(AAN) is charged with developing practice parameters for physicians. This evidence-based review addresses some of the major management issues in patients with ALS, and highlights the many areas in which more research is needed.Justification. ALS is a progressive, degenerative motor neuron disease of unknown cause. Muscle atrophy and spasticity in limb and bulbar muscles result in weakness and loss of ambulation, oropharyngeal dysfunction, weight loss, and ultimately respiratory failure. Although advances in understanding the pathophysiology of ALS have stimulated the development of new drug therapies, 1 the mainstay of treatment for ALS patients remains symptomatic management.The practice parameters presented here comprise the first recommendations for the management of ALS based on a prescribed review and analysis of the peer-reviewed literature. These practice parameters were developed to improve the care and the quality of life of people with ALS by providing a rational basis for managing the disease.Description of the process. A multidisciplinary task force, all with extensive ALS experience, included 19 physicians, 3 patients with ALS, 1 gastroenterologist, 1 pulmonologist, 1 occupational therapist whose mother has ALS, and 1 nurse. In addition, consultants with expertise on ethics, practice parameter development, and medical library research participated in the process. The task force agreed to investigate five areas: 1) informing the patient and the family about the diagnosis and prognosis (also called "breaking the news") of ALS; 2) symptomatic treatment; 3) nutrition, and decisions about percutaneous endoscopic gastroscopy (PEG); 4) respiratory insufficiency and mechanical ventilation; and 5) advance directives and palliative care. To help achieve this goal, they developed several guiding principles or attributes of care: Principles of ALS management1. High priority should be placed on patient self-determination or autonomy as an underlying assumption in the therapeutic relationship. Delivery of both information and care must take into consideration the cultural and psychosocial context of the patient and the family. 2. Patients and families need information that is timed appropriately for decision making, and delivered well in advance of major management crossroads, especially for respiratory care. Moreover, decision making is a dynamic process that may be subject to change as the disease becomes more severe.The investigators and institutions of the ALS Practice Parameters Task Force are listed in the Appendix on page 1320.

Inevitably there are some omissions m such a document and many of the symptoms of patients with ALS are not addressed m this first document but will be taken up m future iterations. Of particular importance m the document is the evidence-based recommendation about the value of percutaneous endoscopic gastrostomy and the importance of timmg m presentmg this option to patients. Similarly, strong evidence-based recommendations are made about the utility of noninvasive mechanical ventilation and the beneficial impact that this has on symptoms of hypoventilation and on survival. Finally, management of patients m the termmal phase of the disease usmg the highest standard of palliative care is discussed m some detail. Perhaps one of the most important contributions of this document is the highlighting of areas where further research is needed. This has been done m each of the major sections of the paper. More rigorous clinical investigations are still needed to help answer some of the most pressmg management issues for patients with ALS. Mission Statement The Quality Standards Subcommittee of the American Academy of Neurology (AAN) is charged with developmg practice parameters for physicians. This evidence-based review addresses some of the major management issues m patients with amyotrophic lateral sclerosis (ALS), and highlights the many areas where more research is needed.

The Quality Standards Subcommittee (QSS) of the American Academy of Neurology (AAN) is charged with developing practice parameters for physicians. This evidence-based review addresses some of the major management issues in patients with ALS, and highlights the many areas in which more research is needed.

The type and quality of end-of-life care varies greatly in ALS; the time to initiate end-of-life care is not defined, and decision making is hampered by logistical and financial barriers. There has been no systematic review of these issues in ALS. The goals of this initiative are to: 1) improve end-of-life care for patients with ALS and families based on what limited evidence is available; 2) increase awareness, interest, and debate on the end-of-life care in ALS; and 3) identify areas needed for new prospective clinical research. The ALS Peer Workgroup reviewed the literature and 1) identified the current state of knowledge, 2) analysed the gaps in care, and 3) provided recommendations for standard of care and future research. It was shown that areas of investigation are needed on the incorporation of an interdisciplinary approach to care in ALS that includes: psychosocial evaluation and spiritual care; the use of validated instruments to assess patient and caregiver quality of life; and the establishment of proactive caregiver programs. Several public policy changes that will improve coverage for medical care, hospice, and caregiver costs are also reviewed. More clinical evidence is needed on how to provide optimal end-of-life care specifically in ALS.

The clinical presentation of amyotrophic lateral sclerosis (ALS) is variable and overlaps with that of other motor neuron diseases such as spinobulbar muscular atrophy (SBMA; Kennedy disease). With the identification of disease-specific mutations such as the CAG repeat expansion in the androgen receptor in SBMA, an accurate molecular diagnosis can be made in some patients with motor neuron disease. To determine the extent of misdiagnosis of ALS we screened 147 male ALS patients and 100 unrelated male patients from 100 familial ALS (FALS) kindreds for the presence of the SBMA mutation using polymerase chain reaction methods. We show that ALS was clinically misdiagnosed in 2% of sporadic cases and in two of the 100 FALS kindreds. This study underscores the difficulty in distinguishing SBMA from ALS clinically, particularly in patients who lack the classic signs of each disease.

No abstract

Objective The objective of this study was to assess the impact of systemic lupus erythematosus (SLE) on patients and carers. Methods Adults with SLE and carers of SLE patients completed a UK-specific online survey covering many aspects of the disease. Surveys were developed in collaboration with an NHS lupus unit and a lupus patient organization. Results A total of 121 patients and 31 carers completed the surveys. Of the 70% of patients initially misdiagnosed with another condition, 59% received treatment for the misdiagnosis. Fatigue was the most debilitating symptom, experienced daily by 79% of patients. The proportion of patients not reporting flares to healthcare providers varied with flare severity: mild flares (43%), moderate flares (15%) and severe flares (5%). Most patients (89%) reported reduced ability to socialize, and 76% had changed employment; of these, 52% stopped working completely. Over one-half (52%) of carers in paid employment missed time from work, and 55% of carers reported a worsened financial status. Most carers (87%) experienced interference with social activities. Conclusion SLE is commonly misdiagnosed and has a considerable impact on the physical, social and financial status of patients and carers. Increased awareness of the disease among healthcare providers and employers of patients and their carers is needed.

Effective supervision models guide the supervisory relationship and supervisory tasks leading to reflective and purposeful practice. The Developmental/Ecological/Problem-Solving (DEP) Model provides a contemporary framework for supervision specific to school psychology. Designed for the school psychology internship, the DEP Model is also applicable to all pre-service and advanced field-based training, as well as career-long continuing professional development. The Developmental domain initiates training at the functioning skill level of the supervisee and progresses toward independent competency. The Ecological domain addresses the multiple systemic contexts that influence school psychology practice and prepares the intern to intervene within both individual and systemic contexts. The Problem-Solving domain focuses on the application of data-based decision making and evidence-based interventions to the full range of school psychology activities. It provides a systematic schema to address student, family, and school needs. C 2014 Wiley Periodicals, Inc.Rather than merely assuming that competent practitioners are automatically effective supervisors, the field of clinical supervision has begun to examine the specific factors that contribute to successful supervisory experiences (Harvey & Struzziero, 2008). Domains being examined include organizational structures such as pre-training skill assessments and goal setting, formal contracts, advance planning for the content and process of supervision, and accountable recordkeeping; the character of the supervisory relationship; the utilization of multiple methods of supervision; the nature of effective feedback and evaluation; standards and methods to ensure multicultural competency; specific training for supervisees toward eventual assumption of professional supervisory roles; and attention to legal and ethical requirements, including those developed by state psychology licensing boards, the American Psychological Association (APA), and professional organizations specific to school psychology, including the National Association of School Psychologists (NASP, 2010b) and the Council of Directors of School Psychology Programs (2012).To integrate these diverse factors into a framework that can guide supervisors in the day-to-day provision of effective supervision, it is necessary to formulate a coherent supervisory model that provides an overall conceptual organization of the supervisory process. A supervisory model grounds supervision in reflective and purposeful practice. A comprehensive model strives to conceptualize, organize, and execute supervisory tasks and functions in a manner that links theory, emerging research, and practice. Although this overarching model guides supervisory practice, it must be firmly grounded in the profession's current understanding of best practices within school psychology. When the formulation of this organizing model is shared with the trainee, it provides a transparent Correspondence to: