attainment, Emotional, Insecurity) and 13 sub-factors, and sexual satisfaction and importance of sex were calculated as Average Treatment Effect of the Treated using 99% confidence intervals.
Results: Persons with MS reported a lower proportion of engaging in sex compared with the controls for the factors Physical (-0.29), Emotional (-0.23) and Insecurity (-0.10); and for the physical sub-factors Pleasure (-0.48), Experience seeking (-0.32), Stress reduction (-0.24), and Physical desirability (-0.16), the emotional sub-factors Love and commitment (-0.27) and Expression (-0.17), and the insecurity sub-factor Self-esteem boost (-0.23). In the control group seven of the top 10 sexual motives were physical versus five in the MS group. The importance of sex was lower in the MS group (-0.68).
Conclusion: Findings of this controlled cross-sectional study suggest a reduction in the number of sexual motives in persons with MS, especially of physical motives related to pleasure and experience seeking. Health care professionals may consider assessing sexual motivation when dealing with persons with MS who suffer from decreased sexual desire or another sexual dysfunction.
Background: Patient-reported outcomes (PROs) are increasingly associated with concurrent and future impairments in persons with multiple sclerosis (pwMS). The structural and pathological relationships with PROs in pwMS have not been elucidated.
Methods: One hundred and forty-two pwMS and 47 healthy controls (HCs) were scanned using 3T MRI and completed a PRO questionnaire named Lifeware® that outlines the physical and psychosocial abilities. Beck's Depression Inventory (BDI) assessed levels of depression. T1- and T2-lesion volume, volumes of the whole brain (WBV), gray matter (GMV), white matter (WMV) and lateral ventricle (LVV) were derived using JIM and SIENAX software. Additional deep GM (DGMV) and nuclei-specific volumes of the thalamus, caudate, globus pallidus, putamen, and hippocampus were calculated using FIRST. Ordinal regression models adjusted for age and depression and mediation analyses were used.
Results: When compared to HCs, pwMS reported significantly greater limitations in mobility domains, including standing up from low seat (p < 0.001), climbing flight of stairs (p < 0.001), lower limb limitation (p < 0.001), limitations in bladder continence (p = 0.001) and fatigability (p < 0.001). Patient-reported limitations related to lower extremity function were explained by age, BDI, and all DGM nuclei volumes (p < 0.029). No such relationships were seen in the HCs. Fatiguability and the extent of life satisfaction were only related to depression (BDI p < 0.001) and not associated with any MRI-based outcomes. Most relationships between structural pathology and PROs were mediated by BDI scores (p < 0.001). In the pwMS group, there were no significant differences in any MRI-based brain volumes between the levels of reported life satisfaction.
Conclusion: PRO measures of lower extremity limitations were associated with DGM structures and DGM-specific nuclei. These findings promote the relevance of measuring DGM structures as measures directly related to subjective well-being and walking limitations. Depression is a significant mediator of PROs and in particular of life satisfaction.
Multiple sclerosis is a degenerative inflammatory disease that causes different musculoskeletal problems. Its impact has led to the study of treatment alternatives such as the use of invasive physiotherapy. In this study, we analyze the effects of ultrasound-guided percutaneous neuromodulation to a 51-year-old man suffering from multiple sclerosis and an associated hemiparesis in the left upper limb. A dry needling needle was placed in contact with the median nerve under ultrasound guidance and 10 trains of 10 seconds of electrostimulation with a frequency of 10 Hz and an impulse width of 240 µs were applied, with 10 seconds of pause between them. There was a significant improvement in the grip strength immediately after the treatment which increased progressively at 24 hours and at 4 days follow-up. There was also an improvement in the hand function, with a decrease in the time necessary to perform the 9 Hole Peg Test immediately after the treatment, which was maintained at 24 hours and at 4 days follow-up. Future studies with larger samples are needed to further test the effects of this invasive physiotherapy technique as well as its possible applications to other neurological conditions.
Parkinson's disease (PD) is one of the most common progressive neurodegenerative diseases caused by the loss of dopamine-producing neuronal cells in the region of substantia nigra pars compacta of the brain. During biological aging, neuronal cells slowly undergo degeneration, but the rate of cell death increases tremendously under some pathological conditions, leading to irreversible neurodegenerative diseases. By the time symptoms of PD usually appear, more than 50 to 60% of neuronal cells have already been destroyed. PD symptoms often start with tremors, followed by slow movement, stiffness, and postural imbalance. The etiology of PD is still unknown; however, besides genetics, several factors contribute to neurodegenerative disease, including exposure to pesticides, environmental chemicals, solvents, and heavy metals. Postmortem brain tissues of patients with PD show mitochondrial abnormalities, including dysfunction of the electron transport chain. Most chemicals present in our environment have been shown to target the mitochondria; remarkably, patients with PD show a mild deficiency in NADH dehydrogenase activity, signifying a possible link between PD and mitochondrial dysfunction. Inhibition of electron transport complexes generates free radicals that further attack the macromolecules leading to neuropathological conditions. Apart from that, oxidative stress also causes neuroinflammation-mediated neurodegeneration due to the activation of microglial cells. However, the mechanism that causes mitochondrial dysfunction, especially the electron transport chain, in the pathogenesis of PD remains unclear. This review discusses the recent updates and explains the possible mechanisms of mitochondrial toxicant-induced neuroinflammation and neurodegeneration in PD.
Introduction: The symptoms of Amyotrophic Lateral Sclerosis (ALS) include muscle weakness and eventual paralysis. These symptoms result from denervation of the neuromuscular junction (NMJ) and motor neuron cell death in the brain and spinal cord. Due to the "dying back" pattern of motor neuron degeneration, protecting NMJs should be a therapeutic priority. Although exercise has the potential to protect against NMJ denervation, its use in ALS has been controversial. Most preclinical studies have focused on aerobic exercise, which report that exercise can be beneficial at moderate intensities. The effects of resistance exercise on NMJ preservation in limb muscles have not been explored.
Methods: We trained male SOD1-G93A rats, which model ALS, to perform a unilateral isometric forelimb resistance exercise task. This task allows within-animal comparisons of trained and untrained forelimbs. We then determined the effects of isometric resistance exercise on NMJ denervation and AMP kinase (AMPK) activation in forelimb muscles.
Results: Our results revealed that SOD1-G93A rats were able to learn and perform the task similarly to wildtype rats, even after loss of body weight. SOD1-G93A rats exhibited significantly greater NMJ innervation in their trained vs their untrained forelimb biceps muscles. Measures of activated (phosphorylated) AMPK (pAMPK) were also greater in the trained vs untrained forelimb triceps muscles.
Discussion: These results demonstrate that isometric resistance exercise may protect against NMJ denervation in ALS. Future studies are required to determine the extent to which our findings generalize to female SOD1-G93A rats and to other subtypes of ALS.
Background: A stroke is a vascular accident that affects both men and women. The threat of stroke and outcome status differ between the sexes. Such data are lacking in Ethiopia. Therefore, this study assessed sex differences in stroke risk factors, clinical profiles, and outcomes in the medical ward of Dessie comprehensive specialized hospital.
Methods: A retrospective cross-sectional study was employed among stroke patients. Medical records with complete information and a confirmed diagnosis of stroke using imaging techniques were included in the study. Using simple random sampling, 344 medical records were selected, 312 of which fulfilled the inclusion criteria. Bivariate and multivariate logistic regression analyses and a chi-square test were employed. The frequency, percentage, and mean and standard deviation of the variables were described using descriptive statistics. Findings with a P-value <0.05 were considered statistically significant.
Results: Most of the patients were above or equal to 45 years old in both sexes. A significantly higher number of male than female patients were aged less than 45 years (p-value-0.001). Younger age (AOR: 2.998, p = 0.000), cigarette smoking (AOR: 2.911, p = 0.009), and Khat chewing (AOR: 3.650, p = 0.001) were risk factors for stroke in males. A higher number of males presented with hemiplegia/hemiparesis 89 (28.5%), aphasia 45 (14.4%), and facial palsy 19 (6.1%). However, more females were unconscious (15.1%). Significant differences were not seen in the stroke outcomes. Furthermore, there were no apparent differences in risk factors for stroke-related mortality.
Conclusion: Males developed stroke at a younger age. Women were older at the time of stroke onset and presented unconscious. More males experienced hemiplegia/hemiparesis, aphasia, and facial palsy. Smoking, drinking, and khat chewing were risk factors for stroke in men. There were no gender differences in the stroke death rate. Therefore, educating the public about stroke risk factors, lifestyle modification, and conducting prospective research is required.
Previous trials describe a decrease of vitamin D levels in patients with Parkinson's disease and relationships to clinical disease severity. This case control study found higher but not significant 25-OH-vitamin D plasma levels in patients with Parkinson's disease compared with age- and sex-matched controls and no associations to clinical parameters, such as rating scores of disease severity or assessments of cognitive function. A certain variability of vitamin D concentrations was observed in both cohorts, which were investigated during the same season. These outcomes put into perspective the emerging discussion on the importance of vitamin D in Parkinson's disease. Our results warrant further confirmatory research with a strict matching design of patients and controls, which has not been done in previous investigations. We stress that this case control study does not allow any comment on the putative beneficial effects of vitamin D supplementation, ie, on bone mass or bone mineral density, in patients with Parkinson's disease.
Behavior change models are used to understand and intervene on health-related behaviors and outcomes. However, there is a gap in the literature regarding how to create and maintain behavior change in patients with complex chronic diseases such as Multiple Sclerosis (MS). To address this gap, the Wahls Behavior ChangeTM Model (WBCM) (The trademark applies to subsequent mention of the model.) was developed based on existing behavior change theory, empirical evidence, and extensive clinical experience caring for patients with complex chronic diseases. A patient-centered, comprehensive, and multimodal approach, this model provides a framework for understanding and implementing lifestyle behavior change. The overall goals of this paper are to: (1) review existing behavior change theories; (2) introduce the WBCM, including the model's 11 Principles for behavior change in patients with complex chronic diseases; and (3) share how providers can be trained to implement the WBCM. The WBCM can potentially improve short- and longer-term function and quality of life outcomes for people with complex chronic diseases.
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