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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.

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.

Amyotrophic lateral sclerosis (ALS) is a progressive, incurable, and fatal neurodegenerative disease associated with average survival of 2 to 5 years. ALS is characterized by the progressive loss of motor neurons resulting in extremity weakness, dysarthria, dysphagia, and respiratory failure. Despite the serious nature of ALS, the mental health of patients with ALS can be relatively preserved; however, unrecognized and untreated mental illness can have a significant impact on quality of life and outcomes. Cognitive and behavioral changes can be prevalent and affect the management of patients. There are also important mental health implications for the caregiver. This article reviews the current understanding of psychiatric disease in patients with ALS. [ Psychiatr Ann . 2017;47(5):258–262. ]

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.

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, thespectrum of cognitiveinvolvementinALS, new concepts aboutprotein synthesis pathologyinthe etiologyofALS, and new geneticassociations 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. Continuum (Minneap Minn) 2014;20(5):1185–1207.

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.

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.

This stroke code simulation was developed as an educational tool for neurology junior residents, emergency department residents, medical students, nurses, and any medical learners involved in stroke management. The goal is to offer hands-on practice in running a stroke code effectively and efficiently as leaders of a team, ensuring appropriate critical medical decision-making and patient care. Duration of the simulation is 20 minutes, with a minimum of 20 minutes dedicated to a debriefing. Standardized patients are utilized for role playing to enable presentation of neurologic deficits and use of the National Institute of Health Stroke Scale. Residents ideally participate in these simulations in groups of two or three, giving one person the opportunity to be the team leader running the stroke code and others to take the roles of team members. In addition, emergency medicine residents and intensive care unit nurses are included to act as confederates in the simulations and participate in the debrief. Debriefing sessions are held immediately after each simulation with intent of formative feedback using the “good judgment” and “advocacy inquiry” method. Pre- and postsimulation learner surveys help assess effectiveness and provide critical feedback for future considerations. Out of 13 participating neurology residents during our implementation, all agreed that these simulation sessions met the stated clinical objectives. All residents also agreed that the overall quality and utility of debriefing sessions were good. Approximately 84% agreed that the simulation experience was more useful than reading a chapter or attending a lecture about the clinical scenario. After completing the sessions, all surveyed residents agreed that they will be able to better facilitate future acute stroke care as compared to before the training.

Impaired graft function in the postoperative course after lung transplantation (LTx) may in part be due to alterations in pulmonary surfactant. Animal data provide increasing evidence for surfactant abnormalities in the early course after graft reperfusion. However, little is known about the integrity of the surfactant system in human lung transplant recipients. We therefore investigated surfactant properties in bronchoalveolar lavage fluid (BALF) of patients with lung transplants (n = 60) in comparison to that of healthy subjects (n = 10). The phospholipid concentrations of BALF and of surfactant subfractions were measured, and total protein was analyzed. Surface activity was measured with a pulsating bubble surfactometer (PBS). Minimum surface tension was 15.8 +/- 1.1 mN/m in lung transplant recipients, whereas healthy subjects had minimum surface tensions of 3.4 +/- 1.9 mN/m (p = 0.0004). As a marker for potential surfactant inhibition, protein-to-phospholipid (PL) ratios showed no significant differences in the two major study groups. The ratio of small surfactant aggregates to large surfactant aggregates was increased in patients with lung transplants (p = 0.043). Episodes of infection or rejection did not change surface activities, nor did they induce altered ratios of protein to PL or of small to large surfactant aggregates. Surfactant activity did not correlate with pulmonary-function data. Moreover, surface tension showed no correlation with the time after transplantation. Our results suggest a persistent impairment of biophysical surfactant properties after LTx, possibly due to type-II-cell malfunction.

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.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.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.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.

We congratulate Sorenson et al.1 for their well-executed IGF-1 trial in patients with amyotrophic lateral sclerosis (ALS). While the title appropriately reflects the subcutaneous mode of IGF-1 delivery, the article fails to distinguish this critical limitation. What is the bioavailability in CSF and brain parenchyma in humans treated twice daily with 0.05 mg/kg IGF-1? Animal studies indicate that subcutaneous delivery of ∼0.8 mg/kg IGF-1 results in a maximal CSF concentration of 3 ng/mL IGF-1 at 2 hours postinjection.2 This equates to 0.4 nM IGF-1 in CSF at peak. The Sorenson et al. trial dose was 16-fold lower, suggesting that the maximal CSF concentration in patients would be 0.025 nM. The Kd of the IGF-1 receptor is 0.15 nM.3 Therefore, at 0.025 nM, only 14% of high-affinity and less than 1% of low-affinity receptors will be occupied. Likewise, the concentration of IGF-1 in CSF overestimates the bioavailable concentration within the brain parenchyma. IGF-1 injected into the lateral ventricle of rats exhibits a half-life of only 12 minutes in the CSF and penetrates …