Neurology Commons^™

Astrocytes Infected With Chlamydia Pneumoniae Demonstrate Altered Expression And Activity Of Secretases Involved In The Generation Of Β-Amyloid Found In Alzheimer Disease, Zein Al-Atrache, Danielle B Lopez, Susan Hingley, Denah Appelt

PCOM Scholarly Works

BACKGROUND: Epidemiologic studies strongly suggest that the pathophysiology of late-onset Alzheimer disease (AD) versus early-onset AD has environmental rather than genetic causes, thus revealing potentially novel therapeutic targets to limit disease progression. Several studies supporting the "pathogen hypothesis" of AD demonstrate a strong association between pathogens and the production of β-amyloid, the pathologic hallmark of AD. Although the mechanism of pathogen-induced neurodegeneration of AD remains unclear, astrocytes, a key player of the CNS innate immune response and producer/metabolizer of β-amyloid, have been implicated. We hypothesized that Chlamydia pneumoniae infection of human astrocytes alters the expression of the amyloid precursor protein …

A Closer Look At Arsa Activity In A Patient With Metachromatic Leukodystrophy., Kathleen Doherty, S. Barron Frazier, Matthew Clark, Anna Childers, Sumit Pruthi, David A. Wenger, Jessica Duis

Department of Neurology Faculty Papers

Metachromatic leukodystrophy (MLD) is an autosomal recessive lysosomal storage disease mainly caused by a deficiency of arylsulfatase A activity. The typical clinical course of patients with the late infantile form includes a regression in motor skills with progression to dysphagia, seizures, hypotonia and death. We present a case of a 4-year-old female with rapidly progressive developmental regression with loss of motor milestones, spasticity and dysphagia. MRI showed volume loss and markedly abnormal deep white matter. Enzymatic testing in one laboratory showed arylsulfatase A activity in their normal range. However, extraction of urine showed a large increase in sulfatide excretion in …

Early Progression Of Krabbe Disease In Patients With Symptom Onset Between 0 And 5 Months., Maria L. Beltran-Quintero, Nicholas A. Bascou, Michele D. Poe, David A. Wenger, Carlos A. Saavedra-Matiz, Matthew J. Nichols, Maria L. Escolar

Department of Neurology Faculty Papers

BACKGROUND: Krabbe disease is a rare neurological disorder caused by a deficiency in the lysosomal enzyme, β-galactocerebrosidase, resulting in demyelination of the central and peripheral nervous systems. If left without treatment, Krabbe disease results in progressive neurodegeneration with reduced quality of life and early death. The purpose of this prospective study was to describe the natural progression of early onset Krabbe disease in a large cohort of patients.

METHODS: Patients with early onset Krabbe disease were prospectively evaluated between 1999 and 2018. Data sources included diagnostic testing, parent questionnaires, standardized multidisciplinary neurodevelopmental assessments, and neuroradiological and neurophysiological tests.

RESULTS: We …

Treatment Combinations For Alzheimer’S Disease: Current And Future Pharmacotherapy Options, Jeffrey L. Cummings, Gary Tong, Clive Ballard

Integrated Health Sciences Faculty Research

Although Alzheimer’s disease (AD) is the world’s leading cause of dementia and the population of patients with AD continues to grow, no new therapies have been approved in more than a decade. Many clinical trials of single-agent therapies have failed to affect disease progression or symptoms compared with placebo. The complex pathophysiology of AD may necessitate combination treatments rather than monotherapy. The goal of this narrative literature review is to describe types of combination therapy, review the current clinical evidence for combination therapy regimens (both symptomatic and disease-modifying) in the treatment of AD, describe innovative clinical trial study designs that …

Hemi-Eses Associated With Agenesis Of The Corpus Callosum And Normal Cognition., Mahmoud Mohammadi, Safoura Kowkabi, Ali Akbar Asadi-Pooya, Reza Azizi Malamiri, Reza Shervin Badv

Department of Neurology Faculty Papers

• Corpus callosum plays the important role in bilateral synchronous expression of focal discharges of ESES.
• Sparing dominant hemisphere form continuous spike and slow waves during sleep accounts for normal cognitive scores.
• Early detection and treatment of ESES have a great impact on cognitive and language scores and final prognosis.

Gitelman Syndrome: A Rare Cause Of Seizure Disorder And A Systematic Review., Muhammad Asim Shahzad, Maryam Mukhtar, Asrar Ahmed, Waqas Ullah, Rehan Saeed, Mohsin Hamid

Abington Jefferson Health Papers

Gitelman syndrome is one of the few inherited causes of metabolic alkalosis due to salt losing tubulopathy. It is caused by tubular defects at the level of distal convoluted tubules, mimicking a thiazide-like tumor. It usually presents in late childhood or in teenage as nonspecific weakness, fatigability, polyuria, and polydipsia but very rarely with seizures. It is classically associated with hypokalemia, hypomagnesemia, hypocalciuria, hyperreninemia, and hyperaldosteronism. However, less frequently, it can present with normal magnesium levels. It is even rarer to find normomagnesemic patients of GS who develop seizures as the main complication since hypomagnesemia is considered the principal etiology …

Combination Therapy With Fingolimod And Neural Stem Cells Promotes Functional Myelination, Yuan Zhang, Xin-Yu Lu, Ze-Qin Ye, Bogoljub Ciric, Cun-Gen Ma, A. M. Rostami, Xing Li, Guang-Xian Zhang

Department of Neurology Faculty Papers

Myelination, which occurs predominantly postnatally and continues throughout life, is important for proper neurologic function of the mammalian central nervous system (CNS). We have previously demonstrated that the combination therapy of fingolimod (FTY720) and transplanted neural stem cells (NSCs) had a significantly enhanced therapeutic effect on the chronic stage of experimental autoimmune encephalomyelitis, an animal model of CNS autoimmunity, compared to using either one of them alone. However, reduced disease severity may be secondary to the immunomodulatory effects of FTY720 and NSCs, while whether this therapy directly affects myelinogenesis remains unknown. To investigate this important question, we used three myelination …

A Propensity-Matched Analysis Of Outcomes For Patients With M2 Branch Occlusions At Endovascular Stroke Centers, R. Ovando, L. Lucas, Elizabeth Baraban, Jason Tarpley

Articles, Abstracts, and Reports

Introduction: Endovascular therapy (EVT) for Emergent Large Vessel Occlusion (ELVO) is recommended for patients with acute proximal MCA (M1 segment) occlusions (Class I, level A evidence), but the benefits of EVT are uncertain in patients with M2 and more distal occlusions. The purpose of this study was to compare the efficacy and outcomes of EVT-treated M2 ELVOs with EVT-treated M1 ELVOs, and to examine the outcomes of EVT-treated M2 ELVO patients with those whose M2 ELVOs were not treated.

Methods: Data were obtained from a multi-hospital system of patients from January 2014 and May 2018. Two propensity score (PS)-based nearest-neighbor …

Primary Trauma Care: A Training Course For Healthcare Providers In Developing Countries, Saqib Kamran Bakhshi, Rashid Jooma

Section of Neurosurgery

Outcomes of injury in low and middle income countries may be compromised by skill deficiencies of healthcare providers. Short subspecialty training courses can be a useful solution to skill-deficits. We report on the Primary Trauma Care programme, a 2-day course designed to train frontline health workers in resuscitation and early management of the injured with limited resources and equipment. Developed for use in weak health systems and now conducted in over 70 countries, the programme is cost-efficient with built-in design sustainability by way of early transfer of ownership to local partners to effect a cascade of trauma courses in their …

Repeat Neuro-Imaging In Patients Presenting With Traumatic Extra-Axial Intracranial Hemorrhage, Austin Saline, Anthony Stefanelli, Md, Omaditya Khanna, Md, Sol Feuerwerker, Somnath Das, Evan Selzer

Phase 1

Introduction: The management of extra-axial intracranial hemorrhage patients is complicated and lacks a systemic algorithm to determine the timing and necessity of head computed tomography (HCT). However, repeat HCTs weakly predict the need for an intervention after mild traumatic brain injury (TBI).

Objective: This study focused on assessing the safety, efficacy, and necessity of obtaining serial HCTs in patients presenting with subdural hemorrhages.

Methods: A retrospective chart review was conducted of patients with subdural hemorrhages and a GCS between 13-15 on admission (n=116). The total number of HCTs, time between repeat HCTs, duration of hospital stay, and factors necessitating surgery …

Contact Sports As A Risk Factor For Amyotrophic Lateral Sclerosis: A Systematic Review., Ronen Blecher, Michael A Elliott, Emre Yilmaz, Joseph R Dettori, Rod J Oskouian, Akil P Patel, Andrew Clarke, Mike Hutton, Robert Mcguire, Robert Dunn, John Devine, Bruce Twaddle, Jens R Chapman

Articles, Abstracts, and Reports

Study Design: Systematic review.

Introduction: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease, ultimately resulting in paralysis and death. The condition is considered to be caused by a complex interaction between environmental and genetic factors. Although vast genetic research has deciphered many of the molecular factors in ALS pathogenesis, the environmental factors have remained largely unknown. Recent evidence suggests that participation in certain types of sporting activities are associated with increased risk for ALS.

Objective: To test the hypothesis that competitive sports at the highest level that involve repetitive concussive head and cervical spinal trauma result in an increased …

Editorial., Jeffrey C Wang, Jens R Chapman, Karsten Wiechert

Articles, Abstracts, and Reports

No abstract provided.

Improving Door-To-Groin Time For Stroke-Alert Patients Arriving At Tjuh, Megan Carney, Mackenzie O'Connor, Robin Dharia, Md

Phase 1

Introduction: Due to the large number of endovascular-eligible acute ischemic stroke patients, the urgent nature of effective stroke treatment protocols has become increasingly recognized at TJUH. Due to the hospital’s unique situation with two city blocks between the ER and endovascular lab, in-hospital factors remain a significant reason for the delay of treatment.

Objective: The purpose was to conduct a quality-assurance trial to determine whether standardizing imaging modalities for inpatient and ER stroke-alert patients at our institution would improve door-to-puncture times.

Methods: After implementation of the new stroke alert protocol, data were prospectively collected for six months for two groups …

Network Influence Of The Cerebellum For Predicting Dbs Response In Patients With Advanced Parkinson’S Disease, Lucy Li, Jennifer Muller, Sara Thalheimer, Mackenzie D. Silverman, Mahdi Alizadeh, Tsao-Wei Liang, Kelly Layton, Daniel Kremens, Victor Romo, Feroze Mohamed, Chengyuan Wu

Phase 1

Introduction: Deep brain stimulation (DBS) is a treatment option for reducing motor symptoms in patients with Parkinson’s Disease (PD) when first-line medication becomes ineffective. Existing literature has hypothesized that the clinical outcome of DBS may depend on brain connectivity profiles of the stimulation site to distant brain regions. However, the potential of brain connectivity profiles to predict response to DBS in PD remains unclear.

Objective: This study aimed to investigate how changes in structural and functional connectivity may relate to patient response to DBS, through the examination of brain network changes using graph theory.

Methods: Ten patients with advanced PD …

Managing Severe Tbi In The Icu Setting Using A Three Tier Approach, Mary Kay Bader

Books, Presentations, Posters, Etc.

Objectives:

• Assess the effects of injury to the brain in the neurologic injured patient and implement a tiered approach to manage severe TBI
• Evaluate TBI patients using multimodality monitoring
• Apply interventions to manage brain injury & integrate the complex multi-system issues and manage these patients using an actual case study.

Implementation Of Coordinated Telestroke Program In An Urban Setting Improves Acute Stroke Care, John Zurasky, Leslie Corless, Lindsay Lucas, Elizabeth Baraban

Books, Presentations, Posters, Etc.

Purpose: Telestroke has been shown to improve acute ischemic stroke (AIS) care in rural settings, but few studies have examined the impact of telestroke in an urban setting. In an urban area, there was a planned transition from an outpatient-based acute stroke provider pool to a centralized telehealth team of neurovascular and neurocritical care providers. This study assessed the impact of this change by comparing patient outcomes during three time periods: pre-initiation (PRE), transition after initiation (TRAN) and post-transition (POST).

Methods: Data for AIS patients 18 and older from five urban hospitals were used. Outcomes were hospital length of stay …

Impact Of Early Mobilization On 90-Day Outcomes In Thrombectomy Patients, Tamela Stuchiner, Diane Clark, Lindsay Lucas, John Robinson, Lisa Yanase

Books, Presentations, Posters, Etc.

Introduction: Early mobilization of ischemic stroke patients receiving IV alteplase (tPA) did not worsen 90-day outcome at two urban stroke centers in a prior study, but there are no studies evaluating outcomes of early mobilization after thrombectomy. The mobility protocol used in the previous study was also utilized to mobilize stroke patients receiving thrombectomy treatment at these two centers, once minimum number of hours for groin precautions was complete and the groin puncture site was stable. The objective of this study was to determine for post-thrombectomy patients mobilized within 24 hours, whether earlier mobilization worsened outcomes.

Methods: Medical records of …

Temperature Management: All Shook Up! Managing Shivering In Normothermia, Mary Kay Bader

Books, Presentations, Posters, Etc.

Objectives:

• Identify key factors related to maintaining normothermia
• Describe the use of the Bedside Shivering Assessment Scale
• Provide interventions to manage shivering in the Neuro patient population

Cost Analysis Of Implementing Standardized Stroke Patient Education Materials In A Large Five State Health System, Natalie Swearingen, Leslie Corless, Jessica Swann, Kailey Cox, Diane Lada, Elizabeth Baraban

Books, Presentations, Posters, Etc.

No abstract provided.

Outcomes And Characteristics Of Large Vessel Occlusion Patients In A Multi-Hospital System With National Institute Of Health Stroke Scale (Nihss) ≤ 5 Treated With Endovascular Thrombectomy Vs Medical Management, Steven Augustine, Lindsay Lucas, Elizabeth Baraban, Jason Tarpley

Books, Presentations, Posters, Etc.

No abstract provided.