Patients' and caregivers' perception of multidimensional and palliative care in amyotrophic lateral sclerosis - protocol of a German multicentre study.

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is an inevitably fatal condition that leads to a progressive loss of physical functioning, which results in a high psychosocial burden and organizational challenges related to medical care. Multidimensional and multiprofessional care is advised to meet the complex needs of patients and their families. Many healthcare systems, including Germany, may not be able to meet these needs because non-medical services such as psychological support or social counselling are not regularly included in the care of patients with ALS (pwALS). Specialised neuropalliative care is not routinely implemented nor widely available. Caregivers of pwALS are also highly burdened, but there is still a lack of support services for them. METHODS: This project aims to assess the perceptions and satisfaction with ALS care in Germany in pwALS and their caregivers. This will be achieved by means of a cross-sectional, multicentre survey. The examination will assess, to which extend the patients' needs in the six domains of physical, psychological, social, spiritual, practical and informational are being met by current care structures. This assessment will be linked to mental well-being, subjective quality of life, attitudes toward life-sustaining measures and physician-assisted suicide, and caregiver burden. The study aims to recruit 500 participants from nationwide ALS centres in order to draw comprehensive conclusions for Germany. A total of 29 centres, mostly acquired via the clinical and scientific German Network for Motor Neuron Diseases (MND-NET), will take part in the project, 25 of which have already started recruitment. PERSPECTIVE: It is intended to provide data-based starting points on how current practice of care in Germany is perceived pwALS and their caregivers and how it can be improved according to their needs. Planning and initiation of the study has been completed. TRIAL REGISTRATION: The study is registered at ClinicalTrails.gov; NCT06418646.

Solriamfetol real world experience study (SURWEY): Initiation, titration, safety, effectiveness, and experience during follow-up for patients with narcolepsy from Germany.

BACKGROUND: Excessive daytime sleepiness (EDS) is a core narcolepsy symptom, for which solriamfetol (Sunosi®) was recently approved in the European Union. SURWEY characterises real-world strategies used by physicians when initiating solriamfetol, and patient outcomes after follow-up. METHODS: SURWEY is an ongoing retrospective chart review conducted by physicians in Germany/France/Italy. Here, data are reported from 70 German patients with EDS and narcolepsy. Eligibility included age ≥18 years, reached a stable solriamfetol dose, and completed ≥6 weeks of treatment. Patients were classified (based on existing EDS treatment) into changeover, add-on, or new-to-therapy subgroups. RESULTS: Patients' mean ± SD age was 36.9 ± 13.9 years. Changeover from prior EDS medication was the most common initiation strategy. Initial solriamfetol dose was typically 75 mg/day (69%). In 30 patients (43%), solriamfetol was titrated; 27/30 (90%) completed titration as prescribed, most within 7 days. Mean ± SD Epworth Sleepiness Scale (ESS) score was 17.6 ± 3.1 at initiation (n = 61) and 13.6 ± 3.8 at follow-up (n = 51). Slight/strong improvements in EDS were perceived for >90% of patients (patient and physician report). Sixty-two percent reported an effect duration of 6 to <10 h; 72% reported no change in perceived nighttime sleep quality. Common adverse events included headache (9%), decreased appetite (6%), and insomnia (6%); no cardiovascular events were reported. CONCLUSIONS: Most patients in this study were switched from a prior EDS medication to solriamfetol. Solriamfetol was typically initiated at 75 mg/day; titration was common. ESS scores improved after initiation, and most patients perceived improvement in EDS. Common adverse events were consistent with those reported in clinical trials. GOV REGISTRATION: N/A.

Sleep quality, daytime sleepiness, fatigue, and quality of life in patients with multiple sclerosis treated with interferon beta-1b: results from a prospective observational cohort study.

BACKGROUND: Sleep disorders and fatigue are common in multiple sclerosis (MS). The underlying causes are not fully understood, and prospective studies are lacking. Therefore, we conducted a prospective, observational cohort study investigating sleep quality, fatigue, quality of life, and comorbidities in patients with MS. METHODS: Patients with relapsing-remitting MS or clinically isolated syndrome treated with interferon beta-1b were followed over two years. The primary objective was to investigate correlations between sleep quality (PSQI), fatigue (MFIS), and functional health status (SF-36). Secondary objectives were to investigate correlations of sleep quality and daytime sleepiness (ESS), depression (HADS-D), anxiety (HADS-A), pain (HSAL), and restless legs syndrome (RLS). We applied descriptive statistics, correlation and regression analyses. RESULTS: 139 patients were enrolled, 128 were available for full analysis. The proportion of poor sleepers (PSQI≥5) was 55.47% at the beginning and 37.70% by the end of the study (106 and 41 evaluable questionnaires, respectively). Poor sleepers performed worse in MFIS, SF-36, ESS, HADS-D, and HADS-A scores. The prevalence of patients with RLS was low (4.5%) and all were poor sleepers. Poor sleep quality was positively correlated with fatigue and low functional health status. These relationships were corroborated by multivariable-adjusted regression analyses. ESS values and poor sleep quality at baseline seem to predict sleep quality at the one-year follow-up. No variable predicted sleep quality at the two-year follow-up. CONCLUSIONS: Our results confirm the high prevalence of poor sleep quality among patients with MS and its persistent correlation with fatigue and reduced quality of life over time. They highlight the importance of interventions to improve sleep quality. TRIAL REGISTRATION: The study was registered at clinicaltrials.gov: NCT01766063 (registered December 7, 2012). Registered retrospectively (first patient enrolled December 6, 2012).

[Quality of life in restless legs syndrome. Influence of daytime sleepiness and fatigue]. / Lebensqualität beim Restless-Legs-Syndrom Einfluss von Tagesschläfrigkeit und Fatigue.

BACKGROUND AND PURPOSE: Restless legs syndrome (RLS) is a frequent neurologic disorder characterized by leg paresthesia and motor restlessness. It is still under debate to which amount the disease affects quality of life as it causes daytime sleepiness and fatigue. The presented study evaluates the daytime problems by different scales. PATIENTS AND METHODS: 28 patients (19 women, nine men, aged 58.6 +/- 11.9 years) with RLS (78% with a severe form) were evaluated with the Epworth Sleepiness Scale (ESS), the Fatigue Severity Scale (FSS) and the SF-36 to judge quality of life. Subscales of the SF-36 contain either rather physical or mental components. RESULTS: 17/28 patients showed an increased daytime sleepiness (ESS) and 17/28 increased fatigue (FSS), whereby these symptoms did not correlate. With regard to the SF-36 scores higher values of fatigue caused a greater limitation of the physical health, daytime sleepiness correlated with limitation of the mental health. CONCLUSION: The study clearly demonstrates an impairment by fatigue and daytime sleepiness in RLS patients. Daytime symptoms worsen quality of life. Different scales have to be used to measure the different daytime symptoms. Therapy must be adjusted to the leading symptoms (e. g., stimulants in daytime sleepiness).

Driving after cerebral ischemia--a driving simulator investigation.

UNLABELLED: Stroke patients with relevant deficits are not allowed to drive. This study tended to assess driving abilities in an early stage after cerebral ischemia with a driving simulator. METHODS: 32 patients (aged 54.9 +/- 14.8 years) with acute ischemia in the middle cerebral artery (MCA) (n = 24) and in the vertebral artery (VA) (n = 8) were investigated with reaction tests and a driving simulator. Controls were 12 healthy volunteers (aged 50.0 +/- 13.9 years). RESULTS: Compared to controls, the accident rate was increased only in patients with MCA-ischemia (2.9 +/- 3.6 vs. 1.3 +/- 1.4, p < 0.05). These patients also had poorer results in the driving simulator (p < 0.05) than patients with VA-ischemia. Driving simulator performance and neuropsychological test results did not correlate. CONCLUSION: All patients had only mild deficits and stated themselves able to drive. We could demonstrate the need to focus on driving skills especially in patients with MCA-infarctions. The driving simulator assesses various physical and neuropsychological functions influencing driving. It may especially be used for expert opinions regarding driving licence. It can also serve as a pre-driver evaluation before on road driving tests.

Comparison of driving simulator performance and neuropsychological testing in narcolepsy.

Daytime sleepiness and cataplexy can increase automobile accident rates in narcolepsy. Several countries have produced guidelines for issuing a driving license. The aim of the study was to compare driving simulator performance and neuropsychological test results in narcolepsy in order to evaluate their predictive value regarding driving ability. Thirteen patients with narcolepsy (age: 41.5+/-12.9 years) and 10 healthy control patients (age: 55.1+/-7.8 years) were investigated. By computer-assisted neuropsychological testing, vigilance, alertness and divided attention were assessed. In a driving simulator patients and controls had to drive on a highway for 60 min (mean speed of 100 km/h). Different weather and daytime conditions and obstacles were presented. Epworth Sleepiness Scale-Scores were significantly raised (narcolepsy patients: 16.7+/-5.1, controls: 6.6+/-3.6, P < or = 0.001). The accident rate of the control patients increased (3.2+/-1.8 versus 1.3+/-1.5, P < or = 0.01). Significant differences in concentration lapses (e.g. tracking errors and deviation from speed limit) could not be revealed (9.8+/-3.5 versus 7.1+/-3.2, pns). Follow-up investigation in five patients after an optimising therapy could demonstrate the decrease in accidents due to concentration lapses (P < or = 0.05). Neuropsychological testing (expressed as percentage compared to a standardised control population) revealed deficits in alertness (32.3+/-28.6). Mean percentage scores of divided attention (56.9+/-25.4) and vigilance (58.7+/-26.8) were in a normal range. There was, however, a high inter-individual difference. There was no correlation between driving performance and neuropsychological test results or ESS Score. Neuropsychological test results did not significantly change in the follow-up. The difficulties encountered by the narcolepsy patient in remaining alert may account for sleep-related motor vehicle accidents. Driving simulator investigations are closely related to real traffic situations than isolated neuropsychological tests. At the present time the driving simulator seems to be a useful instrument judging driving ability especially in cases with ambiguous neuropsychological results.

Assessment of driving performance in patients with relapsing-remitting multiple sclerosis by a driving simulator.

OBJECTIVE: To compare the driving performance using a driving simulator with physical and cognitive functions as measured by the Expanded Disability Status Scale (EDSS) and the Multiple Sclerosis Functional Composite (MSFC) in patients suffering from the relapsing-remitting form of multiple sclerosis (RRMS). METHODS: 31 RRMS patients (18 women, 13 men, mean age 35.6 +/- 8.3 years, EDSS 2.8 +/- 1.4) were compared with 10 healthy controls (8 men, 2 woman, age 45.1 +/- 7.8 years). RESULTS: Compared with controls, the accident rate (5.3 +/- 3.8 vs. 1.3 +/- 1.5, p < 0.001) and concentration faults (21.1 +/- 15.5 vs. 7.1 +/- 2.6, p < 0.01) of RRMS patients using the driving simulator were increased. While there was no correlation with the EDSS score, the accident rate was correlated with the MSFC (r = -0.5, p < 0.05). Regarding the three dimensions of the MSFC, accidents were related to the number of correct answers and Z-score in the paced auditory serial addition test (PASAT) as a measure for cognitive function (r = -0.33, p < 0.05). CONCLUSION: The current study demonstrates the need to focus also on driving skills in MS patients. The risk of accidents should be evaluated after relapses in particular. However, there are great interindividual differences. In the MSFC, most deficits could be evaluated in the PASAT. As there was a significant correlation between the accident rate in the driving simulator and the PASAT results, accidents seem to be more influenced by cognitive decline than by physical impairment. This indicates that the MSFC is a broader, more dimensional scale than the EDSS and should be preferred in the case of driving assessment. At the present time, the driving simulator seems to be a useful instrument judging driving ability, especially in cases with ambiguous neuropsychological results.

Obstructive Sleep Apnea and Hypopnea Efficacy and Safety of a Long-Acting beta2-Agonist.

The effect of inhaled long-acting beta2-agonists in obstructive sleep apnea syndrome (OSAS) is unknown, although from the pharmacological point of view both therapeutic and adverse effects need to be considered. The purpose of this study was to obtain data on the efficacy and safety of salmeterol in patients with OSAS. In a randomized, double-blind, placebo-controlled, cross-over study, effects of salmeterol on respiration during sleep and sleep quality were investigated in 20 patients with OSAS. Of these, 4 patients were female, 16 male; the average age was 53.0 +/- 7.8 years, with average body mass index 28.0 +/- 3.0 kg small middle dot m(-2) and average apnea hypopnea index 35.6 +/- 17.8 h(-1). Patients with asthma, chronic obstructive pulmonary disease (COPD), and left heart failure were excluded. Placebo or verum (50 &mgr;g salmeterol) was administered at 7 pm by meter dose inhaler and spacer device. All patients underwent full polysomnography during baseline, placebo, and verum night. Statistical analysis was performed by StudentOs t-test (p > 0.05). Between the placebo and verum there were no differences in total sleep time, sleep stages, apnea index (AI), apnea hypopnea index (AHI), and nadir oxygen saturation. There was, however, 1) a significant deterioration of mean oxygen saturation (SaO2m; placebo 93.1 +/- 2.0 vs. verum 92.5 +/- 2.2%; p = 0.01), 2) of percent of time spent with an oxygen saturation (SaO2)