Neurology Commons^™

Guillain-Barre Syndrome Following A Snakebite: A Case Report And Review Of Literature, Sajid Hameed, Mubashar Memon, Sara Khan

Section of Neurology

Guillain-Barre syndrome (GBS) is preceded by a respiratory or gastrointestinal illness in more than 50% of the patients. A rare association of GBS with a preceding snakebite is reported in the literature in only four previous cases. We present a case report of a patient diagnosed with GBS following the bite of a yellow-bellied sea snake (Hydrophis platurus) and a brief review of the available literature.

Yield Of Muscle Biopsy In Patients With Findings Of Myopathy On Electrodiagnostic Testing, Sarah H. Siddiqui, Raheel Ahmed, Safia Awan, Ambreen Zain, Sara Khan

Section of Neurology

Background: The evaluation of neuromuscular diseases includes detailed clinical assessment, blood testing, electrodiagnostic studies (EDS), biopsy, and genetic tests. EDS alone cannot provide a specific diagnosis. Further testing in the form of genetic tests or muscle biopsy (MB) is required. Objective The objective of the study is to evaluate the yield of MB in patients with findings of myopathy on electrodiagnostic testing and assess the factors affecting an abnormal biopsy outcome.

Methods: Electromyography (EMG)/nerve conduction studies (NCS) performed for suspected myopathy over 5 years from 2011 to 2016, at the neurophysiology department of a tertiary care center in Pakistan, were …

Biochemical And Clinical Response After Umbilical Cord Blood Transplant In A Boy With Early Childhood-Onset Beta-Mannosidosis., Troy C. Lund, Weston P. Miller, Julie B. Eisengart, Katrina Simmons, Laura Pollard, Deborah L. Renaud, David A. Wenger, Marc C. Patterson, Paul J. Orchard

Department of Neurology Faculty Papers

BACKGROUND: Deficiency in the enzyme β-mannosidase was described over three decades ago. Although rare in occurrence, the presentation of childhood-onset β-mannosidase deficiency consists of hypotonia in the newborn period followed by global development delay, behavior problems, and intellectual disability. No effective pharmacologic treatments have been available.

METHODS: We report 2-year outcomes following the first umbilical cord blood transplant in a 4-year-old boy with early childhood-onset disease.

RESULTS: We show restoration of leukocyte β-mannosidase activity which remained normal at 2 years posttransplant, and a simultaneous increase in plasma β-mannosidase activity and dramatic decrease in urine-free oligosaccharides were also observed. MRI of …

Galcanezumab In Episodic Migraine: Subgroup Analyses Of Efficacy By High Versus Low Frequency Of Migraine Headaches In Phase 3 Studies (Evolve-1 & Evolve-2)., Stephen D. Silberstein, Virginia L. Stauffer, Katie A. Day, Sarah Lipsius, Maria-Carmen Wilson

Department of Neurology Faculty Papers

BACKGROUND: Patients with high-frequency episodic migraine (HFEM) have a greater disease burden than those with low-frequency episodic migraine (LFEM). Acute treatment overuse increases the risk of migraine chronification in patients with HFEM. Galcanezumab, a humanized monoclonal antibody binding calcitonin gene-related peptide (CGRP), is effective for migraine prevention with a favorable safety profile. Here, we investigate whether there are differences in galcanezumab efficacy in patients with LFEM or with HFEM.

METHODS: Data were pooled from two double-blind, placebo-controlled phase 3 trials; EVOLVE-1 and EVOLVE-2. Patients were 18-65 years old, experienced 4-14 monthly migraine headache days (MHDs) for ≥1 year prior, with …

Astragaloside Iv Protects Neurons From Microglia-Mediated Cell Damage Through Promoting Microglia Polarization., Jingwen Yu, Minfang Guo, Yanhua Li, Huiyu Zhang, Zhi Chai, Qing Wang, Yuqing Yan, Jiezhong Yu, Chunyun Liu, Guang-Xian Zhang, Ma Cungen

Department of Neurology Faculty Papers

Astragaloside IV (AST-IV) is a major active ingredient of astragalus, with a neuroprotective effect. The current study is aimed to investigate the impact of AST-IV on the M1/M2 microglial activation in response to lipopolysaccharide (LPS) stimulation, how AST-IV attenuated microglia-mediated neuronal damage, and the molecular mechanisms underlying AST-IV's protection of neurons against microglia-mediated neuronal damage. Our results showed that AST-IV partially protected microglia from death evoked by LPS and downregulated the release of pro-inflammatory (M1) mediators including interleukin (IL)-1β, IL-6, tumour necrosis factor α (TNF-α) and nitric oxide, as well as the expression of Toll-like receptors 4 (TLR4), MyD88, and …

A 24-Week Double-Blind Placebo-Controlled Study Of The Efficacy And Safety Of The Ampa Modulator S47445 In Patients With Mild To Moderate Alzheimer's Disease And Depressive Symptoms, Eileen M. Bulger, Sylvie Gouttefangeas, Sylvie Bretin, Stéphanie Galtier, Philippe Robert, Vjera Holthoff-Detto, Jeffrey Cummings, Maria Pueyo

School of Medicine Faculty Research

Introduction S47445 is a novel positive allosteric modulator of alpha-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid receptors that may emerge as a favorable candidate for the symptomatic treatment of cognitive and depressive disorders in patients suffering from Alzheimer's disease (AD) of mild to moderate severity and with depressive symptoms. Methods For this double-blind, placebo-controlled 24-week phase II trial, 520 outpatients aged between 55 and 85 years, with probable AD at mild to moderate stages (a Mini-Mental State Examination score of 24-15 inclusive) and exhibiting depressive symptoms (Cornell Scale for Depression in Dementia [CSDD] ≥ 8) were recruited in twelve countries and randomized to 3 doses …

Stat3 Genotypic Variant Rs744166 And Increased Tyrosine Phosphorylation Of Stat3 In Il-23 Responsive Innate Lymphoid Cells During Pathogenesis Of Crohn's Disease, Ying Tang, Sanda A. Tan, Atif Iqbal, Jian Li, Sarah C. Glover

Department of Neurology Faculty Papers

Crohn's disease (CD) results from dysregulated immune responses to gut microbiota in genetically susceptible individuals, affecting multiple areas of the gastrointestinal tract. Innate lymphoid cells (ILCs) are tissue-resident innate effector lymphocytes which play crucial roles in mucosal immune defense, tissue repair, and maintenance of homeostasis. The accumulation of IFN-γ-producing ILC1s and increased level of proinflammatory cytokines produced by ILCs has been observed in the inflamed terminal ileum of CD patients. To date, the precise mechanisms of ILC plasticity and gene regulatory pathways in ILCs remain unclear. Signal transducer and activator of transcription 3 (STAT3) regulates gene expression in …

Testosterone Levels In Men With Chronic Migraine, Lisa B. E. Shields, Tad Seifert, Brent J. Shelton, Brian M. Plato

Markey Cancer Center Faculty Publications

Chronic migraine is a frequent and debilitating condition affecting 14% of the general population. This prospective observational pilot study investigated whether men with chronic migraine have lower than expected total serum testosterone levels. We identified 14 men ages 26-51 at our Institution who fulfilled the ICHD-3b criteria for chronic migraine and obtained serum total testosterone levels. The mean total testosterone level in our 14 patients was 322 ng/dL (range: 120-542 ng/dL) which is in the lower 5% of the reference range for our laboratory (300-1080 ng/dL). Men with chronic migraine had lower total testosterone levels compared to published agematched normative …

Immunomodulatory And Neuroprotective Mechanisms Of Huangqi Glycoprotein Treatment In Experimental Autoimmune Encephalomyelitis., Yanxia Xing, Binyu Liu, Yijin Zhao, Lihong Zhang, Rodolfo Thome, Huiqing Xue, Peijun Zhang, Cungen Ma

Department of Neurology Faculty Papers

Previous studies have shown that Huangqi glycoprotein (HQGP) has an anti-inflammatory effect in vitro, and suppressed experimental autoimmune encephalomyelitis (EAE), an animal model of multiple sclerosis; however, the mechanism underlying its effect is largely unknown. In this manuscript we investigated the mechanisms by which HQGP protect mice from EAE. HQGP was extracted from Astragalus membranaceus and purified by anion-exchange and gel filtration chromatography. HQGP delayed disease onset, reduced disease severity and alleviated inflammation and demyelination in the central nervous system (CNS). Moreover, HQGP reduced the infiltration of pathogenic immune cells and increased the expression of microtubule-associated protein 2 (MAP-2) and …

An Integration-To-Bound Model Of Decision-Making That Accounts For The Spectral Properties Of Neural Data, Ramón Guevara Erra, Marco Arbotto, Aaron Schurger

Psychology Faculty Articles and Research

Integration-to-bound models are among the most widely used models of perceptual decision-making due to their simplicity and power in accounting for behavioral and neurophysiological data. They involve temporal integration over an input signal (“evidence”) plus Gaussian white noise. However, brain data shows that noise in the brain is long-term correlated, with a spectral density of the form 1/fα (with typically 1 < α < 2), also known as pink noise or ‘1/f’ noise. Surprisingly, the adequacy of the spectral properties of drift-diffusion models to electrophysiological data has received little attention in the literature. Here we propose a model of accumulation of evidence for decision-making that takes into consideration the spectral properties of brain signals. We develop a generalization of the leaky stochastic accumulator model using a Langevin equation whose non-linear noise term allows for varying levels of autocorrelation in the time course of the decision variable. We derive this equation directly from magnetoencephalographic data recorded while subjects performed a spontaneous movement initiation task. We then propose a nonlinear model of accumulation of evidence that accounts for the ‘1/f’ spectral properties of brain signals, and the observed variability in the power spectral properties of brain signals. Furthermore, our model outperforms the standard drift-diffusion model at approximating the empirical waiting time distribution.

Creation Of An Institutional Toolkit For Evaluation Of Multidisciplinary Handoffs, Richard F. Schmidt, Md, Courtney Devin, Md, Nicholas Tarangelo, Md, Andrew Mendelson, Md, Bracken Babula, Md, Rebecca Jaffe, Md

House Staff Quality Improvement and Patient Safety Conference (2016-2019)

Objectives

1. Create a method for analyzing different handoffs in a variety of clinical settings and scenarios at TJUH.
2. Develop a set of standardized survey tools using existing validated language to quantify the perceptions, quality, and needs for different patient care transitions.
3. Use results from these tools to cater focused handoff improvement interventions for specific patient care settings.

Improving Ed Door To Puncture Times For Endovascular Thrombectomy In Acute Ischemic Stroke, Richard F. Schmidt, Md, Nabeel Herial, Md, Robin D'Ambrosio, Bsn, Erin Simko, Bsn, Fred Rincon, Md, Maria Aini, Md, Pascal Jabbour, Md, Stavropoula Tjoumakaris, Md, M. Reid Gooch, Md, Robert H. Rosenwasswer, Md, Robin Dharia, Md

House Staff Quality Improvement and Patient Safety Conference (2016-2019)

Objectives

• Optimize the management of patients presenting to TJUH with AIS who are candidates for ET.
• Enable continued process improvement through improved data collection methods and identification of new process metrics.

Spinal Cord Injury Activation Alert: Revisiting And Revamping Protocols, Ritam Ghosh, Md, Ralph Marino, Md, Michael Wolf, Md, James S. Harrop, Md

House Staff Quality Improvement and Patient Safety Conference (2016-2019)

Objective

Our objective was to look at the current protocol for spinal cord injury activation alerts and to highlight deficiencies in the system. Currently, any physician at Jefferson can call this alert, which often leads to a misuse of resources and wrongful identification of true spinal cord injury.

In order to refine the protocol, we believe that the Cord System should be used for the following guidelines:

1. Identify patients w Spinal Cord Injury (SCI)
2. Identify patients for SCI research trials
3. Identify patients who require surgery

Lack Of Utility And Excess Cost Of Routine Perioperative Hematologic Testing In Patients Undergoing Elective Neurosurgical Procedures Of The Spine, Lucas Philipp, Md, Mph, Catriona Harrop, Md, David Wyler, Md, James Harrop, Md

House Staff Quality Improvement and Patient Safety Conference (2016-2019)

Objective

To prospectively conduct a "mock trial" among 100% (minimum n=200) of elective neurosurgery spine patients to estimate the total projected cost, savings, risk, and feasibility of a redefined/restricted lab testing protocol over the next 12 months

Developing A Neurosurgical Spine Post-Operative Note Template On Epic For Improved Multi-Disciplinary Care, Omaditya Khanna, Md, Geoffrey P. Stricsek, Md, Giuliana Labella, Rn, James Harrop, Md, Jesse Edwards, Md

House Staff Quality Improvement and Patient Safety Conference (2016-2019)

Introduction

The care of neurosurgical spine patients often involves a multi-disciplinary team, including neurosurgery residents, hospitalists, nursing staff, and physical/occupational therapists.

Oftentimes, post-op spine patients are on a non-neurosurgical service (hospitalist, MICU/SICU, medicine), who are provided with scant sign out on the procedure(s) performed.

The treatment team(s) that are helping manage patient care post-operatively are often unaware of the procedure performed, and what are the salient clinical signs/symptoms, radiographic findings, and laboratory values that need to be closely monitored

Neuroimaging Protocol In Patients Presenting With Spontaneous Intracerebral Hemorrhage, Kevin Hines, Md, Nikolaos Mouchtouris, Md, Karim Hafazalla, Jacob M. Mazza, Phillip Phan, Giuliana Labella, Muhammad Athar, Md, Adam Flanders, Md, Pascal Jabbour, Md

House Staff Quality Improvement and Patient Safety Conference (2016-2019)

Introduction

While lacking evidence based recommendations, most institutions must evaluate spontaneous intracerebral hemorrhages (sICH) for hematoma expansion (HE). If unrecognized, it can be a devastating complication. However, because of the lack of general recommendations for serial imaging, many hospitals, including Thomas Jefferson, have arbitrarily instituted 6 hour an 24 hour stability head CT’s to evaluate for HE.

In our project, we examine the impact of this imaging protocol on the safety and quality of patient care and provide revised recommendations for imaging in sICH at Thomas Jefferson University Hospital and Jefferson Hospital for Neuroscience.

Improving Postoperative Handoffs In The Neuro-Intensive Care Unit, Richard F. Schmidt, Md, Andrew Mendelson, Md, Sonia Gill, Md, Nicole Hollup, Crnp, Matthew Vibbert, Md, Coleen Vernick, Md, Giuliana Labella, Msn, Marie Wilson, Msn, Caitlin Harley, Msn, Maryanne Mccarrin, Msn, Jack Jallo, Md, Rebecca Jaffe, Md

House Staff Quality Improvement and Patient Safety Conference (2016-2019)

Introduction

• Transitions of care represent a major source of medical errors, patient morbidity/mortality, and increased healthcare waste.
• 2018 CLER report indicated largely unfavorable responses toward handoffs and care transitions for perioperative services and neurointensive care.
• Use of the IPASS handoff tool is associated with up to 30% reduction in adverse events and 23% reduction in medical errors.
• Implementation of IPASS for postoperative handoffs in the SICU resulted in improved organization, safety, and communication.

Generation Of Oligodendrocyte Progenitor Cells From Mouse Bone Marrow Cells., Yuan Zhang, Xin-Yu Lu, Giacomo Casella, Jing Tian, Ze-Qing Ye, Ting Yang, Juan-Juan Han, Ling-Yu Jia, A. M. Rostami, Xing Li

Department of Neurology Faculty Papers

Oligodendrocyte progenitor cells (OPCs) are a subtype of glial cells responsible for myelin regeneration. Oligodendrocytes (OLGs) originate from OPCs and are the myelinating cells in the central nervous system (CNS). OLGs play an important role in the context of lesions in which myelin loss occurs. Even though many protocols for isolating OPCs have been published, their cellular yield remains a limit for clinical application. The protocol proposed here is novel and has practical value; in fact, OPCs can be generated from a source of autologous cells without gene manipulation. Our method represents a rapid, and high-efficiency differentiation protocol for generating …

Peripheral Synucleinopathy In A Dj1 Patient With Parkinson Disease, Cataracts, And Hearing Loss, Derek P. Narendra, Risa Isonaka, Diana Nguyen, Alice B. Schindler, Angela D. Kokkinis, Debra Ehrlich, Tanya M. Bardakjian, David S. Goldstein, Tsao-Wei Liang, Pedro Gonzalez-Alegre

Department of Neurology Faculty Papers

No abstract provided.

Brainstem Atrophy In Focal Epilepsy Destabilizes Brainstem-Brain Interactions: Preliminary Findings., Susanne G. Mueller, Lisa M. Bateman, Maromi Nei, Alica M. Goldman, Kenneth D. Laxer

Department of Neurology Faculty Papers

BACKGROUND: MR Imaging has shown atrophy in brainstem regions that were linked to autonomic dysfunction in epilepsy patients. The brainstem projects to and modulates the activation state of several wide-spread cortical/subcortical regions. The goal was to investigate 1. Impact of brainstem atrophy on gray matter connectivity of cortical/subcortical structures and autonomic control. 2. Impact on the modulation of cortical/subcortical functional connectivity.

METHODS: 11 controls and 18 patients with non-lesional focal epilepsy (FE) underwent heart rate variability (HRV) measurements and a 3 T MRI (T1 in all subjects, task-free fMRI in 7 controls/ 12 FE). The brainstem was extracted, and atrophy …