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. An understanding of the impact that these features have on the life of a patient with ALS can help providers to develop a better and more personalized approach for patients related to their clinical prognosis after a diagnosis is made. The ultimate goal of prognostication is to empower patients with ALS to take control and make decisions with their care teams to ensure that their goals are addressed and met.

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. We present a clinical protocol designed for weaning HHFNC to allow a natural death and ensuring adequate symptom management throughout the process. This was a retrospective chart review of 8 patients seen by an inpatient palliative care service of an academic tertiary referral hospital who underwent terminal weaning of HHFNC using a structured protocol to manage dyspnea. Eight patients with diverse medical diagnoses, including COVID-19 pneumonia, underwent terminal weaning of HHFNC according to the clinical protocol with 4 down-titrations of approximately 25% for both fraction of inspired oxygen and liter flow with preemptive boluses of opioid and benzodiazepine. Clinical documentation supported good symptom control throughout the weaning process. This case series provides preliminary evidence that the clinical protocol proposed has the ability to ensure comfort through terminal weaning of 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. RESULTS: All clinics are multidisciplinary, with regular appointments every 3 months and similar appointment times for new visits (60-120 minutes) and follow-up visits (20-45 minutes). Four clinics have an NPC specialist embedded within the ALS clinic, 1 institution has a separate clinic for NPC, and 1 institution has both. The NPC physician at 5 institutions is a neurologist with formal palliative care training and at 1 institution is an internist with formal palliative care training. NPC is part of routine care for all patients at 2 institutions, and the primary reasons for consultation are goals of care (GOC) and severe symptom management. CONCLUSION: NPC is provided to patients with ALS heterogeneously throughout the United States with some variation in services and delivery, but all clinics are addressing similar core needs. Given the poor prognosis and high PC needs, those with ALS are the ideal patients to receive NPC. Future studies are necessary given the paucity of data available to determine best practices and to better define meaningful outcomes.

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 37.7% (354/938); about 2/3 of responders were residents and 1/3 were fellows. Median age of participants was 32 years and 51.1% were female. Seventy-three percent of residents and 55% of fellows had at least one symptom of burnout, the difference largely related to higher scores for depersonalization among residents. For residents, greater satisfaction with work-life balance, meaning in work, and older age were associated with lower risk of burnout; for fellows, greater satisfaction with work-life balance and effective support staff were associated with lower risk of burnout. Trainees experiencing burnout were less likely to report career satisfaction. Career satisfaction was more likely among those reporting meaning in work and more likely for those working in the Midwest compared with the Northeast region. CONCLUSIONS: Burnout is common in neurology residents and fellows. Lack of work-life balance and lack of meaning in work were associated with reduced career satisfaction and increased risk of burnout. These results should inform approaches to reduce burnout and promote career satisfaction and well-being in US neurology trainees.

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. RESULTS: The first child responded to intravenous lidocaine with significant reduction in seizure frequency and was successfully transitioned to enteral mexiletine. Mexiletine was subsequently used in a second infant with reduction in seizure frequency. CONCLUSION: Class 1b antiarrhythmic agents, lidocaine and mexiletine, may be useful in infants with medically refractory early infantile epileptic encephalopathy secondary to mutations in SCN2A.

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. In the control condition, the resting pulmonary artery systolic pressure (PASP) increased from a normoxic value of 25.6±2.3 mmHg to 41.8±7.2 mmHg following 90 min of hypoxia. In the IPC condition, the PASP increased to only 32.4±3.1 mmHg following hypoxia, representing a 72.8% attenuation (p=0.003). No significant difference was detected in cycle ergometer time trial duration between control and IPC conditions with either normoxia or hypoxia. IPC administered prior to hypoxic exposure was associated with profound attenuation of the normal hypoxic increase of pulmonary artery systolic pressure.