Diabetic neuropathies.

Diabetic neuropathies are the most common type of neuropathies seen in clinical practice. These neuropathies can range clinically from asymptomatic to manifesting symptoms caused by motor, sensory, and autonomic nerve dysfunction. These neuropathies can affect the peripheral nervous system, pain receptors, cardiovascular, urogenital, and gastrointestinal systems. This monograph presents an overview of the different types of diabetic neuropathies, their presentations, diagnostic tools, and strategies for management.

Development of a taxonomy of unprofessional behavior in clinical learning environments using learner-generated critical incidents.

PURPOSE: Few studies have examined medical residents' and fellows' (trainees) direct experience of unprofessional behavior in clinical learning environments (CLE). The purpose of this study was to create a taxonomy of unprofessional behavior in CLEs using critical incidents gathered from trainees. METHOD: In step 1 (data collection), the authors collected 382 critical incidents from trainees at more than a dozen CLEs over a six-year period (2013-2019). In step 2 (model generation), nine subject matter experts (SMEs) sorted the incidents into homogenous clusters and this structure was tested with principal components analysis (PCA). In step 3 (model evaluation), two new groups of SMEs each re-sorted half of the incidents into the PCA-derived categories. RESULTS: A 13-component solution accounted for 62.46% of the variance in the critical incidents collected. The SMEs who re-sorted the critical incidents demonstrated good agreement with each other and with the 13-component PCA solution. The resulting taxonomy included 13 dimensions, with 48.7% of behaviors focused on displays of aggression or discriminatory conduct. CONCLUSIONS: Critical incident methodology can provide unique insights into the dimensionality of unprofessional behavior in the CLE. Future research should leverage the taxonomy created to inform professionalism assessment development in the CLE.

Improving Our Ability to Predict Resident Applicant Performance: Validity Evidence for a Situational Judgment Test.

Construct: We investigated whether a situational judgment test (SJT) designed to measure professionalism in physicians predicts residents' performance on (a) Accreditation Council for Graduate Medical Education (ACGME) competencies and (b) a multisource professionalism assessment (MPA). Background: There is a consensus regarding the importance of assessing professionalism and interpersonal and communication skills in medical students, residents, and practicing physicians. Nonetheless, these noncognitive competencies are not well measured during medical education selection processes. One promising method for measuring these noncognitive competencies is the SJT. In a typical SJT, respondents are presented with written or video-based scenarios and asked to make choices from a set of alternative courses of action. Interpersonally oriented SJTs are commonly used for selection to medical schools in the United Kingdom and Belgium and for postgraduate selection of trainees to medical practice in Belgium, Singapore, Canada, and Australia. However, despite international evidence suggesting that SJTs are useful predictors of in-training performance, end-of-training performance, supervisory ratings of performance, and clinical skills licensing objective structured clinical examinations, the use of interpersonally oriented SJTs in residency settings in the United States has been infrequently investigated. The purpose of this study was to investigate whether residents' performance on an SJT designed to measure professionalism-related competencies-conscientiousness, integrity, accountability, aspiring to excellence, teamwork, stress tolerance, and patient-centered care-predicts both their current and future performance as residents on two important but conceptually distinct criteria: ACGME competencies and the MPA. Approach: We developed an SJT to measure seven dimensions of professionalism. During calendar year 2017, 21 residency programs from 2 institutions administered the SJT. We conducted analyses to determine the validity of SJT and USMLE scores in predicting milestone performance in ACGME core competency domains and the MPA in June 2017 and 3 months later in September 2017 for the MPA and 1 year later, in June 2018, for ACGME domains. Results: At both periods, the SJT score predicted overall ACGME milestone performance (r = .13 and .17, respectively; p < .05) and MPA performance (r = .19 and .21, respectively; p < .05). In addition, the SJT predicted ACGME patient care, systems-based practice, practice-based learning and improvement, interpersonal and communication skills, and professionalism competencies (r = .16, .15, .15, .17, and .16, respectively; p < .05) 1 year later. The SJT score contributed incremental validity over USMLE scores in predicting overall ACGME milestone performance (ΔR = .07) 1 year later and MPA performance (ΔR = .05) 3 months later. Conclusions: SJTs show promise as a method for assessing noncognitive attributes in residency program applicants. The SJT's incremental validity to the USMLE series in this study underscores the importance of moving beyond these standardized tests to a more holistic review of candidates that includes both cognitive and noncognitive measures.

Amyotrophic lateral sclerosis-specific quality of life-short form (ALSSQOL-SF): A brief, reliable, and valid version of the ALSSQOL-R.

INTRODUCTION: The Amyotrophic Lateral Sclerosis (ALS)-Specific Quality of Life instrument and its revised version (ALSSQOL and ALSSQOL-R) have strong psychometric properties, and have demonstrated research and clinical utility. In this study we aimed to develop a short form (ALSSQOL-SF) suitable for limited clinic time and patient stamina. METHODS: The ALSSQOL-SF was created using Item Response Theory and confirmatory factor analysis on 389 patients. A cross-validation sample of 162 patients assessed convergent, divergent, and construct validity of the ALSSQOL-SF compared with psychosocial and physical functioning measures. RESULTS: The ALSSQOL-SF consisted of 20 items. Compared with the ALSSQOL-R, optimal precision was retained, and completion time was reduced from 15-25 minutes to 2-4 minutes. Psychometric properties for the ALSSQOL-SF and its subscales were strong. DISCUSSION: The ALSSQOL-SF is a disease-specific global QOL instrument that has a short administration time suitable for clinical use, and can provide clinically useful, valid information about persons with ALS. Muscle Nerve 58: 646-654, 2018.

Shared decision-making in multiple sclerosis.

In a healthcare environment that is trying to achieve better clinical outcomes and patient satisfaction, shared decision-making is a well-established concept that is gaining more interest. Multiple sclerosis is a preference-sensitive condition and provides the opportunity to implement decision aids at various decision points in the disease process. Literature about patient education and outcomes of shared decision aids in multiple sclerosis has been growing over the last decade. In this topical review, we present an overview of the current literature on shared decision-making in multiple sclerosis. While limitations to the generalizability and applicability of decision aids exist, there is evidence that decision aids and shared decision-making can be valuable tools in the clinical care of multiple sclerosis patients.

Not all unprofessional behaviors are equal: The creation of a checklist of bad behaviors.

INTRODUCTION: Professionalism is a key component of medical education and training. However, there are few tools to aid educators in diagnosing unprofessional behavior at an early stage. The purpose of this study was to employ policy capturing methodology to develop two empirically validated checklists for identifying professionalism issues in early-career physicians. METHOD: In a series of workshops, a professionalism competency model containing 74 positive and 70 negative professionalism behaviors was developed and validated. Subsequently, 23 subject matter experts indicated their level of concern if each negative behavior occurred 1, 2, 3, 4, or 5 or more times during a six-month period. These ratings were used to create a "brief" and "extended" professionalism checklist for monitoring physician misconduct. RESULTS: This study confirmed the subjective impression that some unprofessional behaviors are more egregious than others. Fourteen negative behaviors (e.g. displaying obvious signs of substance abuse) were judged to be concerning if they occurred only once, whereas many others (e.g. arriving late for conferences) were judged to be concerning only when they occurred repeatedly. DISCUSSION: Medical educators can use the professionalism checklists developed in this study to aid in the early identification and subsequent remediation of unprofessional behavior in medical students and residents.

Phase II trial of CoQ10 for ALS finds insufficient evidence to justify phase III.

OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is a devastating, and currently incurable, neuromuscular disease in which oxidative stress and mitochondrial impairment are contributing to neuronal loss. Coenzyme Q10 (CoQ10), an antioxidant and mitochondrial cofactor, has shown promise in ALS transgenic mice, and in clinical trials for neurodegenerative diseases other than ALS. Our aims were to choose between two high doses of CoQ10 for ALS, and to determine if it merits testing in a Phase III clinical trial. METHODS: We designed and implemented a multicenter trial with an adaptive, two-stage, bias-adjusted, randomized, placebo-controlled, double-blind, Phase II design (n = 185). The primary outcome in both stages was a decline in the ALS Functional Rating Scale-revised (ALSFRSr) score over 9 months. Stage 1 (dose selection, 35 participants per group) compared CoQ10 doses of 1,800 and 2,700 mg/day. Stage 2 (futility test, 75 patients per group) compared the dose selected in Stage 1 against placebo. RESULTS: Stage 1 selected the 2,700 mg dose. In Stage 2, the pre-specified primary null hypothesis that this dose is superior to placebo was not rejected. It was rejected, however, in an accompanying prespecified sensitivity test, and further supplementary analyses. Prespecified secondary analyses showed no significant differences between CoQ10 at 2,700 mg/day and placebo. There were no safety concerns. INTERPRETATION: CoQ10 at 2,700 mg daily for 9 months shows insufficient promise to warrant Phase III testing. Given this outcome, the adaptive Phase II design incorporating a dose selection and a futility test avoided the need for a much larger conventional Phase III trial.

Residency Training: A practical guide for medical students who are planning a future in neurology.

Medical students choose neurology for many reasons, including interest in neuroscience, the intellectual challenge of diagnosing and treating disorders of the nervous system, and the opportunity for continuity of care. Neurologists have great flexibility in choice of practice environment, ranging from an exclusively inpatient setting to a strictly clinic-based practice. The purpose of this article is to provide practical, actionable, systematic advice for medical students at every level of training on how to prepare for a neurology residency application and a career in neurology. To this end, we include a timeline designed to guide students' efforts throughout their medical school education. These recommendations incorporate the viewpoints of key participants in many aspects of medical student and resident neuroscience education. The timeline and recommendations outline a suggested early, proactive approach, but should not discourage students from considering neurology at a later point in their training. Neurology remains an inclusive specialty that welcomes students of all backgrounds and offers a rewarding career in one of the most exciting fields in medicine.

Predicting Performance of First-Year Residents: Correlations Between Structured Interview, Licensure Exam, and Competency Scores in a Multi-Institutional Study.

PURPOSE: To determine whether scores on structured interview (SI) questions designed to measure noncognitive competencies in physicians (1) predict subsequent first-year resident performance on Accreditation Council for Graduate Medical Education (ACGME) milestones and (2) add incremental validity over United States Medical Licensing Examination (USMLE) Step 1 and Step 2 Clinical Knowledge scores in predicting performance. METHOD: The authors developed 18 behavioral description questions to measure key noncognitive competencies (e.g., teamwork). In 2013-2015, 14 programs (13 residency, 1 fellowship) from 6 institutions used subsets of these questions in their selection processes. The authors conducted analyses to determine the validity of SI and USMLE scores in predicting first-year resident milestone performance in the ACGME's core competency domains and overall. RESULTS: SI scores predicted midyear and year-end overall performance (r = 0.18 and 0.19, respectively, P < .05) and year-end performance on patient care, interpersonal and communication skills, and professionalism competencies (r = 0.23, r = 0.22, and r = 0.20, respectively, P < .05). SI scores contributed incremental validity over USMLE scores in predicting year-end performance on patient care (ΔR = 0.05), interpersonal and communication skills (ΔR = 0.09), and professionalism (ΔR = 0.09; all P < .05). USMLE scores contributed incremental validity over SI scores in predicting year-end performance overall and on patient care and medical knowledge. CONCLUSIONS: SI scores predict first-year resident year-end performance in the interpersonal and communication skills, patient care, and professionalism competency domains. Future research should investigate whether SIs predict a range of clinically relevant outcomes.

Entrustable professional activities: A useful concept for neurology education.

Medical education is currently undergoing a paradigm shift from process-based to competency-based education, focused on measuring the desired competence of a physician. In an attempt to improve the assessment framework used for medical education, the concept of entrustable professional activities (EPAs) has gained traction. EPAs are defined as professional activities that can be entrusted to an individual in a clinical context. The Association of American Medical Colleges (AAMC) defined a set of 13 such EPAs to define the core of what all students should be able to do on day 1 of residency, regardless of specialty choice. The AAMC is currently piloting these EPAs with 10 medical schools to determine if EPAs can be used as a way to observe, measure, and entrust medical students with core clinical activities by the end of the clinical immersion experiences of the third year. The specialty of pediatrics is piloting the use of specialty-specific EPAs at 5 medical schools to assess readiness for transitions from medical school into pediatric residency training and practice. To date, no neurology-specific EPAs have been published for use in neurology clerkships or neurology residencies. This article introduces the concept of EPAs in the context of competency-based medical education and describes how EPAs might be relevant and applicable in neurologic education across the continuum. The Undergraduate Education Subcommittee of the American Academy of Neurology advocates for a proactive approach to incorporating core EPAs in undergraduate medical education and to considering an EPA-based specialty-specific assessment framework for neurology.

ALS and other motor neuron diseases.

PURPOSE OF REVIEW: This review describes the most common motor neuron disease, ALS. It discusses the diagnosis and evaluation of ALS and the current understanding of its pathophysiology, including new genetic underpinnings of the disease. This article also covers other motor neuron diseases, reviews how to distinguish them from ALS, and discusses their pathophysiology. RECENT FINDINGS: In this article, the spectrum of cognitive involvement in ALS, new concepts about protein synthesis pathology in the etiology of ALS, and new genetic associations will be covered. This concept has changed over the past 3 to 4 years with the discovery of new genes and genetic processes that may trigger the disease. As of 2014, two-thirds of familial ALS and 10% of sporadic ALS can be explained by genetics. TAR DNA binding protein 43 kDa (TDP-43), for instance, has been shown to cause frontotemporal dementia as well as some cases of familial ALS, and is associated with frontotemporal dysfunction in ALS. SUMMARY: The anterior horn cells control all voluntary movement: motor activity, respiratory, speech, and swallowing functions are dependent upon signals from the anterior horn cells. Diseases that damage the anterior horn cells, therefore, have a profound impact. Symptoms of anterior horn cell loss (weakness, falling, choking) lead patients to seek medical attention. Neurologists are the most likely practitioners to recognize and diagnose damage or loss of anterior horn cells. ALS, the prototypical motor neuron disease, demonstrates the impact of this class of disorders. ALS and other motor neuron diseases can represent diagnostic challenges. Neurologists are often called upon to serve as a "medical home" for these patients: coordinating care, arranging for durable medical equipment, and leading discussions about end-of-life care with patients and caregivers. It is important for neurologists to be able to identify motor neuron diseases and to evaluate and treat patients affected by them.