All hands on deck: The multidisciplinary rehabilitation assessment and management of hand function in persons with neuromuscular disorders.

Hand function is important in every aspect of our lives. Across a wide range of neuromuscular disorders-inherited ataxias, motor neuron diseases, polyneuropathies, and myopathies-people can experience losses in hand strength, tone, movement, dexterity, joint range, and sensation. Such changes can adversely affect function and independence in daily activities, reducing participation and quality of life. People with neuromuscular disorders (pwNMD) known to involve the hand should be assessed at regular intervals for changes both clinically and using impairment, performance, function, and patient-reported outcome measures as appropriate. A patient-centered approach to management is recommended, with clinicians partnering with the individual, their caregivers and the interprofessional teams to create personalized solutions that can overcome barriers to participation and best meet the goals of individuals affected by neuromuscular disorders. Management strategies should be multifaceted, and may include exercise, orthoses, assistive devices, technological solutions, environmental or task adaptations, medications, and/or surgery. Exercise recommendations and orthoses should be individualized and evolve based on disease progression, impairments, and functional limitations. While medications and surgery have a small role for specific clinical situations, there is a plethora of assistive and technological solutions to assist with basic and instrumental activities of daily living, work/education, and leisure for pwNMD with reduced hand function. In addition, clinicians should advocate for appropriate accommodations for reduced hand function at work/school, and the development of and adherence to legislation supporting accessibility and inclusion.

Scoping review of music therapy and music interventions in spinal cord injury.

PURPOSE: Spinal cord injury (SCI) has lifelong implications requiring treatment for outcomes including respiratory function, voice, pain, mood, and gait, among others. Music therapy (MT) and music-based interventions may be useful in the treatment of several outcomes. This review describes the use of MT and music-based interventions in individuals with SCI for rehabilitation and health and highlights future research priorities. MATERIALS AND METHODS: MEDLINE, Embase, PsycInfo, CINAHL, RILM, Music Periodicals and Music Index were searched. Search terms included: SCI and music. Studies of cohorts with SCI using music interventions and descriptions of adapted instruments or development of MT programs were included. Abstracts and full texts were reviewed in duplicate. Data were extracted according to clinical outcomes. A structured synthesis was performed. RESULTS: Forty-three studies were included. Research in the field includes quantitative, qualitative and mixed-methods studies. Group singing and an individual songwriting program for self-concept were the most studied interventions. Outcomes varied; mood outcomes were most common. CONCLUSION: While qualitative data support the use of MT and music-based interventions in this population for a wide variety of outcomes, randomized controlled trials are needed. There is a lack of research on the use of individual MT in this population. Registration: osf.io/9m8v4 Implications for RehabilitationIndividuals with spinal cord injury (SCI) often suffer from injury complications and significant medical morbidity requiring practical long-term treatment and wellness strategies.Music therapy (MT) and music-based interventions can be used for many rehabilitation and health goals in this population including mood, gait and respiratory function, among others.Preliminary qualitative and quantitative studies have reported the benefits of MT across a range of outcomes in individuals with SCI; however, additional research, especially evaluating individual MT interventions, is needed.

Effects of music therapy on mood, pain, and satisfaction in the neurologic inpatient setting.

PURPOSE: Music therapy (MT) has been used in health care settings for a wide variety of treatment goals. Many inpatients with neurologic impairments struggle with low mood and pain for which MT may be a novel adjunct treatment. The aims of this study were to: (1) evaluate change in mood and pain following a single MT session; (2) explore the impact of an MT program on mood, pain and satisfaction from the perspective of the patient, family and staff. MATERIALS AND METHODS: A mixed-methods study was conducted. Forty participants completed pre/post MT surveys evaluating mood, pain and satisfaction. Individual semi-structured interviews and focus groups were conducted with 14 MT program participants (inpatients), 5 family members of participants and 16 staff. RESULTS: There was significant improvement in mood (p < 0.001) and decrease in pain (p < 0.05) from pre-to-post MT with 74% reporting being "very satisfied" with the session. "Benefits of MT" was the overarching theme of the qualitative data. Subthemes were emotional regulation, pain management, effects on self-concept, enjoyment, and social connectedness. CONCLUSIONS: Improvements in mood and pain were reported from pre-to-post MT session and in interviews. Further evaluation of MT effectiveness against standard of care rehabilitation and cost implications is required.Implications for RehabilitationMusic therapy (MT) is delivered by accredited music therapists (MTAs) in health care settings, including rehabilitation units, as an individual, group or co-treatment therapy.MT can be used for a range of health outcomes, including the treatment of mood and pain, in addition to improving inpatient satisfaction.Early evidence using pre/post MT surveys suggests an improvement in mood and pain following a single therapy session.Qualitative data suggest overall benefit of offering MT services in addition to standard neurorehabilitation therapy, including improvements to emotional regulation, pain management, self-concept, enjoyment, and social connectedness.

Inequality in abundance.

With technological advance has come the possibility of a new era of abundance. Technologies like 3D printing and robotics promise to lower the costs of production and distribution of goods and services, presumably making these goods and services readily available to those across income and wealth spectrums. This undoubtedly is a good thing. But what will be the effect of these technologies on existing wealth inequalities and the psychological and societal burdens they impose? Can we expect that this newfound abundance will help remedy the current historic levels of inequality in the U.S. and other western countries? Unfortunately, the answer is likely no, for two reasons. First, history suggests that inequality often persists even under conditions of abundance due to dynamics of power and politics and ongoing impacts of structural inequalities. Having more than enough of a particular good or service to go around does not guarantee that all will have access to this good or service. Second, even if the new abundance enabled by technology extends into all levels of the socioeconomic spectrum, enabling individuals to access goods and services (and their attendant benefits) previously beyond their reach, the harms that attend unequal societies will persist. Increasing evidence suggests that these harms, including increased violence and decreased health, arise not from access (or a lack thereof) to particular goods and services, but from the adverse psychological consequences of living in an unequal society. This is a psychological burden shared not just by those at the losing end of the inequality equation, but also those who enjoy a relative advantage in society. Unequal societies are psychologically harmful to all who live in them, regardless of where these individuals fall on the socioeconomic spectrum, and largely independent of the particular goods and services they enjoy. The upshot is that society cannot rely on new abundance technologies to automatically solve problems of inequality and the social and psychological burdens that plague those who live in unequal societies. Indeed, depending on how society responds to questions of access to these technologies, their introduction might exacerbate various forms of inequality. In light of this, it is crucial to address conditions of inequality head-on, so that the new era of abundance promised by technological advance can lead to real gains in individual and societal wellbeing.

A Canadian Adult Spinal Muscular Atrophy Outcome Measures Toolkit: Results of a National Consensus using a Modified Delphi Method.

BACKGROUND: Spinal Muscular Atrophy (SMA) is a rare disease that affects 1 in 11 000 live births. Recent developments in SMA treatments have included new disease-modifying therapies that require high quality data to inform decisions around initiation and continuation of therapy. In Canada, there are no nationally agreed upon outcome measures (OM) used in adult SMA. Standardization of OM is essential to obtain high quality data that is comparable among neuromuscular clinics. OBJECTIVE: To develop a recommended toolkit and timing of OM for assessment of adults with SMA. METHODS: A modified delphi method consisting of 2 virtual voting rounds followed by a virtual conference was utilized with a panel of expert clinicians treating adult SMA across Canada. RESULTS: A consensus-derived toolkit of 8 OM was developed across three domains of function, with an additional 3 optional measures. Optimal assessment frequency is 12 months for most patients regardless of therapeutic access, while patients in their first year of receiving disease-modifying therapy should be assessed more frequently. CONCLUSIONS: The implementation of the consensus-derived OM toolkit will improve monitoring and assessment of adult SMA patients, and enrich the quality of real-world evidence. Regular updates to the toolkit must be considered as new evidence becomes available.

A National Spinal Muscular Atrophy Registry for Real-World Evidence.

BACKGROUND: Spinal muscular atrophy (SMA) is a devastating rare disease that affects individuals regardless of ethnicity, gender, and age. The first-approved disease-modifying therapy for SMA, nusinursen, was approved by Health Canada, as well as by American and European regulatory agencies following positive clinical trial outcomes. The trials were conducted in a narrow pediatric population defined by age, severity, and genotype. Broad approval of therapy necessitates close follow-up of potential rare adverse events and effectiveness in the larger real-world population. METHODS: The Canadian Neuromuscular Disease Registry (CNDR) undertook an iterative multi-stakeholder process to expand the existing SMA dataset to capture items relevant to patient outcomes in a post-marketing environment. The CNDR SMA expanded registry is a longitudinal, prospective, observational study of patients with SMA in Canada designed to evaluate the safety and effectiveness of novel therapies and provide practical information unattainable in trials. RESULTS: The consensus expanded dataset includes items that address therapy effectiveness and safety and is collected in a multicenter, prospective, observational study, including SMA patients regardless of therapeutic status. The expanded dataset is aligned with global datasets to facilitate collaboration. Additionally, consensus dataset development aimed to standardize appropriate outcome measures across the network and broader Canadian community. Prospective outcome studies, data use, and analyses are independent of the funding partner. CONCLUSION: Prospective outcome data collected will provide results on safety and effectiveness in a post-therapy approval era. These data are essential to inform improvements in care and access to therapy for all SMA patients.

Roles of syntax information in directing song development in white-crowned sparrows (Zonotrichia leucophrys).

Syntactical cues play an important role in song learning in songbirds. White-crowned sparrows (Zonotrichia leucophrys), whose song typically consists of four to five different phrases, fail to construct normal songs if exposed to all phrase types presented singly (Plamondon, Goller, & Rose, 2008; Soha & Marler 2001b). The specific role of acquired syntax information in guiding ontogenetic trajectories of syntax, however, and the respective contributions of instructive and selective processes to syntax ontogeny remain unknown. We tutored white-crowned sparrows with syntax information ranging from acoustic isolation to full song. Manipulation of tutor syntax influenced developmental trajectories of syntax assembly, suggesting that instructive processes contribute to syntax ontogeny. Early in development, birds tutored with full song or phrase pairs preferentially produced phrase pairings matching tutor syntax. Birds tutored with single phrases showed decreased diversity of pairwise syntactical combinations immediately after tutoring compared with other tutor groups, further illustrating the role of instructive processes. Overproduction of song material was also observed, suggesting that selective forces play a role in syntax development as well. Finally, consistent with the notion that innate influences guide syntax ontogeny, birds from all groups exhibited many similarities in trajectories of syntax assembly.

Species-typical songs in white-crowned sparrows tutored with only phrase pairs.

Modern theories of learned vocal behaviours, such as human speech and singing in songbirds, posit that acoustic communication signals are reproduced from memory, using auditory feedback. The nature of these memories, however, is unclear. Here we propose and test a model for how complex song structure can emerge from sparse sequence information acquired during tutoring. In this conceptual model, a population of combination-sensitive (phrase-pair) detectors is shaped by early exposure to song and serves as the minimal representation of the template necessary for generating complete song. As predicted by the model, birds that were tutored with only pairs of normally adjacent song phrases were able to assemble full songs in which phrases were placed in the correct order; birds that were tutored with reverse-ordered phrase pairs sang songs with reversed phrase order. Birds that were tutored with all song phrases, but presented singly, failed to produce normal, full songs. These findings provide the first evidence for a minimal requirement of sequence information in the acoustic model that can give rise to correct song structure.