Acta neurologica Belgica最新文献

Background: While the barriers and facilitators of physical activity adherence are well-defined, there is a limited number of studies focusing specifically on exercise adherence, which is crucial to identify in patients with multiple sclerosis (MS) due to its known disease-modifying effect.

Objective: To investigate the factors affecting adherence to physical exercise in MS patients.

Methods: Literature search was conducted in PubMed, Cochrane, Web of Science, and Scopus electronic databases up to the current date. Methodological quality was assessed using the Newcastle-Ottawa Scale (NOS) for quantitative studies and the McMaster Critical Appraisal Tool for qualitative studies, with evidence synthesis conducted using the Modified Bakker Scale.

Results: The systematic review incorporated thirteen studies, consisting of seven qualitative and six quantitative ones. These studies' methodological quality was high; 12 were high, and the other was medium (unclear). In all reviewed studies, the primary patient-related and environmental barriers with high levels of evidence regarding exercise adherence included fatigue (38%), false beliefs (18%), cost (18%), and lack of time (15%). Barriers identified in very few studies included cognitive problems (3%), bladder-bowel problems (3%), and family support and advice (3%), indicating a necessity for further research to reveal their impact on exercise adherence.

Conclusion: The scarcity of studies on exercise barriers in MS is partly due to the absence of reliable assessment methods for evaluating exercise adherence, along with the prevalent emphasis on physical activity. Future research should prioritize the development of adherence scales tailored to physical exercise and shifting focus towards less explored barriers.

The Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare neuromuscular disorder characterized by proximal muscle weakness, hyporeflexia or areflexia, and dysautonomia. Ocular and bulbar symptoms may also occur, though respiratory failure is uncommon; we report the case of a 21-year-old woman diagnosed with LEMS, without evidence of a tumor, who was initially treated with symptomatic medication, immunoglobulin, and steroids, resulting in significant clinical improvement. However, she later developed psychotic symptoms, prompting the discontinuation of steroids. Brain MRI and antineuronal antibody tests were negative. Subsequently, her condition deteriorated, leading to respiratory distress that required urgent intubation, and prolonged dysphagia that necessitated the insertion of a gastrostomy tube for nutrition, along with the maintenance of a tracheostomy. Plasmapheresis was performed, resulting in partial motor recovery. Rituximab was then introduced, leading to sustained improvement in her neuromuscular symptoms, although her neuropsychiatric symptoms persisted; this case highlights a severe progression of young-onset LEMS, marked by prominent bulbar dysfunction and respiratory distress. Neuromuscular improvement followed rituximab treatment, while the concurrent psychotic symptoms appeared to follow an independent course, suggesting a primary psychiatric comorbidity.

Objective: Upper extremity dysfunction is frequently seen in Parkinson's disease (PD). Existing research has shown that bradykinesia, which is main symptom of PD, is primarily responsible but the combined effects of spinal posture and axial rigidity on upper extremity functions were not investigated yet. The aim of this study was to investigate upper extremity functions in patients with PD and to evaluate relationship of these with spinal posture and axial rigidity.

Methods: This prospective controlled study included 40 patients with PD and 40 healthy controls. Upper extremity function was measured with the 9-Hole Peg Test. Spinal posture and axial rigidity were measured with a Spinal Mouse.

Results: Compared with the control group, a decrease in upper extremity functions (p < 0.001), decreased lumbar lordosis (p = 0.003), and posterior sacral tilt (p = 0.021) were determined in patients' group. Thoracic and lumbar mobility in the sagittal (all p < 0.001) and frontal planes (p = 0.004, p < 0.001) was found to be reduced in the patient group. A correlation was determined between upper extremity functions and lumbar mobility in the sagittal (p = 0.022, r= -0.362) and frontal planes (p = 0.045, r= -0.319) and lumbar lordosis (p = 0.048, r = 0.302).

Conclusions: The results of this study demonstrated that altered spinal posture and increased axial rigidity were related with decreased upper extremity functions in patients with PD. There is a need for further studies to investigate effect of trunk-based therapies on upper extremity function in patients with PD.

Purpose: The North Star Ambulatory Assessment (NSAA) is a functional motor outcome measure originally developed for patients with Duchenne muscular dystrophy (DMD). The aim of this study was to perform the cultural adaptation and investigate the validity and reliability of the Turkish version of the NSAA (T-NSAA) in DMD.

Methods: After translation process, internal consistency, interrater and test-retest reliability of the NSAA were determined by using the Cronbach Alpha Coefficient and Intraclass Correlation Coefficient (ICC), respectively. Absolute reliability was determined by using the Standard Error of Measurement (SEM) with minimal detectable change at 95% limits of confidence (MDC₉₅). Lower limb functionality of children was evaluated by Vignos Lower Extremity Scale (VLERS). To establish convergent validity, the correlations between T-NSAA and Motor Function Measure (MFM-32), 6-minute walk test (6-MWT), and VLERS were analyzed by using the Spearman's correlation coefficient.

Results: The study was completed with 86 patients with DMD whose age were mean 104.56 ± 24.66 months. The internal consistency (Cronbach's α = 0.94), intra-reliability (ICC = 0.977) and inter-reliability (ICC = 0.972) of T-NSAA were excellent. SEM and MDC values were low indicating satisfactory absolute agreement (< %10). The T-NSAA had strong correlations with the MFM-total score, 6-MWT, and VLERS (p < 0.01).

Conclusion: T-NSAA is a valid and reliable tool to assess ambulatory status of Turkish-speaking DMD population.

Clinical trial number: NCT05549999, Date of registration: September 19, 2022.

Objective: To evaluate the safety and efficacy of bridging intravenous thrombolysis plus mechanical thrombectomy (MT) versus direct MT in different age groups of patients with acute ischemic stroke (AIS) secondary to large vessel occlusion (LVO) of the anterior circulation.

Methods: Consecutive patients from the prospective endovascular stroke registry of the Comprehensive Stroke Center, University of Rome Tor Vergata, Italy, between January 2015 and June 2021 were retrospectively analyzed. Patients were divided into age groups (≤ 80 years old and > 80 years old); for each age group, they were further divided in the bridging therapy group and the direct MT group. We performed a propensity score analysis according to baseline characteristics. Safety outcomes were any intracerebral hemorrhage (ICH), symptomatic ICH (sICH) and 3-month mortality. Efficacy outcomes were successful recanalization (modified Thrombolysis in Cerebral Infarction, mTICI, score ≥ 2b) and 3-month functional independence (modified Rankin Scale, mRS, ≤ 2).

Results: We included 761 AIS patients with anterior circulation LVO (mean age 73.5 ± 12.8 years; 44.8% males; mean baseline NIHSS 16 ± 5). After propensity score, there were 365 patients ≤ 80 years old (52% bridging therapy) and 187 patients > 80 years old (57% bridging therapy). In both age groups of patients, we found no statistically significant differences in the rates of any ICH, sICH, successful recanalization and 3-month mortality and functional independence between bridging therapy and direct MT groups.

Conclusion: In our population, safety and efficacy outcomes of bridging therapy versus direct MT did not differ in both AIS patients ≤ 80 and > 80 years old.