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Alberta Stroke Program Early Ct Score Infarct Location Predicts Outcome Following M2 Occlusion, Muhib Khan, Grayson L. Baird, Richard P. Goddeau, Brian Silver, Nils Henninger 2017 Spectrum Health

Alberta Stroke Program Early Ct Score Infarct Location Predicts Outcome Following M2 Occlusion, Muhib Khan, Grayson L. Baird, Richard P. Goddeau, Brian Silver, Nils Henninger

Open Access Articles

BACKGROUND: Although it is generally thought that patients with distal middle cerebral artery (M2) occlusion have a favorable outcome, it has previously been demonstrated that a substantial minority will have a poor outcome by 90 days. We sought to determine whether assessing the Alberta Stroke Program Early CT Score (ASPECTS) infarct location allows for identifying patients at risk for a poor 90-day outcome.

METHODS: We retrospectively analyzed patients with isolated acute M2 occlusion admitted to a single academic center between January 2010 and August 2012. Infarct regions were defined according to ASPECTS system on the initial head computed tomography. Discriminant ...


P30. Optimizing Current Steering In Deep Brain Stimulation For Treating Parkinsonian Axial Motor Symptoms, Daphne Hui 2017 Western University

P30. Optimizing Current Steering In Deep Brain Stimulation For Treating Parkinsonian Axial Motor Symptoms, Daphne Hui

Western Research Forum

Background: The proposed study will investigate deep brain stimulation (DBS), of the subthalamic nucleus (STN) to improve gait dysfunctions in advanced Parkinson’s Disease (PD). DBS requires implantation of electrodes into the brain that contain contacts where current traverses to stimulate neurons. Imprecise electrode implantations are inherent in surgical implantation; thus, contributing to the elusiveness of DBS on gait.

Methods: The proposed investigation will divide current between two contacts to receive 0, 30, 50, 70, or 100% of current, to find the best combination to improve gait. Gait changes associated with fractional combinations will be visually assessed with clinical scales ...


P35. Investigating The Effect Of Maternal Immune Activation On Sensory Filtering, Social Behaviour And Attention, Faraj Haddad 2017 Western University

P35. Investigating The Effect Of Maternal Immune Activation On Sensory Filtering, Social Behaviour And Attention, Faraj Haddad

Western Research Forum

Background

Altered brain development is associated with many neuropsychiatric disorders like Autism Spectrum Disorder (ASD) and schizophrenia. Environmental insults can interfere with neurodevelopment, and a prominent example is maternal infection during pregnancy. Epidemiological studies show that children born to mothers who were infected during pregnancy display a higher risk of developing ASD and schizophrenia, and this effect is mainly due to the maternal immune response. Polyinosinic-polycytidilic acid (Poly I:C) is a double stranded RNA molecule that mimics viral markers and elicits an immune response. When injected in pregnant rodents, this model produces offspring that exhibit core symptoms of ASD ...


Parental Quality Of Life 10 Years After Their Child’S Epilepsy Diagnosis, Klajdi Puka, Kathy Nixon Speechley 2017 Western University

Parental Quality Of Life 10 Years After Their Child’S Epilepsy Diagnosis, Klajdi Puka, Kathy Nixon Speechley

Western Research Forum

Background: Although the long-term course of childhood epilepsy is favorable in terms of seizure control, patients often face debilitating cognitive and psychosocial deficits that persist even after seizure remission. Pediatric epilepsy also has a large impact on the family and has been shown to be associated with diminished quality of life (QOL) among parents. However, the long-term outcome of parental QOL is unknown. This study aimed to 1) evaluate parental QOL 10 years after their child was diagnosed with epilepsy and 2) identify epilepsy-, child- and family-related characteristics associated with diminished parental QOL.

Methods: Data were derived from the Health-Related ...


Als-Linked Fus Exerts A Gain Of Toxic Function Involving Aberrant P38 Mapk Activation, Reddy Ranjith Kumar Sama, Claudia Fallini, Rodolfo Gatto, Jeanne E. McKeon, Yuyu Song, Melissa S. Rotunno, Saul Penaranda, Izrail Abdurakhmanov, John E. Landers, Gerardo Morfini, Scott T. Brady, Daryl Bosco 2017 University of Massachusetts Medical School

Als-Linked Fus Exerts A Gain Of Toxic Function Involving Aberrant P38 Mapk Activation, Reddy Ranjith Kumar Sama, Claudia Fallini, Rodolfo Gatto, Jeanne E. Mckeon, Yuyu Song, Melissa S. Rotunno, Saul Penaranda, Izrail Abdurakhmanov, John E. Landers, Gerardo Morfini, Scott T. Brady, Daryl Bosco

Open Access Articles

Mutations in Fused in Sarcoma/Translocated in Liposarcoma (FUS) cause familial forms of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease characterized by progressive axonal degeneration mainly affecting motor neurons. Evidence from transgenic mouse models suggests mutant forms of FUS exert an unknown gain-of-toxic function in motor neurons, but mechanisms underlying this effect remain unknown. Towards this end, we studied the effect of wild type FUS (FUS WT) and three ALS-linked variants (G230C, R521G and R495X) on fast axonal transport (FAT), a cellular process critical for appropriate maintenance of axonal connectivity. All ALS-FUS variants impaired anterograde and retrograde FAT in squid ...


Levodopa-Induced Morot Behavior Alterations In Dosophila Larvae, James Stanton 2017 University of Nebraska at Omaha

Levodopa-Induced Morot Behavior Alterations In Dosophila Larvae, James Stanton

Student Research and Creative Activity Fair

Exposing L-Dopa to Drosophila Melanogaster larvae and recording their induced dyskinetic behavior


Als Surveillance In Massachusetts: A One-Of-A-Kind Registry For Tracking An Elusive Disease, Vannalyn Abille, Alicia Fraser, Robert Knorr 2017 Massachusetts Department of Public Health

Als Surveillance In Massachusetts: A One-Of-A-Kind Registry For Tracking An Elusive Disease, Vannalyn Abille, Alicia Fraser, Robert Knorr

Community Engagement and Research Symposia

Background: Amyotrophic Lateral Sclerosis (ALS) is a progressive and fatal neuromuscular disease with an etiology not yet fully understood. The Massachusetts Argeo Paul Cellucci ALS Registry is a unique population-based surveillance system used to monitor the occurrence of ALS throughout the state and to explore possible environmental causes of the disease.

Methods: The Registry gathers annual reports of ALS cases from physicians, hospitals, and clinics in accordance with state regulations. Medical records are obtained and abstracted for each case, and eligible cases are reviewed by a consulting neurologist to confirm diagnosis. Necessary clinical and demographic information are recorded in a ...


Antisense Oligonucleotides Used To Target The Dux4 Mrna As Therapeutic Approaches In Faciosscapulohumeral Muscular Dystrophy (Fshd), Eugenie Ansseau, Celine Vanderplanck, Armelle Wauters, Scott Q. Harper, Frederique Coppee, Alexandra Belayew 2017 University of Mons

Antisense Oligonucleotides Used To Target The Dux4 Mrna As Therapeutic Approaches In Faciosscapulohumeral Muscular Dystrophy (Fshd), Eugenie Ansseau, Celine Vanderplanck, Armelle Wauters, Scott Q. Harper, Frederique Coppee, Alexandra Belayew

Wellstone Center for FSHD Publications and Presentations

FacioScapuloHumeral muscular Dystrophy (FSHD) is one of the most prevalent hereditary myopathies and is generally characterized by progressive muscle atrophy affecting the face, scapular fixators; upper arms and distal lower legs. The FSHD locus maps to a macrosatellite D4Z4 repeat array on chromosome 4q35. Each D4Z4 unit contains a DUX4 gene; the most distal of which is flanked by a polyadenylation site on FSHD-permissive alleles, which allows for production of stable DUX4 mRNAs. In addition, an open chromatin structure is required for DUX4 gene transcription. FSHD thus results from a gain of function of the toxic DUX4 protein that normally ...


Large Family Cohorts Of Lymphoblastoid Cells Provide A New Cellular Model For Investigating Facioscapulohumeral Muscular Dystrophy, Takako I. Jones, Charis L. Himeda, Daniel P. Perez, Peter L. Jones 2017 University of Massachusetts Medical School

Large Family Cohorts Of Lymphoblastoid Cells Provide A New Cellular Model For Investigating Facioscapulohumeral Muscular Dystrophy, Takako I. Jones, Charis L. Himeda, Daniel P. Perez, Peter L. Jones

Open Access Articles

Facioscapulohumeral muscular dystrophy (FSHD) is associated with aberrant epigenetic regulation of the chromosome 4q35 D4Z4 macrosatellite repeat. The resulting DNA hypomethylation and relaxation of epigenetic repression leads to increased expression of the deleterious DUX4-fl mRNA encoded within the distal D4Z4 repeat. With the typical late onset of muscle weakness, prevalence of asymptomatic individuals, and an autosomal dominant mode of inheritance, FSHD is often passed on from one generation to the next and affects multiple individuals within a family. Here we have characterized unique collections of 114 lymphoblastoid cell lines (LCLs) generated from 12 multigenerational FSHD families, including 56 LCLs from ...


Leukoaraiosis, Intracerebral Hemorrhage, And Functional Outcome After Acute Stroke Thrombolysis, Kannikar Kongbunkiat, Duncan Wilson, Narongrit Kasemsap, Somsak Tiamkao, Fatima Jichi, Vanessa Palumbo, Michael D. Hill, Alastair M. Buchan, Simon Jung, Heinrich P. Mattle, Nils Henninger, David J. Werring 2017 UCL Institute of Neurology

Leukoaraiosis, Intracerebral Hemorrhage, And Functional Outcome After Acute Stroke Thrombolysis, Kannikar Kongbunkiat, Duncan Wilson, Narongrit Kasemsap, Somsak Tiamkao, Fatima Jichi, Vanessa Palumbo, Michael D. Hill, Alastair M. Buchan, Simon Jung, Heinrich P. Mattle, Nils Henninger, David J. Werring

Psychiatry Publications and Presentations

OBJECTIVE: To perform a systematic review and pooled meta-analysis of published studies to assess whether the presence of leukoaraiosis on neuroimaging before treatment with thrombolysis (IV or intra-arterial) is associated with an increased risk of symptomatic intracerebral hemorrhage (sICH) or poor functional outcome.

METHODS: We included studies of patients with acute ischemic stroke, treated with IV or intra-arterial thrombolysis, which assessed functional outcome (3-month modified Rankin Scale [mRS]) or sICH in relation to leukoaraiosis on pretreatment neuroimaging (CT or MRI). We used random-effects models to calculate pooled relative risks (RR) of sICH and poor functional outcome (mRS > /= 2) for any ...


Herpes Simplex Virus Type Ii In A Patient Having Periodic Lateralized Epileptiform Discharges, Justin Chuang, Sona K. Shah 2017 Marshall University

Herpes Simplex Virus Type Ii In A Patient Having Periodic Lateralized Epileptiform Discharges, Justin Chuang, Sona K. Shah

Marshall Journal of Medicine

Herpes Simplex Encephalitis has been found to be one of the most common causes of fatal viral encephalitis (1). The mortality rate in untreated patients has been found to be as high as 70%, thus, correct management and early diagnosis are important for good prognoses and crucial for predicting outcomes. (1,2) In patients with Herpes Simplex Virus (HSV), specific findings such as the Periodic lateralized epileptic form discharges (PLEDs) are considered a specific finding on electroencephalogram (EEG). PLEDs are usually seen in the context of destructive structural lesions involving the cortex. Other causes may include acute ischemic stroke and ...


Brain Microvasculature Defects And Glut1 Deficiency Syndrome Averted By Early Repletion Of The Glucose Transporter-1 Protein, Maoxue Tang, Guangping Gao, Huapeng Li, Qin Su, Darryl C. De Vivo, Umrao R. Monani 2017 Columbia University Medical Center

Brain Microvasculature Defects And Glut1 Deficiency Syndrome Averted By Early Repletion Of The Glucose Transporter-1 Protein, Maoxue Tang, Guangping Gao, Huapeng Li, Qin Su, Darryl C. De Vivo, Umrao R. Monani

Open Access Articles

Haploinsufficiency of the SLC2A1 gene and paucity of its translated product, the glucose transporter-1 (Glut1) protein, disrupt brain function and cause the neurodevelopmental disorder, Glut1 deficiency syndrome (Glut1 DS). There is little to suggest how reduced Glut1 causes cognitive dysfunction and no optimal treatment for Glut1 DS. We used model mice to demonstrate that low Glut1 protein arrests cerebral angiogenesis, resulting in a profound diminution of the brain microvasculature without compromising the blood-brain barrier. Studies to define the temporal requirements for Glut1 reveal that pre-symptomatic, AAV9-mediated repletion of the protein averts brain microvasculature defects and prevents disease, whereas augmenting the ...


We Have No Real Evidence Related To Anticoagulation Plus Aspirin For Stroke Prevention In Atrial Fibrillation, Yuxiang Wang 2017 wayne state university school of medicine

We Have No Real Evidence Related To Anticoagulation Plus Aspirin For Stroke Prevention In Atrial Fibrillation, Yuxiang Wang

Clinical Research in Practice: The Journal of Team Hippocrates

A critical appraisal and clinical application of Flaker GC, Gruber M, Connolly SJ, et al. Risks and benefits of combining aspirin with anticoagulant therapy in patients with atrial fibrillation: an exploratory analysis of stroke prevention using an oral thrombin inhibitor in atrial fibrillation (SPORTIF) trials. Am Heart J. 2006;152:967-973. doi: 10.1016/j.ahj.2006.06.024


Three Damn Letters, Katherine Ammon 2017 Rollins College

Three Damn Letters, Katherine Ammon

Honors Program Theses

Amyotrophic lateral sclerosis (ALS) is a terminal disease that destroys a patient's motor neurons. In the play Three Damn Letters, a sarcastic woman must accept her own ALS diagnosis while also balancing her relationships with her father, husband, and son. This seriocomedic play explores themes of family roles, disability, love, and death.


Oxidative Stress And Proteomic Studies Of Mammalian Models Of Age-Related Metabolic Dysfunction In Neurodegenerative Disorders, Aaron M. Swomley 2017 University of Kentucky

Oxidative Stress And Proteomic Studies Of Mammalian Models Of Age-Related Metabolic Dysfunction In Neurodegenerative Disorders, Aaron M. Swomley

Theses and Dissertations--Chemistry

Expression proteomics is the field of science wherein proteins that make up the cellular proteome are identified both by name and by fold-change. Depending on the application of proteomics, this change in level could be due to internal cellular stressors or introduction of xenobiotics. Global oxidative stress measures use immunohistochemistry to determine the relative level of oxidative stress of macromolecules within the cell. In this dissertation, both global oxidative stress measures and expression proteomics were used in a variety of mammalian models in order to determine the effects of protein upregulation, intranasal insulin administration, and resveratrol supplementation on the cellular ...


Spinal Epidural Abscess Complicated By Meningitis, Sepsis And Thrombocytopenia In A Patient Lacking Traditional Risk Factors, Christian Spano, Michael Ward, Nicole Zagelbaum 2017 Touro College of Osteopathic Medicine (Middletown)

Spinal Epidural Abscess Complicated By Meningitis, Sepsis And Thrombocytopenia In A Patient Lacking Traditional Risk Factors, Christian Spano, Michael Ward, Nicole Zagelbaum

Touro College of Osteopathic Medicine (Middletown) Publications and Research

Spinal epidural abscess is a rare diagnosis with a classic triad of fever, spinal pain and neurologic deficits. Only a small proportion of patients have all three findings, making the diagnosis challenging. Here we present a case of cervical and thoracic spinal epidural abscess complicated by meningitis, sepsis and thrombocytopenia in a patient lacking traditional risk factors. The patient was initially treated non-operatively secondary to thrombocytopenia but subsequently required transfer to a tertiary care facility for surgical drainage after clinical deterioration. This case report highlights the need for a high index of suspicion and low threshold for imaging when considering ...


Metabolomic Profiling Of Chiari Malformation Type I: Comparison Of Bioinformatic Programs For Untargeted Analysis, Hunter W. Korsmo 2017 The University of Akron

Metabolomic Profiling Of Chiari Malformation Type I: Comparison Of Bioinformatic Programs For Untargeted Analysis, Hunter W. Korsmo

Honors Research Projects

Chiari Malformation Type I is a neurodegenerative trait that can result from disease or from acquiring. Metabolomic analysis was done on normal pressure hydrocephalous and Chiari CSF samples using LC-MS and multiple bioinformatic programs. After analysis from multiple programs, we were able to analyze the stringency in statistical algorithms done by each program and determined qualities that are shared between programs that offer multiple details. We identified dysregulation in glucuronated metabolites in CSF of Chiari versus NPH. Using LC-MS, we established the experimental MS/MS of glucuronic acid in attempt to identify similarities in mass-to-charge features primarily identified. We could ...


Meta Analysis Of Human Alzgene Database: Benefits And Limitations Of Using C. Elegans For The Study Of Alzheimer's Disease And Co-Morbid Conditions, Behrad Vahdati Nia, Christine Kang, Michelle G. Tran, Deborah Lee, Shin Murakami 2017 Touro University California

Meta Analysis Of Human Alzgene Database: Benefits And Limitations Of Using C. Elegans For The Study Of Alzheimer's Disease And Co-Morbid Conditions, Behrad Vahdati Nia, Christine Kang, Michelle G. Tran, Deborah Lee, Shin Murakami

College of Osteopathic Medicine (TUC) Publications and Research

Human genome-wide association studies (GWAS) and linkage studies have identified 695 genes associated with Alzheimer's disease (AD), the vast majority of which are associated with late-onset AD. Although orthologs of these AD genes have been studied in several model species, orthologs in the nematode, Caenorhabditis elegans, remain incompletely identified, with orthologs to only 17 AD-related genes identified in the C. elegans database, WormBase. Therefore, we performed a comprehensive search for additional C. elegans orthologs of AD genes using well-established programs, including OrthoList, which utilizes four ontology prediction programs. We also validated 680 of the AD genes as a unique ...


Iss-N1 Makes The First Fda-Approved Drug For Spinal Muscular Atrophy, Eric W. Ottesen 2017 Iowa State University

Iss-N1 Makes The First Fda-Approved Drug For Spinal Muscular Atrophy, Eric W. Ottesen

Biomedical Sciences Publications

Spinal muscular atrophy (SMA) is one of the leading genetic diseases of children and infants. SMA is caused by deletions or mutations of Survival Motor Neuron 1 (SMN1) gene. SMN2, a nearly identical copy of SMN1, cannot compensate for the loss of SMN1 due to predominant skipping of exon 7. While various regulatory elements that modulate SMN2 exon 7 splicing have been proposed, intronic splicing silencer N1 (ISS-N1) has emerged as the most promising target thus far for antisense oligonucleotide-mediated splicing correction in SMA. Upon procuring exclusive license from the University of Massachussets Medical School in 2010, Ionis Pharmaceuticals (formerly ...


Distinct Neural Bases Of Disruptive Behavior And Autism Symptom Severity In Boys With Autism Spectrum Disorder., Y.J. Daniel Yang, Denis G Sukhodolsky, Jiedi Lei, Eran Dayan, Kevin A. Pelphrey, Pamela Ventola 2017 George Washington University

Distinct Neural Bases Of Disruptive Behavior And Autism Symptom Severity In Boys With Autism Spectrum Disorder., Y.J. Daniel Yang, Denis G Sukhodolsky, Jiedi Lei, Eran Dayan, Kevin A. Pelphrey, Pamela Ventola

Pediatrics Faculty Publications

BACKGROUND: Disruptive behavior in autism spectrum disorder (ASD) is an important clinical problem, but its neural basis remains poorly understood. The current research aims to better understand the neural underpinnings of disruptive behavior in ASD, while addressing whether the neural basis is shared with or separable from that of core ASD symptoms.

METHODS: Participants consisted of 48 male children and adolescents: 31 ASD (7 had high disruptive behavior) and 17 typically developing (TD) controls, well-matched on sex, age, and IQ. For ASD participants, autism symptom severity, disruptive behavior, anxiety symptoms, and ADHD symptoms were measured. All participants were scanned while ...


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