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.

Burden and benefit-A mixed methods study of informal Amyotrophic Lateral Sclerosis caregivers in Ireland and the Netherlands.

OBJECTIVES: Amyotrophic Lateral Sclerosis (ALS) is a systemic and terminal disorder of the central nervous system which causes paralysis of limbs, respiratory and bulbar muscles, impacting on physical, communication, cognitive and behavioural functioning. Informal caregivers play a key role in the care of people with ALS. This study aimed to explore experiences of burden along with any beneficial aspects of caregiving in ALS. An understanding of both burden and benefit is important to support the informal caregiver and the person with ALS. METHODS/DESIGN: This exploratory mixed methods study characterizes two groups of informal caregivers in Ireland (n = 76) and the Netherlands (n = 58). In a semi-structured interview, quantitative data were collected in the form of standardized measures assessing psychological distress, quality of life and burden. Qualitative data were collected from an open ended question, in which caregivers identified positive aspects in their caregiving experience. These data types were purposefully mixed in the analysis and interpretation stages, to provide a greater depth of evidence through diverse research lenses. RESULTS: The caregiver cohorts were predominantly female (69%) and spouse/partners (84%) of the person with ALS. Greater levels of self-assessed burden were found among the caregivers in the Netherlands (p < 0.05), and higher levels of quality of life among the cohort from Ireland (p < 0.05). Themes generated through qualitative analysis identified caregiver satisfaction, ability to meet the patient's needs and the (re) evaluation of meaning and existential aspects of life as positive aspects of caregiving. Existential factors were identified frequently by the caregivers in Ireland, and personal satisfaction and meeting their care recipient's needs by caregivers in the Netherlands. Three percent of all respondents reported there was nothing positive about caregiving. CONCLUSIONS: Based on our findings, we suggest that both burden and the presence of positive factors should be evaluated and monitored. The possibility of concurrent positive and challenging experiences should be considered in the design and delivery of supportive interventions for informal caregivers.

'This battle, between your gut feeling and your mind. Try to find the right balance': Parental experiences of children with spinal muscular atrophy during COVID-19 pandemic.

AIMS: Parents of children with spinal muscular atrophy (SMA) often struggle with the all-consuming nature of the demands of caring for a child with substantial physical needs. Our aim was to explore experiences, challenges and needs of parents of a child with SMA in a COVID-19 pandemic situation. METHOD: Nineteen parents of 21 children (15 months to 13 years of age) with SMA types 1-3 participated in semi-structured interviews in June to July 2020. The interviews were analysed using inductive thematic analysis. RESULTS: Parents mentioned the protection of the health and well-being of the child as the central perspective and driving force during the COVID-19 pandemic. Three subthemes were identified: (1) responsibility, (2) balancing vulnerability and resilience and (3) (in)security. Some parents focused on the positive aspects during the lockdown, such as continuation of nusinersen treatment and family life. Some parents described helpful and positive cognitions to cope with the situation. In general, parents described a need for information with regard to COVID-19 and their child with SMA and a need for discussing their dilemmas and insecurities with a healthcare professional. INTERPRETATION: Parents put the health and well-being of their children first during the pandemic. From this study, we learned that parents of children with SMA need information and value direct contact with a healthcare professional to share their dilemmas and insecurities. The dialogue can help to empower parents in the conflicts and decisions they have to make during a pandemic.

Pattern of muscle strength improvement after intravenous immunoglobulin therapy in multifocal motor neuropathy.

INTRODUCTION: In multifocal motor neuropathy (MMN), knowledge about the pattern of treatment response in a wide spectrum of muscle groups, distal as well as proximal, after intravenous immunoglobulin (IVIg) initiation is lacking. METHODS: Hand-held dynamometry data of 11 upper and lower limb muscles, from 47 patients with MMN was reviewed. Linear mixed models were used to determine the treatment response after IVIg initiation and its relationship with initial muscle weakness. RESULTS: All muscle groups showed a positive treatment response after IVIg initiation. Changes in SD scores ranged from +0.1 to +0.95. A strong association between weakness at baseline and the magnitude of the treatment response was found. DISCUSSION: Improved muscle strength in response to IVIg appears not only in distal, but to a similar degree also in proximal muscle groups in MMN, with the largest response in muscle groups that show the greatest initial weakness.

A Road Map for Remote Digital Health Technology for Motor Neuron Disease.

Despite recent and potent technological advances, the real-world implementation of remote digital health technology in the care and monitoring of patients with motor neuron disease has not yet been realized. Digital health technology may increase the accessibility to and personalization of care, whereas remote biosensors could optimize the collection of vital clinical parameters, irrespective of patients' ability to visit the clinic. To facilitate the wide-scale adoption of digital health care technology and to align current initiatives, we outline a road map that will identify clinically relevant digital parameters; mediate the development of benefit-to-burden criteria for innovative technology; and direct the validation, harmonization, and adoption of digital health care technology in real-world settings. We define two key end products of the road map: (1) a set of reliable digital parameters to capture data collected under free-living conditions that reflect patient-centric measures and facilitate clinical decision making and (2) an integrated, open-source system that provides personalized feedback to patients, health care providers, clinical researchers, and caregivers and is linked to a flexible and adaptable platform that integrates patient data in real time. Given the ever-changing care needs of patients and the relentless progression rate of motor neuron disease, the adoption of digital health care technology will significantly benefit the delivery of care and accelerate the development of effective treatments.

Discussing personalized prognosis in amyotrophic lateral sclerosis: development of a communication guide.

BACKGROUND: Personalized ENCALS survival prediction model reliably estimates the personalized prognosis of patients with amyotrophic lateral sclerosis. Concerns were raised on discussing personalized prognosis without causing anxiety and destroying hope. Tailoring communication to patient readiness and patient needs mediates the impact of prognostic disclosure. We developed a communication guide to support physicians in discussing personalized prognosis tailored to individual needs and preferences of people with ALS and their families. METHODS: A multidisciplinary working group of neurologists, rehabilitation physicians, and healthcare researchers A) identified relevant topics for guidance, B) conducted a systematic review on needs of patients regarding prognostic discussion in life-limiting disease, C) drafted recommendations based on evidence and expert opinion, and refined and finalized these recommendations in consensus rounds, based on feedback of an expert advisory panel (patients, family member, ethicist, and spiritual counsellor). RESULTS: A) Topics identified for guidance were 1) filling in the ENCALS survival model, and interpreting outcomes and uncertainty, and 2) tailoring discussion to individual needs and preferences of patients (information needs, role and needs of family, severe cognitive impairment or frontotemporal dementia, and non-western patients). B) 17 studies were included in the systematic review. C) Consensus procedures on drafted recommendations focused on selection of outcomes, uncertainty about estimated survival, culturally sensitive communication, and lack of decisional capacity. Recommendations for discussing the prognosis include the following: discuss prognosis based on the prognostic groups and their median survival, or, if more precise information is desired, on the interquartile range of the survival probability. Investigate needs and preferences of the patients and their families for prognostic disclosure, regardless of cultural background. If the patient does not want to know their prognosis, with patient permission discuss the prognosis with their family. If the patient is judged to lack decisional capacity, ask the family if they want to discuss the prognosis. Tailor prognostic disclosure step by step, discuss it in terms of time range, and emphasize uncertainty of individual survival time. CONCLUSION: This communication guide supports physicians in tailoring discussion of personalized prognosis to the individual needs and preferences of people with ALS and their families.

Effects of upper extremity surgery on activities and participation of children with cerebral palsy: a systematic review.

AIM: To evaluate and synthesize the evidence for effects of upper extremity surgery (UES) on activities and participation of children and adolescents with cerebral palsy (CP). METHOD: The databases MEDLINE, Embase, and PsycINFO were searched for publications up to September 2018. Studies included were comparative studies with or without concurrent comparison groups or case series with pretest/posttest outcomes with a minimal sample size of 10 participants; those that reported the effects of UES with a follow-up time of at least 5 months; those including patients diagnosed with CP aged up to 20 years; and those that used a validated activity-based instrument. Risk of bias was assessed using the ROBINS-I (Risk Of Bias In Non-randomised Studies - of Interventions) tool and quality assessment was performed using the Grading of Recommendations Assessment, Development and Evaluation. RESULTS: Twelve studies, involving 310 children and adolescents, were included. The ability and perception of the patient to use the hand(s) and perform activities (measured with the Shriners Hospital Upper Extremity Evaluation, Assisting Hand Assessment, and House Functional Classification) improved significantly after UES. The quality of evidence was very low for each of the activity outcomes of interest. INTERPRETATION: The very low evidence prohibits recommendations on the use of UES to guide clinical practice. More high-quality comparative studies are needed to obtain better insight into the effects of UES on activities and participation. WHAT THIS PAPER ADDS: Low quality of evidence for effects of upper extremity surgery (UES) on activities and participation. Limited evidence for improvement in activities and participation after UES.

EFECTOS DE LA CIRUGÍA DE LA EXTREMIDAD SUPERIOR SOBRE LAS ACTIVIDADES Y LA PARTICIPACIÓN DE NIÑOS CON PARÁLISIS CEREBRAL: UNA REVISIÓN SISTEMÁTICA: OBJETIVO: Evaluar y sintetizar la evidencia de los efectos de la cirugía de extremidades superiores (UES) sobre las actividades y la participación de niños y adolescentes con parálisis cerebral (PC). MÉTODO: En las bases de datos MEDLINE, Embase y PsycINFO se buscaron publicaciones hasta septiembre de 2018. Los estudios incluidos fueron estudios comparativos con o sin grupos de comparación concurrentes o series de casos con resultados de prueba previa / prueba posterior con un tamaño de muestra mínimo de 10 participantes; aquellos que informaron los efectos de UES con un tiempo de seguimiento de al menos 5 meses; aquellos que incluyen pacientes diagnosticados con PC de hasta 20 años; y aquellos que utilizaron un instrumento validado basado en actividades. El riesgo de sesgo se evaluó mediante la herramienta ROBINS-I (Riesgo de sesgo en estudios no aleatorios - Intervenciones) y la evaluación de la calidad se realizó mediante la evaluación, desarrollo y evaluación de la calificación de las recomendaciones. RESULTADOS: Se incluyeron 12 estudios con 310 niños y adolescentes. La capacidad y la percepción del paciente para usar la/s mano/s, y realizar las actividades (medidas con la Evaluación de la extremidad superior del Hospital Shriners, la Evaluación de la mano auxiliar y la Clasificación funcional de la casa) mejoraron significativamente después de la UES. La calidad de la evidencia fue muy baja para cada uno de los resultados de actividad de interés. INTERPRETACIÓN: La evidencia muy baja previene realizar recomendaciones sobre el uso de UES para guiar la práctica clínica. Se necesitan más estudios comparativos de alta calidad para obtener una mejor comprensión de los efectos de UES en las actividades y la participación.

EFEITOS DE CIRURGIA DO MEMBRO SUPERIOR EM ATIVIDADES E PARTICIPAÇÃO DE CRIANÇAS COM PARALISIA CEREBRAL: UMA REVISÃO SISTEMÁTICA: OBJETIVO: Avaliar e sintetizar a evidência dos efeitos de cirurgia do membro superior (CMS) sobre as atividades e participação de crianças com paralisia cerebral (PC). MÉTODO: As bases de dados MEDLINE, Embase, e PsycINFO foram pesquisadas quanto a publicações até setembro de 2018. Foram incluídos estudos comparativos com ou sem grupos de comparação concorrentes ou séries de casos com resultados pré/pós-teste com um tamanho amostral mínimo de 10 participantes; aqueles que reportaram os efeitos de CMS com tempo de acompanhamento de pelo menos 5 meses; aqueles incluindo pacientes com diagnóstico de PC e até 20 anos de idade; e aqueles que usaram um instrumento válido para avaliar atividade. O risco de viés foi avaliado usando o instrumento RVENA-I (Risco de viés em estudos não aleatorizados - de intervenções) e a avaliação da qualidade foi realizada com a Pontuação de Recomendações, Avaliação, Desenvolvimento e Mensuração. RESULTADOS: Doze estudos, envolvendo 310 crianças e adolescentes, foram incluídos. A capacidade e percepção do paciente em utilizar as mãos e realizar atividades (mensuradas com a Avaliação do Hospital Shriners para a extremidade superior, a Avaliação da Mão Auxiliar, e a Classificação Funcional de House) melhoraram significantemente após CMS. A qualidade da evidência foi muito baixa para os resultados de atividade de interesse. INTERPRETAÇÃO: A evidência muito baixa proíbe recomendações sobre o uso de CMS para guiar a prática clínica. Mais estudos comparativos de alta qualidade são necessários para obter mais informações a respeito dos efeitos de CMS nas atividades e participação.

Psychological distress in partners of patients with amyotrophic lateral sclerosis and progressive muscular atrophy: what's the role of care demands and perceived control?

Caregivers of Amyotrophic Lateral Sclerosis (ALS) and Progressive Muscular Atrophy (PMA) patients often experience psychological distress. Yet, it is unclear which factors explain the variance in psychological distress. This study seeks to evaluate how care demands and perceived control over caregiving influence psychological distress using moderation and mediation analysis. Data were collected as part of a RCT and 148 partners of patients with ALS or PMA were included. Psychological distress was assessed using the Hospital Anxiety and Depression Scale. Care demands were operationalized as physical functioning (Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised) and behavioural changes of the patient (Amyotrophic Lateral Sclerosis-Frontotemporal Dementia-Questionnaire). Perceived control over caregiving was assessed using items adapted from the Job Content Questionnaire. Results showed that more behavioural changes and lower perceived control over caregiving were associated with higher levels of psychological distress in caregivers. Patients' physical functioning was not significantly related to caregivers' psychological distress. No moderation or mediation effects were found of perceived control over caregiving on the relationship between demand and psychological distress. Monitoring, psychoeducation and caregiver support with regard to behavioural changes in patients, seem to be important for the wellbeing of caregivers. Caregivers' perceived control might be a target for future interventions.

Marfan syndrome in adolescence: adolescents' perspectives on (physical) functioning, disability, contextual factors and support needs.

Although essential for providing optimal adolescent patient support, knowledge of the impact of Marfan syndrome in adolescence is limited. To explore adolescents' perceived impact of Marfan syndrome on (physical) functioning (activities, participation), disability (limitations, restrictions), contextual factors and support needs, we interviewed 19 adolescents with Marfan syndrome. Audio-recordings were transcribed, coded and analysed using thematic analysis. Identified themes were "difficulties in keeping up with peers" and "being and feeling different from peers". Furthermore, an adolescent Marfan syndrome-specific International Classification of Functioning, Disability and Health for Children and Youth (ICF-CY) model derived from the data describing the adolescent perceived impact of Marfan syndrome on functioning, disability and its contextual factors. Adolescents perceived problems in keeping up with peers in school, sports, leisure and friendships/relationships, and they could not meet work requirements. Moreover, participants perceived to differ from peers due to their appearance and disability. Contextual factors: coping with Marfan syndrome, self-esteem/image, knowledge about Marfan syndrome, support from family/friends/teachers, ability to express needs and peer-group acceptation acted individually as barrier or facilitator for identified themes.Conclusion: Adolescents with Marfan syndrome perceived limitations and restrictions in (physical) functioning. They perceived problems in keeping up with peers and perceived to differ from peers due to their appearance and disability. This warrants awareness and tailored physical, psychosocial, educational and environmental support programmes to improve (physical) functioning and empowerment of adolescents with Marfan syndrome.What is known:• Marfan syndrome is a hereditary connective tissue disorder.• Marfan syndrome affects multiple systems.What is new:• Adolescents with Marfan syndrome perceive (1) problems in keeping up with peers in school, sports, leisure, friendships/relationships and work (2) to differ from peers due to their appearance and disability.• An adolescent Marfan syndrome-specific International Classification of Functioning, Disability and Health for Children and Youth model derived from the data describing the adolescent perceived impact of Marfan syndrome on functioning, disability and contextual factors.

Validity and reproducibility of the Functional Gait Assessment in persons after stroke.

OBJECTIVE:: To evaluate construct validity and reproducibility of the Functional Gait Assessment (FGA) for measuring walking balance capacity in persons after stroke. DESIGN:: Cross-sectional study. SETTING:: Inpatient and outpatient rehabilitation center. SUBJECTS:: Fifty-two persons post-stroke (median (25% and 75% percentiles)) time post-stroke 6 (5-10) weeks) with independent walking ability (mean gait speed 1.1 ± .4 m/s). METHODS:: Subjects completed a standardized FGA twice within one to eight days by the same investigator. Validity was evaluated by testing hypotheses on the association with two timed walking tests, Berg Balance Scale, and the mobility domain of the Stroke Impact Scale using correlation coefficients ( r), and with Functional Ambulation Categories using the Kruskal-Wallis test. Reproducibility of FGA scores was assessed with intraclass correlation coefficient and standard error of measurement. RESULTS:: Subjects scored a median of 22 out of 30 points at the first FGA. Moderate to high significant correlations ( r .61-.83) and significant differences in FGA median scores between the Functional Ambulation Categories were found. Eight hypotheses (80%) could be confirmed. Inter-rater, intra-rater, and test-retest reliability of the total scores were excellent. The standard error of measurement and minimal detectable change were 2 and 6 points, respectively. No relevant ceiling effect was observed. CONCLUSION:: The FGA demonstrated good measurement properties in persons after stroke and yielded no ceiling effect in contrast to other capacity measures. In clinical practice, a measurement error of 6 points should be taken into account in interpreting changes in walking balance.

Marfan syndrome in childhood: parents' perspectives of the impact on daily functioning of children, parents and family; a qualitative study.

BACKGROUND: Marfan syndrome (MFS) is a heritable connective tissue disease caused by a defect in FBN1. The diagnosis is based on the revised Ghent criteria. The main features involve the cardiovascular, musculoskeletal, ophthalmic, pulmonary systems and facial features. Although the clinical manifestations of MFS in children are thoroughly addressed in several studies, literature on the impact of MFS on daily functioning is restricted to pediatric advice on sports and leisure participation. Therefore, the full impact of MFS on daily functioning remains unclear. The aim of this qualitative study was to explore parents' perspectives on the impact of MFS on daily functioning of children with MFS aged 4-12 years, themselves and family regarding functional performance, activities, participation, personal and environmental factors, and disease burden. METHODS: In this qualitative study parents participated in individual semi-structured interviews (n = 10) and 3 focus groups (n = 5, n = 5 and n = 6). Meetings were transcribed, and data were analyzed using thematic analysis. Meaningful concepts were coded, and concepts concerning children with MFS were linked to the International Classification of Functioning, Disability and Health for Children and Youth. Thereafter themes were identified and interpreted. RESULTS: Parents reported their children could not keep up with peers because of fatigue, pain and physical impairments. Children experienced participation restrictions in school, sports, play and other leisure activities. Parents reported their child as being different due to physical appearance, which provoked unsupportive attitudes. Parental burden was caused by high care needs, lack of support, a limited social life, and concerns about the child's development. Family burden was caused by adjusted and complex family schedules, other family members with MFS, and reproductive planning decision-making, whereas family cohesiveness and caring were positively perceived factors. CONCLUSIONS: Parents perceived a large impact of MFS on daily functioning of their children with MFS, themselves and their family. More awareness among all professionals involved in the care of children with MFS and their families is needed so that professionals can address their support needs and provide tailored interventions, rehabilitation and/or educational programs to empower and improve daily functioning of the children, parents and family.

Support needs of caregivers of patients with amyotrophic lateral sclerosis: A qualitative study.

OBJECTIVE: The aim of this study was to explore the support needs of Dutch informal caregivers of patients with amyotrophic lateral sclerosis (ALS). METHOD: Individual semi-structured interviews were conducted with 21 caregivers of ALS patients. Audio-taped interviews were transcribed and data were analyzed thematically. RESULT: A total of four global support needs emerged: "more personal time", "assistance in applying for resources", "counseling", and "peer contact". Despite their needs, caregivers are reluctant to apply for and accept support. They saw their own needs as secondary to the needs of the patients. SIGNIFICANCE OF RESULTS: ALS seems to lead to an intensive caregiving situation with multiple needs emerging in a short period. This study offers targets for the development of supportive interventions. A proactive approach seems essential, acknowledging the importance of the role of the caregivers in the care process at an early stage, informing them about the risk of burden, monitoring their wellbeing, and repeatedly offering support opportunities. Using e-health may help tailor interventions to the caregivers' support needs.

Caregiver burden in amyotrophic lateral sclerosis: A systematic review.

BACKGROUND: Informal caregivers of patients with amyotrophic lateral sclerosis experience increased levels of caregiver burden as the disease progresses. Insight in the factors related to caregiver burden is needed in order to develop supportive interventions. AIM: To evaluate the evidence on patient and caregiver factors associated with caregiver burden in amyotrophic lateral sclerosis informal caregivers. DESIGN: A systematic review. DATA SOURCES: Four electronic databases were searched up to 2017. Studies that investigated quantitative relations between patient or caregiver factors and caregiver burden were included. The overall quality of evidence for factors was assessed using the Grading of Recommendations Assessment, Development and Evaluation approach. RESULTS: A total of 25 articles were included. High quality of evidence was found for the relation between caregiver burden and the factor "behavioral impairments." Moderate quality of evidence was found for the relations between caregiver burden and the factors "feelings of depression" of the caregiver and "physical functioning" of the patient. The remaining rated caregiver factors-"feelings of anxiety," "distress," "social support," "family functioning," and "age"-and patient factors-"bulbar function," "motor function," "respiratory function," "disease duration," "disinhibition," "executive functioning," "cognitive functioning," "feelings of depression," and "age"-showed low to very low quality of evidence for their association with caregiver burden. CONCLUSION: Higher caregiver burden is associated with greater behavioral and physical impairment of the patient and with more depressive feelings of the caregiver. This knowledge enables the identification of caregivers at risk for caregiver burden and guides the development of interventions to diminish caregiver burden.

Reliability of the Assisting Hand Assessment in adolescents.

AIM: To investigate the interrater and test-retest reliability of the Assisting Hand Assessment in adolescents (Ad-AHA) with cerebral palsy (CP) and to evaluate the alternate-form reliability of different test activities. METHOD: Participants were 112 adolescents with unilateral CP (60 males, 52 females; mean age 14y 5mo [standard deviation {SD} 2y 8mo], Manual Ability Classification System levels I-III). Reliability was evaluated using intraclass correlation coefficients (ICC), smallest detectable change (SDC), and Bland-Altman plots. RESULTS: ICCs for interrater (n=38) and test-retest reliability (n=31) were excellent: 0.97 (95% CI 0.94-0.98) and 0.99 (95% CI 0.98-0.99) respectively. The alternate-form reliability of different test activities was excellent for children (age 10-12y, n=30) performing the School-Kids AHA and Ad-AHA Board Game 0.99 (95% CI 0.98-0.99) and for adolescents (age 13-18y) performing the Ad-AHA Board Game compared to the Ad-AHA Present (n=28) 0.99 (95% CI 0.95-0.98), or the Ad-AHA Sandwich (n=29) 0.99 (95% CI 0.98-0.99) tasks. SDC for test-retest was 4.5 AHA-units. INTERPRETATION: Ad-AHA scores are consistent across different raters and occasions. The good alternate-form reliability indicates that the different test activities can be used interchangeably in adolescents with unilateral CP. Differences greater than or equal to 5 AHA-units can be considered a change beyond measurement error. The use of logit based AHA-units makes change comparable for persons at different ability levels.

Factors related to caregiver strain in ALS: a longitudinal study.

OBJECTIVE: To examine the longitudinal associations between caregiver strain and patients' clinical and psychosocial characteristics as well as caregivers' psychosocial characteristics. METHODS: At 4-month intervals during the 12 months study period, longitudinal data on caregiver strain and patient and caregiver factors potentially associated with caregiver strain were collected from 126 couples, who participated in a randomised controlled trial on the effectiveness of case management in amyotrophic lateral sclerosis (ALS). Caregiver strain was assessed with the Caregiver Strain Index (CSI). Patient and caregiver factors included sociodemographic characteristics, distress, coping style and perceived quality of care, as well as the patient's functional status and emotional functioning. Multilevel regression analyses were performed. RESULTS: Caregiver strain increased significantly during the study period (ß=0.315 points/months, p<0.001) and was significantly associated with patient time-dependent factors functional status (ß=-0.131 points/months, p<0.001) and emotional functioning (ß=0.022 points/months, p=0.03), and caregiver time-dependent factors passive coping style (ß=0.152 points/months, p=0.03), symptoms of anxiety (ß=0.186 points/months, p<0.001) and perceived quality of care for the caregiver (ß=-0.452 points/months, p<0.001). CONCLUSIONS: Our study has identified that apart from the patient's physical disability and emotional well-being, a passive coping style of the caregiver, increased symptoms of anxiety and feeling less supported by the ALS-team impact on caregiver strain. The multidisciplinary teams involved with the care of patients with ALS need to be aware of these factors and increase their attention for the caregiver. This will help guide the development of evidence-based supportive interventions that focus on caregiver's coping style and avoiding distress. TRIAL REGISTRATION NUMBER: Netherlands Trial Register, number NTR1270.

Development of the Assisting Hand Assessment for adolescents (Ad-AHA) and validation of the AHA from 18 months to 18 years.

AIM: To develop and evaluate a test activity from which bimanual performance in adolescents with unilateral cerebral palsy (CP) can be observed and scored with the Assisting Hand Assessment (AHA), and to evaluate the construct validity of the AHA test items for the extended age range 18 months to 18 years. METHOD: A new test activity was developed and evaluated for its ability to elicit bimanual actions in adolescents with (n=20) and without (n=10) unilateral CP. The AHA scores of 126 adolescents (mean age 14y 3mo, SD 2y 6mo; 71 males, 55 females) and 157 children with unilateral CP (mean age 6y 1mo, SD 2y 10mo; 102 males, 55 females) were analysed using the Rasch measurement model. RESULTS: The test activity elicited bimanual actions in 100% of typically developing adolescents and in 96.8% and 57.9% of adolescents with unilateral CP (moderately and severely limited hand function respectively). The scale demonstrated good construct validity; thus the same scoring criteria can be used for the age range studied. INTERPRETATION: The new Assisting Hand Assessment for adolescents (Ad-AHA) activity is valid for use with 13- to 18-year-olds to elicit bimanual performance in adolescents with unilateral CP. The same AHA scoring criteria can be used both for children and for adolescents within the age range 18 months to 18 years.

Quantitative muscle ultrasound and quadriceps strength in patients with post-polio syndrome.

INTRODUCTION: We investigated whether muscle ultrasound can distinguish muscles affected by post-polio syndrome (PPS) from healthy muscles and whether severity of ultrasound abnormalities is associated with muscle strength. METHODS: Echo intensity, muscle thickness, and isometric strength of the quadriceps muscles were measured in 48 patients with PPS and 12 healthy controls. RESULTS: Patients with PPS had significantly higher echo intensity and lower muscle thickness than healthy controls. In patients, both echo intensity and muscle thickness were associated independently with muscle strength. A combined measure of echo intensity and muscle thickness was more strongly related to muscle strength than either parameter alone. CONCLUSIONS: Quantitative ultrasound distinguishes healthy muscles from those affected by PPS, and measures of muscle quality and quantity are associated with muscle strength. Hence, ultrasound could be a useful tool for assessing disease severity and monitoring changes resulting from disease progression or clinical intervention in patients with PPS.

Treatment for postpolio syndrome.

BACKGROUND: Postpolio syndrome (PPS) may affect survivors of paralytic poliomyelitis and is characterised by a complex of neuromuscular symptoms leading to a decline in physical functioning. The effectiveness of pharmacological treatment and rehabilitation management in PPS is not yet established. This is an update of a review first published in 2011. OBJECTIVES: To systematically review the evidence from randomised and quasi-randomised controlled trials for the effect of any pharmacological or non-pharmacological treatment for PPS compared to placebo, usual care or no treatment.  SEARCH METHODS: We searched the following databases on 21 July 2014: Cochrane Neuromuscular Disease Group Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, PsycINFO and CINAHL Plus. We also checked reference lists of all relevant articles, searched the Database of Abstracts of Reviews of Effects (DARE), the Health Technology Assessment (HTA) Database and trial registers and contacted investigators known to be involved in research in this area. SELECTION CRITERIA: Randomised and quasi-randomised trials of any form of pharmacological or non-pharmacological treatment for people with PPS. The primary outcome was self perceived activity limitations and secondary outcomes were muscle strength, muscle endurance, fatigue, pain and adverse events. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by The Cochrane Collaboration. MAIN RESULTS: We included 10 pharmacological (modafinil, intravenous immunoglobulin (IVIg), pyridostigmine, lamotrigine, amantadine, prednisone) and three non-pharmacological (muscle strengthening, rehabilitation in a warm climate (that is temperature ± 25°C, dry and sunny) and a cold climate (that is temperature ± 0°C, rainy or snowy), static magnetic fields) studies with a total of 675 participants with PPS in this review. None of the included studies were completely free from any risk of bias, the most prevalent risk of bias being lack of blinding.There was moderate- and low-quality evidence that IVIg has no beneficial effect on activity limitations in the short term and long term, respectively, and inconsistency in the evidence for effectiveness on muscle strength. IVIg caused minor adverse events in a substantial proportion of the participants. Results of one trial provided very low-quality evidence that lamotrigine might be effective in reducing pain and fatigue, resulting in fewer activity limitations without generating adverse events. Data from two single trials suggested that muscle strengthening of thumb muscles (very low-quality evidence) and static magnetic fields (moderate-quality evidence) are safe and beneficial for improving muscle strength and pain, respectively, with unknown effects on activity limitations. Finally, there was evidence varying from very low quality to high quality that modafinil, pyridostigmine, amantadine, prednisone and rehabilitation in a warm or cold climate are not beneficial in PPS. AUTHORS' CONCLUSIONS: Due to insufficient good-quality data and lack of randomised studies, it was impossible to draw definite conclusions about the effectiveness of interventions for PPS. Results indicated that IVIg, lamotrigine, muscle strengthening exercises and static magnetic fields may be beneficial but need further investigation to clarify whether any real and meaningful effect exists.

Parenting Stress Related to Behavioral Problems and Disease Severity in Children with Problematic Severe Asthma.

Our study examined parenting stress and its association with behavioral problems and disease severity in children with problematic severe asthma. Research participants were 93 children (mean age 13.4 ± 2.7 years) and their parents (86 mothers, 59 fathers). As compared to reference groups analyzed in previous research, scores on the Parenting Stress Index in mothers and fathers of the children with problematic severe asthma were low. Higher parenting stress was associated with higher levels of internalizing and externalizing behavioral problems in children (Child Behavior Checklist). Higher parenting stress in mothers was also associated with higher airway inflammation (FeNO). Thus, although parenting stress was suggested to be low in this group, higher parenting stress, especially in the mother, is associated with more airway inflammation and greater child behavioral problems. This indicates the importance of focusing care in this group on all possible sources of problems, i.e., disease exacerbations and behavioral problems in the child as well as parenting stress.