eMSQOL-29: Prospective validation of the abbreviated, electronic version of MSQOL-54.

BACKGROUND: We recently devised a shortened version of the 54-item Multiple Sclerosis Quality of Life (MSQOL-54) in paper (MSQOL-29, consisting of 25 items forming 7 subscales and 4 single items, and one filter question for 3 'sexual function' items) and electronic format (eMSQOL-29). OBJECTIVES: To prospectively assess eMSQOL-29 psychometric properties, acceptability/equivalence versus MSQOL-29. METHODS: Multiple sclerosis (MS) patients ( n = 623; Expanded Disability Status Scale (EDSS) range 0.0-9.0) completed eMSQOL-29, Hospital Anxiety and Depression Scale, Functional Assessment of MS (FAMS), European Quality of life Five Dimensions-3L, and received EDSS and Symbol Digit Modalities Test (SDMT). Equivalence versus MSQOL-29 was assessed in 242 patients (randomized cross-over design). RESULTS: 'Sexual function' items were filtered out by 273 patients (47%). No multi-item scale had floor effect, while five had ceiling effect. Cronbach's alpha range was 0.88-0.90. Confirmatory factor analysis showed good overall fit and the two-factor solution for composite scores was confirmed. Criterion validity was sub-optimal for 'cognitive function' (vs SDMT, r = 0.25) and 'social function' (vs FAMS social function, r = 0.38). eMSQOL-29 equivalence was confirmed and its acceptability was good. CONCLUSION: eMSQOL-29 showed good internal consistency, factor structure and no floor effect, while most subscales had some ceiling effect. Criterion validity was sub-optimal for two subscales. Equivalence and acceptability were good.

Multimorbidity in patients with chronic migraine and medication overuse headache.

OBJECTIVES: Patients with chronic migraine (CM) display a considerable amount of comorbidities, particularly psychiatric and cardiovascular, and the presence of multiple comorbidities, that is, the so-called multimorbidity, is a risk factor for migraine chronification or maintenance of CM. Our aim was to address the rate and impact of multimorbidity in patients with CM and medication overuse headache (MOH). MATERIALS & METHODS: In a sample of patients with CM attending a structured withdrawal for coexisting MOH, we defined multimorbidity as the presence of two or more conditions in addition to CM-MOH. We compared patients with and without multimorbidity for demographic and clinical variables, quality of life, and disability; we also tested whether patients with multimorbidity had higher likelihood to attend emergency room, relapse into CM, and require further withdrawal treatments by 12 months. RESULTS: One hundred and ninety-four patients were enrolled as follows: 61% had at least one comorbidity, the most common being mental (34%), circulatory (18%), and endocrine conditions (13%); 32% were multimorbidity cases. Patients with multimorbidity had higher headaches frequency, older age, lower education and lower employment rates, higher disability and lower QoL. They were more frequently opioids/barbiturates overusers and were more likely to attend ER (OR: 2.36), relapse into CM (OR: 2.19), and undergo another withdrawal (OR: 2.75) by 12 months after discharge, after controlling for age, gender, years of education, and headache frequency. CONCLUSIONS: Recognizing multimorbidity in patients with CM-MOH is important to enhance the management of these complex patients, who are at risk of polypharmacy and increased health care utilization.

Evidence of altered pressure pain thresholds in persons with disorders of consciousness as measured by the Nociception Coma Scale-Italian version.

Pain assessment in patients with disorders of consciousness (DoC) is a controversial issue for clinicians, who require tools and standardised procedures for testing nociception in non-communicative patients. The aims of the present study were, first, to analyse the psychometric properties of the Italian version of the Nociception Coma Scale and, second, to evaluate pressure pain thresholds in a group of patients with DoC. The authors conducted a multi-centre study on 40 healthy participants and 60 DoC patients enrolled from six hospitals in Italy. For each group an electronic algometer was used to apply all nociceptive pressure stimuli. Our results show that the Italian version of the NCS retains the good psychometric properties of the original version and is therefore suitable for standardised pain assessment in clinical practice. In our study, pressure pain thresholds measured in a group of patients in vegetative and minimally conscious state were relatively lower than pain threshold values found in a group of healthy participants. Such findings motivate additional investigation on possible pain sensitisation in patients with severe brain injury and multiple co-morbidities, and on application of tailored therapeutic approaches useful for pain management in patients unable verbally to communicate their feelings.

Psychosocial difficulties in patients with Parkinson's disease.

The aim of this study was to report the most frequent psychosocial difficulties (PSDs) in patients with Parkinson disease (PD), to explore the relationship between PSDs, disability and quality of life (QoL), and to address the predictors of PSDs. Patients with PD were interviewed using a protocol composed of a questionnaire investigating PSDs (PARADISE 24), QoL, disability, comorbidity, and social support questionnaires, scales on resilience, personality traits, and empathy in physician. Most frequent PSDs were reported. Spearman's correlation was used to address the relationship between PARADISE 24 and QoL and disability measures. Multiple linear regression was performed to investigate predictors of PARADISE 24. Eighty patients were enrolled: 40% women, mean age 61.2 years. The most frequent PSDs were related to cognitive and motor slowness, tiredness, sleeping, facing all things to do, depressive mood, and anxiety. PARADISE 24 were correlated with disability (ρ=0.831) and QoL (ρ=-0.685). Lower QoL, higher disability, early age at onset, and shorter disease duration were significant predictors of PSDs (adjusted R=0.762). PARADISE 24 is an easy to use questionnaire that could contribute toward describing the impact of PD on patients' life more extensively, thus helping to define more tailored interventions.

Predictors of 12-Months Relapse After Withdrawal Treatment in Hospitalized Patients With Chronic Migraine Associated With Medication Overuse: A Longitudinal Observational Study.

BACKGROUND: Studies addressing relapse rates conflate relapse into chronic migraine (CM) and medication overuse (MO), and the consequent need to repeat withdrawal. We aim to identify 12-months predictors of relapse into CM (based on headaches frequency) separately from occurrence of another structured withdrawal. METHODS: Hospitalized patients with CM-MO under withdrawal were enrolled. Candidate predictors included demographic, disability, quality of life, depression scores, general self-efficacy, social support, headaches frequency and intensity, class of overused medications, history of withdrawal treatment in the three years prior to enrollment, attendance to emergency room (ER) between enrollment and follow-up, nonattendance to outpatient neurological examinations. Logistic regressions was used to address the significant predictors for the two outcomes. RESULTS: Complete data were available for 177 patients: 60 (33.9%) relapsed into CM, 38 (21.5%) underwent another withdrawal treatment. Recent history of withdrawal treatments, ER admission after discharge and high baseline BDI-II scores were significant predictors in both models. In addition to this, high baseline headache frequency predicted relapse into another withdrawal treatment. CONCLUSIONS: Predictors or relapse into CM and of occurrence of another withdrawal by 12-months are somehow similar. It is important to assess presence of recent previous withdrawal treatments and to plan regular follow-up afterwards, in particular for patients with high headache frequency and relevant mood disturbances: in this way, it will be more likely that situations requiring further structured withdrawal treatments can be identified before patients have to refer to ER.

Psychosocial difficulties of individuals with multiple sclerosis: the PARADISE-24 questionnaire.

The aim of this study is to identify the most relevant psychosocial difficulties (PSDs) experienced by persons with multiple sclerosis (PwMS); to evaluate the relation between an overall level of PSDs measured with the PARADISE-24 and other variables; to assess which factors may predict PARADISE-24 overall score. Adults PwMS were consecutively enrolled and completed a battery of eight self-reported instruments (i.e. SCQ, EUROHIS-QOL 8, BRS, BFI-10, JSPE, OSS-3, WHODAS-12, PARADISE-24). A clinical evaluation was performed for each patient and the Expanded Disability Status Scale (EDSS) score was determined by neurologists. A total of 80 PwMS, mean age 41.0, 65% women, 62% married, and 76.3% working, 86.3% relapsing-remitting MS, with an EDSS median score of 1.5, took part in the study. The mean disease duration was 7.7 years. The most frequently reported PSDs involved motor and emotional functioning. Free of charge access to medicines; health treatments and family, friends and health professionals' assistance represented the most important facilitators. WHODAS-12 and EUROHIS-QOL 8 were excluded from regression because of their strong correlation with PARADISE-24. EDSS, BRS, OSS-3, and neuroticism were the main predictors of the PARADISE-24 score. Despite MS heterogeneity, a common pattern of PSDs can be observed among PwMS. The level of physical impairment and personal resilience were the main predictors of the overall level of PSDs in PwMS, with a lower but significant additional role played by social support and personality traits. Attention to PSDs and their predictors, using PARADISE-24, can help clinicians to plan tailored and personalized rehabilitation programs.

Psychosocial difficulties in patients with episodic migraine: a cross-sectional study.

To explore the relationships between psychosocial difficulties (PSDs), quality of life (QoL), and disability and to explore the degree to which PSDs can be predicted by demographic variables, clinical variables, and risk and protective factors. Patients with episodic migraine completed a protocol inclusive of PARADISE 24 questionnaire (the 24-item Psychosocial Difficulties Relevant to Brain Disorders questionnaire), a new questionnaire that captures PSDs relevant to brain disorders, and assessments of disability, QoL, disease severity, presence of comorbidities, social support, and clinical and risk factors (i.e., smoking and body mass index). Spearman's correlation was used to address the relationship between PARADISE 24, and the assessments of disability and QoL; multivariable linear regression analysis was carried out to address PARADISE 24 predictors. Eighty patients were enrolled (86.3 % females, mean age 44.5). PARADISE 24 was well correlated with disability (ρ = 0.787) and moderately with QoL (ρ = -0.526). The regression analysis shows that younger age, higher migraine frequency, higher comorbidities index and being a smoker were predictors of PARADISE 24 (R 2: 0.470). Addressing the burden associated with PSDs in migraineurs is important as these might be the reason why patients look for specialists in headache disorders. PARADISE 24 represents a viable way to address patients' difficulties in daily practice.

A longitudinal ICF-CY-based evaluation of functioning and disability of children born with very low birth weight.

This paper aims to describe the longitudinal changes in disability, defined by the International Classification of Functioning, Disability, and Health - Children and Youth version (ICF-CY) biopsychosocial model, and developmental outcomes in a cohort of 56 very low birth weight children over 14-20 months. We used a neurofunctional assessment, the Griffiths Mental Development Scales-Revised: 2-8 years (Griffiths 2-8) to evaluate psychomotor development and the ICF-CY questionnaire for ages 0-3 and 3-6 to address children's disability. Extension indexes on the basis of ICF-CY categories were computed, and longitudinal change was tested. Complete follow-up was available for 55 children (mean age 36.7 months, SD 6.7). Considering the sample as a whole, neurofunctional assessment, Griffiths score and disability were basically stable. When the subsample of children with the higher baseline functioning was taken into account, some degree of worsening, in terms of an increase in the number of impairments and limitations, was found. Our results show that disability profiles, neurofunctional assessment and global development were basically stable, except for the subgroup of children who were in the intermediate/high-functioning cluster at baseline. The increased disability among these children might be because of the possibility to observe a wider set of age-specific problems, such as emotional, regulation and social abilities that are not detectable at an early stage of development and that might lead to reduced participation in social activities.

Perceived efficacy of salbutamol by persons with spinal muscular atrophy: A mixed methods study.

INTRODUCTION: The aim of this study was to assess the perceived effect of salbutamol in adult patients with spinal muscular atrophy and to evaluate the usefulness of the World Health Organization Disability Assessment Schedule II (WHODAS II) and Fatigue Severity Scale (FSS) for its measurement. METHODS: A longitudinal mixed methods study was performed. Ten patients were interviewed and completed WHODAS II and FSS questionnaires to assess disability and fatigue at 2 time-points. Inductive thematic analysis was used for qualitative data. The non-parametric Wilcoxon test was performed for quantitative analysis. RESULTS: All participants reported an improvement in their condition after salbutamol consumption. WHODAS II and FSS reliably captured changes in patients' disability and fatigue. CONCLUSIONS: The mixed methods design allowed us to identify the functional domains in which participants experienced effects of salbutamol. Patients were satisfied with the treatment as shown by decreased fatigue, improved functioning, and infrequent side effects. Muscle Nerve, 2016 Muscle Nerve 54: 843-849, 2016.

The Coma Recovery Scale Modified Score: a new scoring system for the Coma Recovery Scale-revised for assessment of patients with disorders of consciousness.

The differential diagnosis between vegetative state and minimally conscious state is still complex and the development of an evaluation systems is one of the challenging tasks for researchers and professionals. The Coma Recovery Scale-revised is considered the gold standard for clinical/behavioral assessment and for the differential diagnosis of patients with disorder of consciousness. However, the scale presents some limitations in that (i) scores may partially overlap between different diagnoses and (ii) there is an underlying assumption that if a patient is able to show higher-level behaviors, he/she is also able to show lower-level responses. In the present study, a procedure to calculate a modified Coma Recovery Scale-revised score is presented that attempts to avoid these problems. To exemplify this new scoring approach, 60 patients with disorder of consciousness were studied and the results showed the usefulness of the Modified Score.

Assessment of patients with disorder of consciousness: do different Coma Recovery Scale scoring correlate with different settings?

Differential diagnosis between Vegetative State and Minimally Conscious State is a challenging task that requires specific assessment scales, involvement of expert neuropsychologists or physicians and use of tailored stimuli for eliciting behavioural responses. Although misdiagnosis rate as high as 40% has been reported, no clear guidelines are available in literature on the optimal setting for assessment. The present study aims to analyse score differences in behavioural assessments of persons with disorders of consciousness (DOC) with or without family members and to determine whether the presence of caregivers could improve clinical accuracy in diagnostic evaluation. The research was conducted on 92 adults with DOC among 153 consecutive patients enrolled in the Coma Research Centre of the Neurological Institute C. Besta of Milan between January 2011 and May 2013. The results indicate that in almost half of the sample the scoring, thus the performance, observed with caregivers was better than without them. Furthermore, in 16% of the sample, when assessment was performed with caregivers there was a change in diagnosis, from Vegetative to Minimally Conscious State or from that to Severe Disability. Finally, statistical differences were found in relation to diagnosis between mean scores in the "visual function" Coma Recovery Scale revised's subscale obtained by raters plus caregiver and rates only assessment. This study demonstrates how the presence of caregivers can positively affect behavioural assessments of persons with DOC, thus contributing to the definition of the optimal setting for behavioural evaluation of patients, to decrease misdiagnosis rates.

ICF-DOC: the ICF dedicated checklist for evaluating functioning and disability in people with disorders of consciousness.

Clinicians need a comprehensive description of patients' functioning state to capture the complex interaction between symptoms and environmental factors, and to determine the actual level of functioning in patients in a vegetative state or a minimally conscious state. The aim of this study is to develop an International Classification of Functioning, Disability, and Health (ICF) checklist for patients with disorders of consciousness (DOC) so as to capture and describe, with a tailored list of categories, the most common health, disability, and functioning issues of adult patients with DOC. The WHO ICF checklist was used as a basis for collecting data. This was an observational, cross-sectional, multicenter study conducted in 69 Italian centers. Specific methodological procedures were used to identify the most appropriate categories for DOC patients to be added to or deleted from the ICF checklist so as to develop the ICF-DOC checklist. A total of 566 adult patients were enrolled: 398 in a vegetative state and 168 in a minimally conscious state. A total of 127 ICF categories reached the threshold of 20% concerning the presence of a problem: 37 categories from the body functions chapter, 13 from the body structures chapter, 46 from the activities and participations chapter, and 31 from the environmental factors chapter. ICF categories identified in this study can be useful guidelines for clinicians and researchers to collect data on functioning and disability of adult patients with DOC. The new ICF-DOC checklist allows monitoring of the effects of interventions on functional areas and possible changes in each patient in follow-up studies.

The Italian version of the Nociception Coma Scale.

Pain assessment and management represent a crucial issue in planning rehabilitative programmes and pharmacological therapies in chronic patients. In noncompetent and nonverbally communicative individuals, as for patients in a vegetative state or minimally conscious state, the evaluation of the presence and intensity of pain is often complex. Validated scales and instruments to standardize the observation of patients' behavioural and motor responses to noxious stimuli are needed. The aim of this study is to translate the Nociception Coma Scale from English into Italian. The process of translation and back translation involved four translators and two expert raters. No item received quality ratings below 90 (range 0-100). The Italian version of the Nociception Coma Scale is now available for Italian clinicians and researchers. It will be useful for an accurate management and care planning in rehabilitative and long-term care centres in Italy.

Factors related to difficulties with employment in patients with multiple sclerosis: a review of 2002-2011 literature.

We assess the knowledge available on the difficulties experienced by multiple sclerosis (MS) patients in work-related activities. A literature review was carried out using the keywords 'multiple sclerosis' and 'employment' or 'work' through PubMed and EMBASE. Papers reporting patient-derived data on difficulties at work as primary or secondary outcome measures and published in the period 2002-December 2011 were searched. A total of 26 papers were selected, for a total of 32 507 patients (mean age 46.2 years; 42.1% with relapsing-remitting MS). Most papers reported observational studies or cross-sectional surveys focused on health-related quality of life and MS costs. Symptoms more frequently addressed are fatigue, mobility and cognitive impairments. Limited research has been carried out on the working environment. We found a relatively small number of papers published in the last 10 years on the difficulties that patients with MS can experience at work, and this kind of information always appeared as a secondary outcome. In general, it is possible to affirm that MS has a strong impact on patients' employment status, as the mean unemployment rate was 59%. Research on factors promoting maintenance of remunerative employment is required.

Psychosocial difficulties in people with epilepsy: a systematic review of literature from 2005 until 2010.

The aims of this paper are to identify factors that influence the psychosocial difficulties (PSDs) that persons with epilepsy experience in their everyday life, to describe their onset and the way they evolve over time, and to analyze the determinants of changes over time and other related variables. Electronic databases were searched for studies published in English between January 2005 and May 2010, and information from thirteen studies was extracted. The most frequent PSDs found in people with epilepsy were depressive symptoms, memory functions, quality of life, anxiety, stigma, locus of control, cognitive functions in general, and emotional functions in general. It can be stated that patients' life areas are affected by cognitive, emotional, and psychological problems. However, the majority of studies focus on isolated PSDs or on the effects of a specific determinant in the course of epilepsy, leaving some gaps that could encourage further research.

Functioning and disability of children and adolescents in a vegetative state and a minimally conscious state: identification of ICF-CY-relevant categories.

Children in a vegetative state (VS) and a minimally conscious state (MCS) experience severe limitations as a consequence of nervous system deficits and require consistent environmental support. However, disability in VS and MCS children has never been described following a model that accounts for the presence of the symptoms, limitations and the support required. Therefore, the aim of this paper is to describe the functioning and disability of children in VS and MCS using the International Classification of Functioning, Disability and Health - version for Children and Youth (ICF-CY). VS and MCS children were enrolled in postacute settings and at home. ICF-CY questionnaires were filled in using information available from clinical documentation, direct observation and from children's parents. ICF-CY categories were considered as relevant if used in at least one-third of the children. In total, 36 children and adolescents (22 in VS, 25 males) were enrolled. The majority developed VS and MCS following a nontraumatic event; the mean age was 114.8 months and the mean duration of condition was 50.1 months. A total of 94 ICF-CY categories were reported as relevant: 26 were from body functions, mostly from mental functions and mobility chapters; nine from body structures, 32 from activities and participation, mostly from learning, mobility and self-care chapters; and 27 from environmental factors. The use of ICF-CY enables to obtain a specific profile of functioning for each child that can be coupled with known issues, such as loss of brain functions and provision of life-sustaining interventions.