Health-related quality of life across disease stages in patients with amyotrophic lateral sclerosis: results from a real-world survey.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is characterized by a rapid disease course, with disease severity being associated with declining health-related quality of life (HRQoL) in persons living with ALS (pALS). The main objective of this study was to assess the impact of disease progression on HRQoL across King's, Milano-Torino Staging (MiToS), and physician-judgement clinical staging. Additionally, we evaluated the impact of the disease on the HRQoL of care partners (cALS). METHODS: Data were sourced from the Adelphi ALS Disease Specific Programme (DSP)™, a cross-sectional survey of neurologists, pALS and cALS presenting in a real-world clinical setting between July 2020 and March 2021 in Europe and the United States. RESULTS: Neurologists (n = 142) provided data for 880 pALS. There were significant negative correlations between all three clinical staging systems and EuroQol (European Quality of Life) Five Dimension Five Level Scale (EQ-5D-5L) utility scores and visual analogue scale (VAS) ratings. Although not all differences were significant, 5-item Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-5) scores showed a stepwise increase in HRQoL impairment at each stage of the disease regardless of the staging system. At later stages, high levels of fatigue and substantial activity impairment were reported. As pALS disease states progressed, cALS also experienced a decline in HRQoL and increased burden. CONCLUSIONS: Across outcomes, pALS and cALS generally reported worse outcomes at later stages of the disease, highlighting an unmet need in this population for strategies to maximise QoL despite disease progression. Recognition and treatment of symptoms such as pain and fatigue may lead to improved outcomes for pALS and cALS.

Object-Tool-Actor Interaction: Object Information Drives Intended Action.

Tool use is typically explored via actor-tool interactions. However, the target-object (that which is being acted on) may influence perceived action possibilities and thereby guide action. Three different tool-target-object pairings were tested (Experiment 1). The hammering action demonstrated the greatest sensitivity and therefore subsequently used to further investigate target-object pairings. The hammer was removed as an option and instructions were provided using pictorial (Experiment 2), written (Experiment 3), and both pictorial and written formats (Experiment 4). The designed tool is chosen when available (Experiment 1) and when removed as a choice (i.e., the hammer), participants perform the same action associated with the designed tool (i.e., hammering) regardless of instruction method (Experiments 2, 3, and 4).

The influence of action execution on end-state comfort and underlying movement kinematics: An examination of right and left handed participants.

People typically move in an anticipatory manner, planning the intended action in advance to minimize the energy costs associated with producing the action (e.g., Rosenbaum et al., 2009). This is exemplified behaviorally in the end-state comfort effect, which is characterized by the selection of an uncomfortable initial posture to enable a comfortable posture upon completion of the movement (Rosenbaum et al., 1990). The main objective of this study was to further investigate the end-state comfort effect in left- and right-handers (N=20). More specifically, to: (a) understand the influence of mode of action execution; and (b) delineate the role of handedness. The overturned glass task (Fischman, 1997) was used as means of assessment, where participants were asked to demonstrate picking up a glass to pour water in four modes of execution: (1) pantomime without a stimulus; (2) pantomime with image of the glass as a guide; (3) pantomime with glass as a guide; and (4) grasping the glass. End-state comfort was displayed regardless of mode of execution, hand used to complete the task or handedness group. However, kinematic analysis revealed distinct differences, highlighting how movement parameters are altered as a result the mode of action execution.

Comparative efficacy of tyrosine kinase inhibitor treatments in the third-line setting, for chronic-phase chronic myelogenous leukemia after failure of second-generation tyrosine kinase inhibitors.

We compared the efficacy of ponatinib and second-generation tyrosine kinase inhibitors (2G-TKIs: bosutinib, dasatinib, and nilotinib) in chronic phase CML resistant/intolerant to ≥1 prior 2G-TKI. Estimated probabilities of CCyR with 2G-TKI ranged from 22% to 26%, compared with 60% (95% CrI 52-68%) with ponatinib. The estimated probability of ponatinib providing higher response rate than all other included treatments was 99% (CCyR) and 97% (MCyR). Use of further 2G-TKI may provide limited benefit in CP-CML patients resistant/intolerant to prior 2G-TKI treatment. Compared with 2G-TKIs, ponatinib is estimated to provide substantially higher probability of achieving CCyR and MCyR; safety was not compared.

Motor skills in Czech children with attention-deficit/hyperactivity disorder and their neurotypical counterparts.

Attention-deficit/hyperactivity disorder (ADHD) is the most commonly diagnosed neurobehavioural disorder. Characterized by recurring problems with impulsiveness and inattention in combination with hyperactivity, motor impairments have also been well documented in the literature. The aim of this study was to compare the fine and gross motor skills of male and female children with ADHD and their neurotypical counterparts within seven skill assessments. This included three fine motor tasks: (1) spiral tracing, (2) dot filling, (3) tweezers and beads; and four gross motor tasks: (1) twistbox, (2) foot tapping, (3) small plate finger tapping, and (4) large plate finger tapping. It was hypothesized that children with ADHD would display poorer motor skills in comparison to neurotypical controls in both fine and gross motor assessments. However, statistically significant differences between the groups only emerged in four of the seven tasks (spiral tracing, dot filling, tweezers and beads and foot tapping). In line with previous findings, the complexity underlying upper limb tasks solidified the divide in performance between children with ADHD and their neurotypical counterparts. In light of similar research, impairments in lower limb motor skill were also observed. Future research is required to further delineate trends in motor difficulties in ADHD, while further investigating the underlying mechanisms of impairment.

An examination of handedness and footedness in children with high functioning autism and Asperger syndrome.

Motor control deficits have been documented in children with high functioning autism and Asperger syndrome (HFA/AS), but the extent to which these disorders affect the children's footedness must be delineated. Twelve typically developing (TD) children and 12 children with HFA/AS, ages 6-9 years, were recruited. Motor control skills were assessed through a variety of footedness tasks to determine location and nature of impairment, regarding motor dominance. Overall, greater inconsistencies in dominance arose in children with HFA/AS, through disparities in measures of preference. Results will have broader implications for understanding motor impairments in children with HFA/AS as determined by comparing performance on footedness tasks, as well as for the design of interventions to account for these deficits.

Can an observational method of assessing hand preference be used to predict language lateralisation?

The current study investigated (a) whether or not the WatHand Cabinet Test (WHCT, Bryden, Roy, & Spence, 2007) could be used as accurately as the Waterloo Handedness Questionnaire (WHQ) to classify individuals into language lateralisation groups based on their hand preference, and (b) the relationship between direction and degree of hand preference and language lateralisation. A total of 142 participants (82 right-handers and 60 left-handers) completed the WHQ and the WHCT, and performed a fused-words dichotic listening test. Findings indicated that the WHCT was robust alternative to the WHQ in providing a measure of hand preference as there was a high correlation between the WHCT and the WHQ, and individuals were divided into similar language lateralisation groups when using either the WHCT or the WHQ as the classifying variable. More specifically, there existed a predictable pattern of language lateralisation into which members of different handedness groups fell. The same pattern exists whether handedness is defined using subjective questionnaires or more objective observational measures of hand preference.

The liberal party and the achievement of national Medicare.

The process that led to the implementation of a full national health insurance system in Canada was as complicated and contested as the battles that were fought over Medicare in Saskatchewan. The federal Liberal party had to first adopt health insurance as a serious component of its electoral platform, devise a strategy for dealing with provinces which had constitutional jurisdiction over health, and finally wrestle with those within the party--and within the cabinet--who continued to question whether Canada was financially prepared to administer such a costly program. The strategies were devised and the battles were fought privately, but had an important effect on the timing and shape of a national health insurance system.

An observational method of assessing handedness in children and adults.

The current study was performed to assess the WatHand Cabinet Test (WHCT), a newly developed multidimensional observational test of handedness. Because the test is observational, it is ideal for assessing children, as it does not require a high degree of verbal comprehension on the part of the participants. 548 individuals participated in the present study on a voluntary basis. Individuals of varying ages were examined (including 3 to 5, 6, 7, 8, 9, 10, and 11-year-olds, and 19-to 24-year-olds). Each participant was asked to complete the WHCT, the Annett Pegboard (Annett, 1985), and the Waterloo Handedness Questionnaire (WHQ) (M.P. Bryden, 1977). Overall, the total score on the WHCT was significantly correlated with both the WHQ, r = .795, p < .01, and the Annett Pegboard, r = .542, p < .01. Sub-scores measuring skilled performance, bimanual performance, and internal consistency were also examined, as well as performance on the three tests as a function of age. Overall, findings indicate that that the WHCT is a valid test of hand preference in both children and adults. Its ease of use, quick administration, and built-in quantitative sub-scores offer a robust alternative method for measuring hand preference.

Coordination and concurrency in bimanual rotation tasks when moving away from and toward the body.

In the present series of experiments we investigated how object transport and rotate movements are performed when they are directed away from (Experiment 1) and toward (Experiment 2) the body under both unimanual and bimanual conditions. Our results indicated that unimanual conditions are faster and more efficiently produced than bimanual movements in far peripersonal space, suggesting that there is a cost to performing bimanual movements. However, in near peripersonal space, bimanual same movements were performed in a manner similar to unimanual movements, indicating that there is no significant cost associated with similar bimanual movements that are performed using the lower visual field and in near peripersonal space. Both experiments also indicate that the two hands are tightly synchronized when the two movements being performed require the same rotation. However, when performing bimanual movements where the rotation being performed by the two hands is different, this synchronization is weaker. Finally, the combined results from the two experiments indicated that movements made toward the body are not performed in a similar manner to movements that are made away from the body. Specifically, it is clear from the current studies that movements toward the body are performed faster and possibly that the hands are less synchronized for bimanual movements requiring different rotations by the two hands.

Task demands affect manual asymmetries in pegboard performance.

Previous work by Bryden and Roy (1999) showed a larger performance difference between the hands for placing pegs into holes on the Grooved Pegboard test than for removing pegs from the holes. The authors argued that these data provided evidence of the importance of task demands in manual asymmetries. However the study failed to control for the differing starting positions of the pegs. To clarify this possible confound, the purpose of the current investigation was to determine the influence of starting position on the between-hand performance differences on the Grooved Pegboard Test. To do so, both the start and end positions were manipulated, such that participants moved the pegs from the receptacle to another receptacle or to a set of holes, or participants moved the pegs from a set of holes to a receptacle or another set of holes. A total of 30 right-handed individuals (as classified using the Waterloo Handedness Questionnaire) participated in the experiment, completing five trials with each hand for each of the four conditions. While no significant effects of start position were found, a significant interaction between hand and end position, F(1.29) = 30.85, P<.001, was found for the time to complete the task, where larger differences between the hands, fovouring the right hand, were seen for placing pegs into the holes as opposed to the receptacles. This effect was also found when the data were expressed using a laterality quotient. The results are discussed in terms of the influence of task complexity on manual asymmetries.

Handedness and health: an examination of the association between different handedness classifications and health disorders.

The current study investigated the incidence of various health disorders in a sample population of undergraduate students, using a 25-item hand preference questionnaire. Analysis revealed significant differences existed in the incidence of several health disorders as a function of handedness: however, these differences depended on how hand preference was defined. Overall, the results showed that non-right-handed participants (i.e., those individuals with left-handed, ambidextrous, or inconsistent hand preferences) were significantly more likely to report specific health disorders (such as high blood pressure and epilepsy) than right-handed participants. The findings provide support for the argument that left-handedness, or non-right-handedness, is associated with various health disorders. The results are discussed in light of the Geschwind-Behan-Galaburda model of cerebral lateralisation.

A new method of administering the Grooved Pegboard Test: performance as a function of handedness and sex.

The purpose of the present study was twofold: first to examine the influences of sex and handedness on manual performance on the Grooved Pegboard Test; and secondly to provide normative data for two versions (Place and Remove tasks) of the Grooved Pegboard Test, as previous work (Bryden & Roy, 1999) had suggested that the Remove task of the Grooved pegboard may provide a purer measure of motor speed of the two hands than the standard administration of the Grooved Pegboard Test. One hundred and fifty-three (47 males and 106 females) participants completed the Grooved Pegboard Test. Individuals performed the standard version of the Grooved Pegboard Test (Place task) and a novel version of the test (Remove task). In the standard version, participants were timed on their speed for placing the pegs, while in the novel version they were timed on their speed for removing the pegs. Results confirmed previously noted hand and sex differences in the Place task of the Grooved Pegboard Test, as well as the lack of effect of handedness on performance (Bornstein, 1995; Ruff & Parker, 1993). Significant performance differences between the hands were also noted for the Remove task. Findings also indicated that the Remove task was sensitive to sex and handedness effects.

Using U.S. Poison Control Center records to identify bystander pesticide exposures: a one-year surveillance of four southeastern states.

The extent to which bystanders are exposed to pesticide applications is unknown. Systematic monitoring around spray areas is not routine. Quantifying exposures is extremely difficult. Persons inadvertently exposed to pesticides often do not know the chemical or quantity, and persons living near areas of frequent field spraying may receive multiple exposures. In the U.S., concerns about health consequences from these exposures may prompt calls to poison control centers. The goal of this study was to determine what surveillance poison control centers can provide on environmental pesticide exposures to bystanders. We searched the American Association of Poison Control Centers' 2001 electronic medical records for exposure reports involving persons from 129 agriculturally intensive counties in Kentucky, Tennessee, Louisiana, and Arkansas that implicated at least one of 54 generic classifications of agricultural chemicals. We abstracted 980 pesticide-related records. Narrative sections were reviewed to determine bystander status of the exposed person. Forty-six bystander exposures were identified from 32 events. Bystander ages ranged from 2 to 81 (median: 45; 16 females, 13 males). All pertinent information for bystander classification came from narrative sections of the record. 28% identified aircraft crop dusters as the pesticide source. The most implicated substance was malathion (30.4%), while 19.6% did not know the exposure substance. 73.9% of cases were symptomatic; 65.2% of the exposed persons were seen in or referred to a healthcare facility. No hospitalizations or deaths were reported. Although they may underestimate the true numbers, U.S. poison control center data can provide valuable information about bystander environmental pesticide exposures.

Unimanual performance across the age span.

The purpose of the current investigation was to examine the age-related changes in the performance of the two hands on the Annett pegboard (Annett, 1970). The current study was part of a large-scale study investigating the development of unimanual and bimanual performance. Three hundred and two right-handed individuals participated in the present study, comprising five different age groups (3-5 year olds, 6 and 7 year olds, 8 and 9 year olds, 10-12 year olds, and 19-24 year olds). All participants completed the Waterloo Handedness Questionnaire and the standard Annett pegboard. Analyses revealed significant overall performance differences between the hands, as expected, where the right hand was significantly faster than the left hand. Additionally, significant performance differences between the hands were noted as a function of age. More specifically, the adults showed a much smaller performance difference between the hands than the other age groups. The results are discussed in light of current theories of the development of hand preference and manual asymmetry.

Development of handedness: comparison of questionnaire and performance-based measures of preference.

Hand preference has been assessed primarily using questionnaires. This approach is difficult to use with children so researchers have frequently used observation of hand choice when children pick up and use tools. Recently we developed such a performance-based measure, the WatHand Box Test (WBT). Participants ranging in age from 2 to 24 years each completed the WBT and the Revised Waterloo Handedness Questionnaire. Analyses revealed a strong preference for the right hand across all age groups on both tests with no significant differences across age and a significant correlation between the two measures. These findings do not concur with other work showing children younger than six years exhibited a much smaller right-hand preference. The concordance between these two preference measures is consistent with our previous findings. However, the relatively small common variance explained in this relationship suggests that these two tests may reflect somewhat different components of preference.

Knowledge of the risks and benefits associated with oral contraception in a university-aged sample of users and non-users.

As research has shown that knowledge concerning the health risks and benefits of oral contraceptive (OC) use is a contributor to OC compliance, the following study examined an educated sample of young women to determine the level of knowledge concerning the risks and benefits of OC use. A 28-item questionnaire was developed by the research team and distributed to female undergraduate students. Five questions were designed to assess the respondent's knowledge of OC. Overall, it found that OC users answered 71.9% of the questions correctly, whereas non-users answered significantly fewer questions correctly. As well, the number of correct responses increased with academic year, indicating that younger women were less knowledgeable about OC. The results of this study indicate that despite increased efforts to educate women, knowledge of OC remains a major problem, even in a sample of women with relatively high socio-economic status.

Preliminary examination of oral contraceptive use among university-aged females.

The etymology and use of oral contraceptives (OCs) has greatly influenced the sexual practices and social lives (e.g., decisions concerning childbearing, careers) of adolescent and adult women. In addition, OCs have the potential to benefit or harm its users depending upon the utilization practices and characteristics/lifestyle habits of the women consuming them. A 28-item questionnaire was distributed to female students at a university in Central Ontario and examined: (1) socio-demographic information; (2) background information concerning oral contraceptive use (e.g., length of time taking the pill, concerns about pill use, side effects while on the pill); and (3) procedure for taking the pill (e.g. maximizing effectiveness and safety). Preliminary analysis indicated that females may not be equipped with adequate knowledge or receiving the necessary support to practice the most efficacious behaviours surrounding pill use.

Can I twist your arm? The influence of target orientation on the magnitude of the right-hand advantage.

Controversy exists concerning whether handedness is a unidimensional trait or a multidimensional trait. However, researchers espousing the view that handedness is multidimensional are in disagreement regarding what the dimensions are that underlie handedness. Healey, Liederman, and Geschwind (1986) have provided some evidence that the type of musculature utilised (i.e., proximal versus distal) may be an underlying factor in determining handedness. The present research attempted to test this notion by examining the effects of target orientation on the relative performance of the two hands, using a peg-moving task. Target orientation was examined by utilising key-shaped pegs that fit securely into key-shaped holes. The results showed that the presence of target orientation increased the preferred-hand advantage. It is considered that this increased advantage for the right hand may be due to the hemispheric control of proximal versus distal musculature suggested by Geschwind and Galaburda (1985).