Chronic inflammatory neuropathies and their impact on activities and participation

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) are inflammatory neuropathies that can lead to considerable limitations in daily activities and in social participation. However, systematic evaluation of these self-reported limitations is lacking in the currently available studies. Understanding the impact of these diagnoses on patients' life is important to optimize management strategies.Aim: To systematically assess the self-reported limitations in activities and participation and determine associated factors.Methods: A survey study was conducted in 2021 in a cohort of patients with CIDP (n = 257) and MMN (n = 148) from a university hospital. The survey included the Rasch-built Overall Disability Scale and the Utrecht Scale for Evaluation of Rehabilitation-Participation, questions addressing personal and disease-related factors and treatment. Multivariate linear regression analysis was used to determine associations with disease-related and personal factors.Results: A total of 147 CIDP and 103 MMN patients responded. Limitations in activities were reported by 70.7% CIDP and 52.2% MMN patients with moderate to severe limitations in 22.4% and 5.9% patients, respectively. Participation restrictions were reported by 50% of CIDP and 40% of MMN patients, nevertheless satisfaction with participation was high. Fatigue, pain and resilience were independently associated with limitations in activities and satisfaction with participation in CIDP patients.Conclusions: Activity limitations and restrictions in participation are common in CIDP patients and to a lesser extent in MMN patients. Fatigue, pain and resilience independently contributed to perceived limitations in CIDP patients. Referral to a rehabilitation physician is warranted to address these limitations appropriately.</p

Walking in postpoliomyelitis syndrome: The relationships between time-scored tests, walking in daily life and perceived mobility problems

Objective: To compare walking test results with walking in daily life, and to investigate the relationships between walking tests, walking activity in daily life, and perceived mobility problems in patients with post-poliomyelitis syndrome. Subjects: Twenty-four ambulant patients with post-poliomyelitis syndrome. Methods: Walking tests were performed at self-preferred and maximal speed. Walking activity was measured with an ambulatory activity monitor. Heart rate, step cadence and walking speed in the test and in daily life were compared. Walking speed in daily life was represented by the intensity of walking. Perceived mobility problems were assessed with the Nottingham Health Profile. Results: Heart rate during walking was lower in the test at self-preferred speed than in daily life (mean difference: 11.3 ± 10.4; p = 0.001). Self-preferred walking speed in the test and in daily life correlated significantly (r = 0.55; p = 0.04). In a sub-group with a test performance below the median value, test performance correlated significantly with walking activity. No significant correlation was found between perceived mobility problems and walking activity. Conclusion: Walking in daily life may be more demanding than walking under standardized conditions. Patients with post-poliomyelitis syndrome with the lowest test performance walked less in daily life. Patients do not necessarily match their activity pattern to their perceived mobility problems

Impaired Visual Integration in Children with Traumatic Brain Injury: An Observational Study

Background Axonal injury after traumatic brain injury (TBI) may cause impaired sensory integration. We aim to determine the effects of childhood TBI on visual integration in relation to general neurocognitive functioning. Methods We compared children aged 6-13 diagnosed with TBI (n = 103; M = 1.7 years post-injury) to children with traumatic control (TC) injury (n = 44). Three TBI severity groups were distinguished: mild TBI without risk factors for complicated TBI (mildRF- TBI, n = 22), mild TBI with ≥ 1 risk factor (mildRF+ TBI, n = 46) or moderate/severe TBI (n = 35). An experimental paradigm measured speed and accuracy of goal-directed behavior depending on: (1) visual identification; (2) visual localization; or (3) both, measuring visual integration. Group-differences on reaction time (RT) or accuracy were tracked down to task strategy, visual processing efficiency and extra-decisional processes (e.g. response execution) using diffusion model analysis. General neurocognitive functioning was measured by a Wechsler Intelligence Scale short form. Results The TBI group had poorer accuracy of visual identification and visual integration than the TC group (Ps ≤ .03; ds ≤ -0.40). Analyses differentiating TBI severity revealed that visual identification accuracy was impaired in the moderate/severe TBI group (P = .05, d = -0.50) and that visual integration accuracy was impaired in the mildRF+ TBI gro

Inactive lifestyle in adults with bilateral spastic cerebral palsy

Objective: To quantify the level of everyday physical activity in adults with bilateral spastic cerebral palsy, and to study associations with personal and cerebral palsy-related characteristics. Participants and methods: Fifty-six adults with bilateral spastic cerebral palsy (mean age 36.4 (standard deviation (SD) 5.8) years, 62% male) participated in the study. Approximately 75% had high gross motor functioning. Level of everyday physical activity was measured with an accelerometry-based Activity Monitor and was characterized by: (i) duration of dynamic activities (composite measure, percentage of 24 h); (ii) intensity of activity (motility, in gravitational acceleration (g)); and (iii) number of periods of continuous dynamic activity. Outcomes in adults with cerebral palsy were compared with those for able-bodied age-mates. Results: Duration of dynamic activities was 8.1 (SD 3.7) % (116 min per day), and intensity of activity was 0.020 (SD 0.007) g; both outcomes were significantly lower compared with able-bodied age-mates. Of adults with cerebral palsy, 39% had at least one period of continuous dynamic activities lasting longer than 10 min per day. Gross motor functioning was significantly associated with level of everyday physical activity (Rs -0.34 to -0.48; p≤0.01). Conclusion: Adults with bilateral spastic cerebral palsy, especially those with low-level gross motor functioning, are at risk for an inactive lifestyle

Exercise therapy and cognitive behavioural therapy to improve fatigue, daily activity performance and quality of life in Postpoliomyelitis Syndrome: the protocol of the FACTS-2-PPS trial

Contains fulltext : 88661.pdf (publisher's version ) (Open Access)BACKGROUND: Postpoliomyelitis Syndrome (PPS) is a complex of late onset neuromuscular symptoms with new or increased muscle weakness and muscle fatigability as key symptoms. Main clinical complaints are severe fatigue, deterioration in functional abilities and health related quality of life. Rehabilitation management is the mainstay of treatment. Two different therapeutic interventions may be prescribed (1) exercise therapy or (2) cognitive behavioural therapy (CBT). However, the evidence on the effectiveness of both interventions is limited. The primary aim of the FACTS-2-PPS trial is to study the efficacy of exercise therapy and CBT for reducing fatigue and improving activities and quality of life in patients with PPS. Additionally, the working mechanisms, patients' and therapists' expectations of and experiences with both interventions and cost-effectiveness will be evaluated. METHODS/DESIGN: A multi-centre, single-blinded, randomized controlled trial will be conducted. A sample of 81 severely fatigued patients with PPS will be recruited from 3 different university hospitals and their affiliate rehabilitation centres. Patients will be randomized to one of three groups i.e. (1) exercise therapy + usual care, (2) CBT + usual care, (3) usual care. At baseline, immediately post-intervention and at 3- and 6-months follow-up, fatigue, activities, quality of life and secondary outcomes will be assessed. Costs will be based on a cost questionnaire, and statistical analyses on GEE (generalized estimated equations). Analysis will also consider mechanisms of change during therapy. A responsive evaluation will be conducted to monitor the implementation process and to investigate the perspectives of patients and therapists on both interventions. DISCUSSION: A major strength of the FACTS-2-PPS study is the use of a mixed methods design in which a responsive and economic evaluation runs parallel to the trial. The results of this study will generate new evidence for the rehabilitation treatment of persons with PPS. TRIAL REGISTRATION: Dutch Trial Register NTR1371

No evidence for the effectiveness of bracing in patients with thoracolumbar fractures

Background and purpose The use of braces is widespread in patients with thoracolumbar fractures. The effectiveness of bracing, however, is controversial. We sought evidence for the effect of bracing in patients with traumatic thoracolumbar fractures based on outcome and length of hospital stay (LOS). Furthermore, we evaluated the incidence of complications of bracing. Methods An electronic search strategy with extensive MeSH headings was used in various databases to identify studies that compared bracing and non-bracing therapies. Two reviewers independently selected systematic reviews, randomized controlled trials (RCTs), controlled clinical trials, and observational studies, and both assessed the methodological quality and extracted the data. Results No systematic reviews or RCTs were found. 7 retrospective studies were included. None of these studies showed an effect of bracing. Because of poor methodological quality, no best-evidence synthesis could be performed. One observational study was selected in which a complication of bracing was reported. Interpretation In the present literature, there is no evidence for the effectiveness of bracing in patients with traumatic thoracolumbar fractures. The lack of high-quality studies prevents relevant conclusions from being draw

Clinical assessment of spasticity in children with cerebral palsy: a critical review of available instruments

This study reviews the instruments used for the clinical assessment of spasticity in children with cerebral palsy, and evaluates their compliance with the concept of spasticity, defined as a velocity-dependent increase in muscle tone to passive stretch. Searches were performed in Medline, Embase, and Cinahl, including the keywords 'spasticity', 'child', and 'cerebral palsy', to identify articles in which a clinical method to measure spasticity was reported. Thirteen clinical spasticity assessment instruments were identified and evaluated using predetermined criteria. This review consists of reports on the standardization applied for assessment at different velocities, testing posture, and quantification of spasticity. Results show that most instruments do not comply with the concept of spasticity; standardization of assessment method is often lacking, and scoring systems of most instruments are ambiguous. Only the Tardieu Scale complies with the concept of spasticity, but this instrument has a comprehensive and time-consuming clinical scoring syste

Reproducibility of maximal quadriceps strength and its relationship to maximal voluntary activation in postpoliomyelitis syndrome

OBJECTIVES: To determine what changes in maximal isometric strength can be detected in a symptomatic quadriceps muscle in patients with postpoliomyelitis syndrome (PPS) and to investigate the association between the variability in maximal strength and maximal voluntary activation (MVA). DESIGN: Repeated-measures over a 3-week interval. SETTING: University hospital. PATIENTS: Convenience sample of 65 patients with PPS. INTERVENTION: Dynamometer testing. MAIN OUTCOME MEASURES: Maximal voluntary contraction (MVC) torque of the quadriceps was measured with a Kin-Com dynamometer and MVA was determined by twitch interpolation. RESULTS: The mean difference between the 2 consecutive measurements was -0.7+/-12.8 Nm (95% confidence interval [CI], -3.9 to 2.5). The test-retest reliability was excellent for MVC torque (intraclass correlation coefficient [ICC]=.96; 95% CI,.93-.98) and moderate for MVA (ICC=.73; 95% CI,.56-.85). The smallest detectable change in MVC torque was 25% for an individual. The variability in MVA explained 18% of the variability in maximal strength. CONCLUSIONS: Variability in maximal quadriceps strength, measured with a fixed dynamometer, was large and partly related to variability in MVA. This implies that even with optimally standardized strength testing, a follow-up of many years is required to objectify progression of quadriceps weakness in an individual patient with PPS. To demonstrate changes in strength in groups of patients in follow-up or intervention studies, feasible sample sizes are required