Neuroprognostication for Patients with Amyotrophic Lateral Sclerosis: An Updated, Evidence-Based Review.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder that presents and progresses in various ways, making prognostication difficult. Several paradigms exist for providers to elucidate prognosis in a way that addresses not only the amount of time a patient has to live, but also a patient's quality of their life moving forward. Prognostication, with regard to both survivability and quality of life, is impacted by several features that include, but are not limited to, patient demographics, clinical features on presentation, and over time, access to therapy, and access to multidisciplinary clinics.

Development and Dissemination of a Neurology Palliative Care Curriculum: Education in Palliative and End-of-Life Care Neurology.

Despite increasing awareness of the importance of a palliative care approach to meet the needs of persons living with neurologic illness, residency and fellowship programs report meeting this educational need due to a limited pool of neuropalliative care educators and a lack of adequate educational resources. To meet this need, a group of experts in neuropalliative care and palliative medicine leveraged resources from the Education in Palliative and End-of-life Care (EPEC) program and the National Institutes of Nursing Research to create a library of modules addressing topics relevant for neurology trainees, palliative medicine fellows, and clinicians in practice. In this article, we describe the development and dissemination plan of the EPEC Neurology program, initial evidence of efficacy, and opportunities for neurology educators and health services researchers to use these resources.

Compassionate Removal of Heated High-Flow Nasal Cannula for End of Life: Case Series and Protocol Development.

Patients often receive burdensome care at the end of life in the form of interventions that may need to be removed. Heated high-flow oxygen delivered through a nasal cannula (HHFNC) is one such intervention that can be delivered in the hospital yet is rarely available outside of this setting. During the COVID-19 (coronavirus disease 2019) pandemic, health care systems continue to face the possibility of rationing critical life-sustaining equipment that may include HHFNC.

Models of outpatient neuropalliative care for patients with amyotrophic lateral sclerosis.

Objective: To describe some current models of outpatient neuropalliative care (NPC) available to patients with amyotrophic lateral sclerosis at different institutions within the United States.

Methods: Six NPC physicians were asked to contribute written descriptions about the PC available in their ALS clinics. Descriptions were then compiled and assessed for similarities and differences.

Motor Neuron Disease: Pathophysiology, Diagnosis, and Management.

Patients with motor neuron diseases may present to primary care clinic or may be initially encountered in the inpatient setting. Timely diagnosis of these conditions is a key factor in early intervention and therapy, and accuracy of diagnosis is of extreme importance, in particular for amyotrophic lateral sclerosis with its poor prognosis. The aim of this review article is to provide a clinical and diagnostic framework for the diagnosis and evaluation of motor neuron disease for primary care physicians.

Burnout, career satisfaction, and well-being among US neurology residents and fellows in 2016.

Objective: To study prevalence of and factors contributing to burnout, career satisfaction, and well-being in US neurology residents and fellows.

Methods: A total of 938 US American Academy of Neurology member neurology residents and fellows were surveyed using standardized measures of burnout, career satisfaction, and well-being from January 19 to March 21, 2016.

Results: Response rate was 37.

Infantile Epileptic Encephalopathy Associated With SCN2A Mutation Responsive to Oral Mexiletine.

Background: Genetic alterations are significant causes of epilepsy syndromes; especially early-onset epileptic encephalopathies and voltage-gated sodium channelopathies are among the best described. Mutations in the SCN2A subunit of voltage-gated sodium channels have been associated with benign familial neonatal-infantile seizures, generalized epilepsy febrile seizures plus, and an early-onset infantile epileptic encephalopathy.

Method: We describe two infants with medically refractory seizures due to a de novo SCN2A mutation.

Ischemic preconditioning of the lower extremity attenuates the normal hypoxic increase in pulmonary artery systolic pressure.

Ischemic pre-condition of an extremity (IPC) induces effects on local and remote tissues that are protective against ischemic injury. To test the effects of IPC on the normal hypoxic increase in pulmonary pressures and exercise performance, 8 amateur cyclists were evaluated under normoxia and hypoxia (13% F(I)O(2)) in a randomized cross-over trial. IPC was induced using an arterial occlusive cuff to one thigh for 5 min followed by deflation for 5 min for 4 cycles.