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Building the Neurology Pipeline With Undergraduate Students in Research and Clinical Practice

Minen, Mia T; Szperka, Christina L; Cartwright, Michael S; Wells, Rebecca Erwin
There is currently a shortage of neurologists in the United States. Multiple efforts are underway to increase the strength of the neurology workforce. One potential approach is early exposure to neurology research and clinical care to pique interest and promote the specialty as a career choice. This study details the rewarding experience of working with undergraduate students, both in clinical research and clinical care. The logistics, benefits to students, and positive aspects for neurologists are outlined. Examples provided by undergraduate students who have participated in neurology research and clinical care are presented. The ultimate goals of this work are to encourage and inspire academic neurologists to involve undergraduate students in research and clinical care, to facilitate this process by outlining the steps needed to make this pairing successful, and to ultimately promote a pathway to build the neurology pipeline.
PMID: 33293387
ISSN: 1526-632x
CID: 4828582

Editors' Note: Acute Hypokinetic-Rigid Syndrome After SARS-CoV-2 Infection [Comment]

Siegler, James E; Galetta, Steven
PMID: 33649084
ISSN: 1526-632x
CID: 5092832

Practical Approach to the Tele-Neuro-Ophthalmology and Neuro-Otology Visits: Instructional Videos

Calix, Rachel; Grossman, Scott N; Rasool, Nailyn; Small, Leslie; Cho, Catherine; Galetta, Steven L; Balcer, Laura J; Rucker, Janet C
ABSTRACT/UNASSIGNED:A collection of instructional videos that illustrate a step by step approach to tele-neuro-ophthalmology and neuro-otology visits. These videos provide instruction for patient preparation for their video visit, patient and provider interface with an electronic medical record associated video platform, digital applications to assist with vision testing, and practical advice for detailed remote neuro-ophthalmologic and neuro-otologic examinations.
PMID: 33587534
ISSN: 1536-5166
CID: 4786512

Critical Care Medicine Practice: A Pilot Survey of US Anesthesia Critical Care Medicine-Trained Physicians

Siddiqui, Shahla; Bartels, Karsten; Schaefer, Maximilian S; Novack, Lena; Sreedharan, Roshni; Ben-Jacob, Talia K; Khanna, Ashish K; Nunnally, Mark E; Souter, Michael; Simmons, Shawn T; Williams, George
BACKGROUND:This survey assessed satisfaction with the practice environment among physicians who have completed fellowship training in critical care medicine (CCM) as recognized by the American Board of Anesthesiology (and are members of the American Society of Anesthesiology) and evaluated the perceived effectiveness of training programs in preparing fellows for critical care practice. METHODS:A cross-sectional online survey composed of 39 multiple choice and open-ended questions was administered between August and December 2018 to all members of the American Society of Anesthesiologists (ASA) who self-identified as being CCM trained. The survey instrument was developed and revised in an iterative fashion by ASA committee on CCM and the Society for Education in Anesthesia (SEA). Survey results were analyzed using a mixed-method approach. RESULTS:Three hundred fifty-three of the 1400 anesthesiologists who self-identified to the ASA as having CCM training (25.2%) completed the survey. Most were men (72.3%), board certified in CCM (98.7%), and had practiced a median of 5 years. Half of the respondents rated their training as "excellent." A total of 70.6% described currently working in academic centers with 53.6% providing care in open surgical intensive care units (ICUs). Most anesthesiologist intensivists (75%) spend at least 25% of their clinical time providing ICU care (versus clinical anesthesia). A total of 89% of the respondents were involved in educational activities, 60% reported being in administrative leadership roles, and 37% engaged in scholarly activity. Areas of dissatisfaction included fatigue, lack of collegiality or respect, lack of research training, decreased job satisfaction, and burnout. Analysis suggested moderate levels of job satisfaction (49%), work-life balance (52%), and high levels of burnout (74%). A significant contributor to burnout was with a perception of lack of respect (P = .005) in the work environment. Burnout was not significantly associated with gender or duration of practice. Qualitative analysis of the open-ended responses also identified these 3 variables as major themes. CONCLUSIONS:This survey of CCM-trained anesthesiologists described a high rate of board certification, practice in academic settings, and participation in resident education. Areas of dissatisfaction with an anesthesia/critical care practice included burnout, work/life balance, and lack of respect. These results may increase recruitment of anesthesiologists into critical care and inform strategies to improve satisfaction with anesthesia critical care practice, fellowship training.
PMID: 32665465
ISSN: 1526-7598
CID: 4528192

NF106: A Neurofibromatosis Clinical Trials Consortium Phase II Trial of the MEK Inhibitor Mirdametinib (PD-0325901) in Adolescents and Adults With NF1-Related Plexiform Neurofibromas

Weiss, Brian D; Wolters, Pamela L; Plotkin, Scott R; Widemann, Brigitte C; Tonsgard, James H; Blakeley, Jaishri; Allen, Jeffrey C; Schorry, Elizabeth; Korf, Bruce; Robison, Nathan J; Goldman, Stewart; Vinks, Alexander A; Emoto, Chie; Fukuda, Tsuyoshi; Robinson, Coretta T; Cutter, Gary; Edwards, Lloyd; Dombi, Eva; Ratner, Nancy; Packer, Roger; Fisher, Michael J
PURPOSE/OBJECTIVE:Patients with neurofibromatosis type 1 (NF1) frequently develop plexiform neurofibromas (PNs), which can cause significant morbidity. We performed a phase II trial of the MAPK/ERK kinase inhibitor, mirdametinib (PD-0325901), in patients with NF1 and inoperable PNs. The primary objective was response rate based on volumetric magnetic resonance imaging analysis. METHODS:/dose (maximum dose = 4 mg twice a day) in a 3-week on/1-week off sequence. Each course was 4 weeks in duration. Evaluations were performed after four courses for the first year and then after every six courses. Patients could receive a maximum of 24 total courses. RESULTS:Nineteen patients were enrolled, and all 19 received mirdametinib. The median age was 24 years (range, 16-39 years); the median baseline tumor volume was 363.8 mL (range, 3.9-5,161 mL). Eight of the 19 patients (42%) achieved a partial response of the target PN by course 12, and 10 (53%) had stable disease. One patient (5%) developed progressive disease at course 8. Significant and durable decreases were observed in pain ratings. CONCLUSION/CONCLUSIONS:/dose (maximum dose, 4 mg) twice daily in a 3-week on/1-week off sequence resulted in a 42% partial response rate with preliminary evidence of reduction in pain.
PMID: 33507822
ISSN: 1527-7755
CID: 4819512

Long-standing Multifocal Motor Neuropathy Presenting With Delayed Clinical Features of Anti-Myelin-Associated Glycoprotein Neuropathy and Elevated Anti-Myelin-Associated Glycoprotein Antibody Titers

Bernardo, Katrina A; Weiss, Michael D
ABSTRACT/UNASSIGNED:Multifocal motor neuropathy with conduction block (MMN) and anti-myelin-associated glycoprotein (MAG) neuropathy are rare chronic acquired demyelinating neuropathies with distinct clinical and electrophysiological characteristics. These neuropathies are generally not known to coexist. This report describes a patient with long-standing MMN who subsequently developed clinical features of anti-MAG neuropathy. This suggests that subtypes of chronic inflammatory neuropathies may not be sharply defined. In addition, a presentation of MMN with anti-MAG titers may be a prognostic indicator of poor response to standard MMN treatment.
PMID: 33596002
ISSN: 1537-1611
CID: 4795422

Laterality and region-specific tau phosphorylation correlate with PTSD-related behavioral traits in rats exposed to repetitive low-level blast

Perez Garcia, Georgina; De Gasperi, Rita; Gama Sosa, Miguel A; Perez, Gissel M; Otero-Pagan, Alena; Pryor, Dylan; Abutarboush, Rania; Kawoos, Usmah; Hof, Patrick R; Dickstein, Dara L; Cook, David G; Gandy, Sam; Ahlers, Stephen T; Elder, Gregory A
Military veterans who experience blast-related traumatic brain injuries often suffer from chronic cognitive and neurobehavioral syndromes. Reports of abnormal tau processing following blast injury have raised concerns that some cases may have a neurodegenerative basis. Rats exposed to repetitive low-level blast exhibit chronic neurobehavioral traits and accumulate tau phosphorylated at threonine 181 (Thr181). Using data previously reported in separate studies we tested the hypothesis that region-specific patterns of Thr181 phosphorylation correlate with behavioral measures also previously determined and reported in the same animals. Elevated p-tau Thr181 in anterior neocortical regions and right hippocampus correlated with anxiety as well as fear learning and novel object localization. There were no correlations with levels in amygdala or posterior neocortical regions. Particularly striking were asymmetrical effects on the right and left hippocampus. No systematic variation in head orientation toward the blast wave seems to explain the laterality. Levels did not correlate with behavioral measures of hyperarousal. Results were specific to Thr181 in that no correlations were observed for three other phospho-acceptor sites (threonine 231, serine 396, and serine 404). No consistent correlations were linked with total tau. These correlations are significant in suggesting that p-tau accumulation in anterior neocortical regions and the hippocampus may lead to disinhibited amygdala function without p-tau elevation in the amygdala itself. They also suggest an association linking blast injury with tauopathy, which has implications for understanding the relationship of chronic blast-related neurobehavioral syndromes in humans to neurodegenerative diseases.
PMCID:7923605
PMID: 33648608
ISSN: 2051-5960
CID: 4801262

The transformation of patient-clinician relationships with AI-based medical advice

Nov, Oded; Aphinyanaphongs, Yindalon; Lui, Yvonne W.; Mann, Devin; Porfiri, Maurizio; Riedl, Mark; Rizzo, John Ross; Wiesenfeld, Batia
The transformation of patient-clinician relationships with AI-based medical advice is discussed. many new tools are based on entirely new "˜black-box"™ AI-based technologies, whose inner workings are likely not fully understood by patients or clinicians. Most patients with Type 1 diabetes now use continuous glucose monitors and insulin pumps to tightly manage their disease. Their clinicians carefully review the data streams from both devices to recommend dosage adjustments. Recently new automated recommender systems to monitor and analyze food intake, insulin doses, physical activity, and other factors influencing glucose levels, and provide data-intensive, AI-based recommendations on how to titrate the regimen, are in different stages of FDA approval using "˜black box"™ technology, which is an alluring proposition for a clinical scenario that requires identification of meaningful patterns in complex and voluminous data.
SCOPUS:85101579091
ISSN: 0001-0782
CID: 4832842

Resident and Fellow Training in a Pandemic

Grossman, Scott N; Galetta, Steven L; Lee, Andrew G; Biousse, Valerie; Ishida, Koto
PMID: 33587533
ISSN: 1536-5166
CID: 4786502

Capturing seizures in clinical trials of antiseizure medications for KCNQ2-DEE

Millichap, John J; Harden, Cynthia L; Dlugos, Dennis J; French, Jacqueline A; Butterfield, Noam N; Grayson, Celene; Aycardi, Ernesto; Pimstone, Simon N
Literature review of patients with KCNQ2 developmental and epileptic encephalopathy (KCNQ2-DEE) reveals, based on 16 reports including 139 patients, a clinical phenotype that includes age- and disease-specific stereotyped seizures. The typical seizure type of KCNQ2-DEE, focal tonic, starts within 0-5 days of life and is readily captured by video-electroencephalography VEEG for clinical and genetic diagnosis. After initial identification, KCNQ2-DEE seizures are clinically apparent and can be clearly identified without the use of EEG or VEEG. Therefore, we propose that the 2019 recommendations from the International League against Epilepsy (ILAE), the Pediatric Epilepsy Research Consortium (PERC), for capturing and recording seizures for clinical trials (Epilepsia Open, 4, 2019, 537) are suitable for use in KCNQ2-DEE‒associated antiseizure medicine (ASM) treatment trials. The ILAE/PERC consensus guidance states that a caregiver-maintained seizure diary, completed by caregivers who are trained to recognize seizures using within-patient historical recordings, accurately captures seizures prospectively in a clinical trial. An alternative approach historically endorsed by the Food and Drug Administration (FDA) compares seizure counts captured on VEEG before and after treatment. A major advantage of the ILAE/PERC strategy is that it expands the numbers of eligible patients who meet inclusion criteria of clinical trials while maintaining accurate seizure counts (Epilepsia Open, 4, 2019, 537). Three recent phase 3 pivotal pediatric trials investigating ASMs to treat syndromic seizures in patients as young as 2 years of age (N Engl J Med, 17, 2017, 699; Lancet, 21, 2020, 2243; Lancet, 17, 2018, 1085); and ongoing phase 2 open-label pediatric clinical trial that includes pediatric epileptic syndromes as young as 1 month of age (Am J Med Genet A, 176, 2018, 773), have already used caregiver-maintained seizure diaries successfully. For determining the outcome of a KCNQ2-DEE ASM treatment trial, the use of a seizure diary to count seizures by trained observers is feasible because the seizures of KCNQ2-DEE are clinically apparent. This strategy is supported by successful precedent in clinical trials in similar age groups and has the endorsement of the international pediatric epilepsy community.
PMCID:7918316
PMID: 33681646
ISSN: 2470-9239
CID: 4807632