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Objectives: The purpose of this study was to determine if intraindividual variability would be more sensitive than speed or accuracy in detecting subtle cancer-related cognitive disturbance. Methods: Data were from a previous study in which 60 breast cancer (BC) patients underwent neuropsychological assessment before commencement of chemotherapy and again following each chemotherapy cycle. Sixty healthy controls were tested at equivalent intervals. Hierarchical linear modeling was used to compare the BC and control groups in terms of accuracy, mean reaction time, and intraindividual variability in reaction time on a computerized continuous performance test with three conditions: a simple reaction time task, a “1-back” task, and a “2-back” task. Results: An increase in accuracy and response speed over sessions was noted on some tasks in the sample as a whole but there were no differences in these parameters between the BC patients and the controls on any condition. There was a significant group difference in change in intraindividual variability across sessions (i.e., a “group × session interaction”), albeit only on the most complex “2-back” task. Intraindividual variability declined in the control group (i.e., consistency improved with practice) but this practice effect was significantly attenuated in the BC patients. There was no main effect of group on the “2-back” task. Conclusions: Results support our hypothesis that intraindividual variability is a more sensitive indicator of subtle cognitive disturbance than conventional speed or accuracy measures and may have potential in the assessment of mild cognitive impairment in patients with non-central nervous system cancers. (JINS, 2018, 24, 1–11)

Numerous studies have shown that there are acute cognitive side-effects of chemotherapy for breast cancer. Presumably, patients are more concerned about chronic treatment effects. This report from a prospective longitudinal study compares cognitive functioning in 56 breast cancer patients 1 year after chemotherapy to that of 56 healthy individuals. Neuropsychological test scores were combined into verbal memory, visual memory, working memory, and processing speed scores, as well as an overall summary score, and analyzed using multi-level growth modeling. Frequency of cognitive decline was assessed using regression-based change scores. There was significant rebound in the overall summary score from end of treatment to 1-year follow-up as well as a substantial reduction in the frequency of cognitive decline. However, more than one-third of the breast cancer patients who showed cognitive decline immediately following completion of chemotherapy showed persistent cognitive decline 1 year later. Furthermore, recovery was not seen in all cognitive domains. In fact, the rebound was significant only for working memory. Longer multi-site studies are recommended to explore the risk factors for and the permanence of these longer-term cognitive effects. (JINS, 2013, 20, 1–10)

BackgroundThe diagnosis of facioscapulohumeral muscular dystrophy (FSHD) can be challenging in patients not displaying the classical phenotype or with atypical clinical features. Despite the identification by magnetic resonance imaging (MRI) of selective patterns of muscle involvement, their specificity and added diagnostic value are unknown.MethodsWe aimed to identify the radiological features more useful to distinguish FSHD from other myopathies and test the diagnostic accuracy of MRI. A retrospective cohort of 295 patients (187 FSHD, 108 non-FSHD) studied by upper and lower-limb muscle MRI was analyzed. Scans were evaluated for the presence of 15 radiological features. A random forest machine learning algorithm was used to identify the most relevant for FSHD diagnosis. Different patterns were created by their combination and diagnostic accuracy of each of them was tested.ResultsThe combination of trapezius involvement and bilateral subscapularis muscle sparing achieved the best diagnostic accuracy (0.89, 95% Confidence Interval [0.85–0.92]) with 0.90 [0.85–0.94] sensitivity and 0.88 [0.80–0.93] specificity. This pattern correctly identified 91% atypical FSHD patients of our cohort. The combination of trapezius involvement, bilateral subscapularis and iliopsoas sparing and asymmetric involvement of upper and lower-limb muscles was pathognomonic for FSHD, yielding a specificity of 0.99 [0.95–1.00].ConclusionsWe identified MRI patterns that showed a high diagnostic power in promptly discriminating FSHD from other muscle disorders, with comparable performance irrespective of typical or atypical clinical features. Upper girdle in addition to lower-limb muscle imaging should be extensively implemented in the diagnostic workup to support or exclude a diagnosis of FSHD.

PurposeFew studies have examined how psychosocial risk and protective factors in adolescence shape mental health outcomes and other multimorbid conditions in adulthood, particularly among Canadian youth. The Research on Eating and Adolescent Lifestyle (REAL) 2.0 study was a 15-year follow-up cohort study designed to investigate how early etiological factors, including body image and disordered eating symptoms in adolescence, contribute to the development of eating, weight-related concerns, mental health and substance use health problems in early adulthood. In this paper, we describe the REAL 2.0 cohort’s demographic and clinical characteristics alongside an overview of the study procedures, laying the groundwork for collaboration on future learnings with this unique data.ParticipantsThe cross-sectional REAL study initially surveyed middle and high school students from 2004 to 2010 (n=3043) across 43 schools in the Ottawa, Canada region. Of those, respondents in grade 7 or 9 (n=1197 from 25 of the 43 original schools) were asked to participate in a longitudinal arm of the study that consisted of yearly follow-ups. From the longitudinal cohort, there were 278 participants (29.1% male; Mage=28.6) from those who consented to be re-contacted (n=912), who completed the REAL 2.0 survey electronically (30.4%), providing comprehensive data on demographic, clinical, eating and weight-related behaviour, psychological, social, environmental and substance use health factors in adulthood.Findings to date9.4% of REAL 2.0 participants met DSM-5 criteria for an eating disorder, while 17.6% met criteria for disordered eating. Moderate to severe anxiety was reported by 28% of participants, while 21.6% experienced moderate to severe depressive symptoms. Regarding substance use, 16.9% engaged in hazardous drinking, 16.9% used cannabis daily or almost daily, and 4.3% reported daily tobacco use.Future plansREAL 2.0 has the potential to answer multiple research questions about several mental health outcomes, but its priority focus is to answer questions related to risk and protective factors of multimorbidity in adulthood. Additionally, profiling work, linked to health service utilisation data for systems planning work and predictive modelling studies are secondary goals. By leveraging the Health Data Nexus (HDN) platform, we welcome collaboration with interested researchers who would like to utilise the breadth of data both in adolescence and adulthood to answer other pertinent aetiological questions in mental health and substance use health outcomes. Future plans to conduct additional follow-ups remain feasible.

Quantitative muscle MRI (water-T2 and fat mapping) is being increasingly used to assess disease involvement in muscle disorders, while imaging techniques for assessment of the dynamic and elastic muscle properties have not yet been translated into clinics. In this exploratory study, we quantitatively characterized muscle deformation (strain) in patients affected by facioscapulohumeral muscular dystrophy (FSHD), a prevalent muscular dystrophy, by applying dynamic MRI synchronized with neuromuscular electrical stimulation (NMES). We evaluated the quadriceps muscles in 34 ambulatory patients and 13 healthy controls, at 6-to 12-month time intervals. While a subgroup of patients behaved similarly to controls, for another subgroup the median strain decreased over time (approximately 57% over 1.5 years). Dynamic MRI parameters did not correlate with quantitative MRI. Our results suggest that the evaluation of muscle contraction by NMES-MRI is feasible and could potentially be used to explore the elastic properties and monitor muscle involvement in FSHD and other neuromuscular disorders.

Facioscapulohumeral muscular dystrophy (FSHD) is one of the most common muscular dystrophies and is characterized by a non-conventional genetic mechanism activated by pathogenic D4Z4 repeat contractions. By muscle Magnetic Resonance Imaging (MRI) we observed that T2-short tau inversion recovery (T2-STIR) sequences identify two different conditions in which each muscle can be found before the irreversible dystrophic alteration, marked as T1-weighted sequence hyperintensity, takes place. We studied these conditions in order to obtain further information on the molecular mechanisms involved in the selective wasting of single muscles or muscle groups in this disease. Histopathology, gene expression profiling and real time PCR were performed on biopsies from FSHD muscles with different MRI pattern (T1-weighted normal/T2-STIR normal and T1-weighted normal/T2-STIR hyperintense). Data were compared with those from inflammatory myopathies, dysferlinopathies and normal controls. In order to validate obtained results, two additional FSHD samples with different MRI pattern were analyzed. Myopathic and inflammatory changes characterized T2-STIR hyperintense FSHD muscles, at variance with T2-STIR normal muscles. These two states could be easily distinguished from each other by their transcriptional profile. The comparison between T2-STIR hyperintense FSHD muscles and inflammatory myopathy muscles showed peculiar changes, although many alterations were shared among these conditions. At the single muscle level, different stages of the disease correspond to the two MRI patterns. T2-STIR hyperintense FSHD muscles are more similar to inflammatory myopathies than to T2-STIR normal FSHD muscles or other muscular dystrophies, and share with them upregulation of genes involved in innate and adaptive immunity. Our data suggest that selective inflammation, together with perturbation in biological processes such as neoangiogenesis, lipid metabolism and adipokine production, may contribute to the sequential bursts of muscle degeneration that involve individual muscles in an asynchronous manner in this disease.

Bjarne Udd and colleagues show that mutations affecting the cytoplasmic functions of the co-chaperone DNAJB6 result in limb-girdle muscular dystrophy. Their studies suggest that the mutations reduce the protective anti-aggregation effects of DNAJB6, leading to protein accumulation and autophagic pathology. Limb-girdle muscular dystrophy type 1D (LGMD1D) was linked to chromosome 7q36 over a decade ago 1 , but its genetic cause has remained elusive. Here we studied nine LGMD-affected families from Finland, the United States and Italy and identified four dominant missense mutations leading to p.Phe93Leu or p.Phe89Ile changes in the ubiquitously expressed co-chaperone DNAJB6. Functional testing in vivo showed that the mutations have a dominant toxic effect mediated specifically by the cytoplasmic isoform of DNAJB6. In vitro studies demonstrated that the mutations increase the half-life of DNAJB6, extending this effect to the wild-type protein, and reduce its protective anti-aggregation effect. Further, we show that DNAJB6 interacts with members of the CASA complex, including the myofibrillar myopathy–causing protein BAG3. Our data identify the genetic cause of LGMD1D, suggest that its pathogenesis is mediated by defective chaperone function and highlight how mutations in a ubiquitously expressed gene can exert effects in a tissue-, isoform- and cellular compartment–specific manner.

Only few studies have reported muscle involvement in spinal muscular atrophy using muscle MRI but this has not been systematically investigated in a large cohort of both pediatric and adult patients with type 2 and type 3 spinal muscular atrophy. The aim of the present study was to define possible patterns of muscle involvement on MRI, assessing both fatty replacement and muscle atrophy, in a cohort of type 2 and type 3 spinal muscular atrophy children and adults (age range 2–45 years), including both ambulant and non-ambulant patients. Muscle MRI protocol consisted in T1-weighted sequences acquired on axial plane covering the pelvis, the thigh, and the leg with contiguous slices. Each muscle was examined through its whole extension using a grading system that allows a semiquantitative evaluation of fatty infiltration. Thigh muscles were also grouped in anterior, posterior, and medial compartment for classification of global atrophy. The results showed a large variability in both type 2 and type 3 spinal muscular atrophy, with a various degree of proximal to distal gradient. Some muscles, such us the adductor longus and gracilis were always selectively spared. In all patients, the involvement was a combination of muscle atrophy and muscle infiltration. The variability observed may help to better understand both natural history and response to new treatments.

Systematic evaluation of patient outcome and progress is important for determining the effects of psychotherapy and for providing quality information to therapists to adjust treatment. In this report, we reviewed the research on outcome and progress monitoring, including barriers to their implementation. The research indicates that outcome monitoring (the assessment, at both intake and at the cessation of treatment, of patient functioning), and progress monitoring (repeated assessment of patient progress during therapy with continuous feedback to the therapist on the patient's status) by using psychometrically sound instruments, has the potential to benefit both psychological service providers and the populations they serve. However, despite ethical imperatives to do so, the majority of psychotherapists in North America currently do not assess patient outcome or progress. Barriers to implementing outcome or progress monitoring include insufficient knowledge, insufficient training, and practical concerns. The Canadian Psychological Association Task Force on Outcome and Progress Monitoring in Psychotherapy recommends widespread implementation of outcome and progress monitoring in clinical contexts including public and privately funded practices; that psychologists use the principles outlined in this report to advocate for adequate implementation and uptake of outcome and progress monitoring in their places of practice; that outcome and progress monitoring be part of the clinical training curriculum and of accreditation standards of clinical training programs; and that the Canadian Code of Ethics for Psychologists state more explicitly that psychologists monitor patient outcomes and progress. Une évaluation systématique des résultats et progrès des patients est essentielle pour déterminer les effets de la psychothérapie et fournir des renseignements de qualité aux thérapeutes afin que ces derniers puissent ajuster les traitements. Dans le présent rapport, nous avons examiné les recherches sur le suivi des résultats et progrès, y compris les obstacles à leur mise en œuvre. La recherche indique que le suivi des résultats (l'évaluation, tant au début qu'à la fin du traitement, du fonctionnement du patient), et le suivi des progrès (évaluation répétée de la progression du patient pendant le traitement avec une rétroaction continue au thérapeute sur l'état du patient) à l'aide d'instruments fiables sur le plan psychométrique, ont le potentiel de profiter à la fois aux fournisseurs de services psychologiques et aux populations qu'ils servent. Or, malgré les impératifs éthiques que cela pose, la majorité des psychothérapeutes en Amérique du Nord n'évaluent pas les résultats et progrès des patients à l'heure actuelle. Les obstacles à la mise en œuvre de processus de suivi des résultats et progrès incluent notamment, le manque de connaissances, le manque de formation, et des préoccupations d'ordre pratique. Le groupe de travail de l'Association canadienne de psychologie sur le suivi des résultats et progrès en psychothérapie recommande une mise en œuvre généralisée de suivis des résultats et progrès dans des contextes cliniques financés par les secteurs privé et public; que les psychologues utilisent les principes énoncés dans le présent rapport afin de plaider pour la mise en œuvre et l'adoption de suivis des résultats et progrès dans leurs lieux de pratique; que ces suivis des résultats et progrès fassent partie du programme de formation clinique et de normes d'agrément des programmes de formation clinique; et que le Code canadien de déontologie professionnelle des psychologues énonce de manière plus explicite que les psychologues effectuent un suivi des résultats et progrès des patients. Public Significance Statement Assessing patient outcomes, regularly monitoring patient progress, and feeding the information back to clinicians has the potential to improve patient mental health following psychological interventions. However, many clinicians do not regularly assess patient outcomes or monitor progress. Increasing psychotherapists' knowledge, giving them ongoing training, sensitizing clinicians to the ethics of outcome and progress monitoring, and providing practical support in places of work will increase the use of these methods among psychological providers.