Neurology Commons^™

Rare Case Of Diffuse Spinal Arachnoiditis Following A Complicated Vertebral Artery Dissection, Elias Atallah, Md, Sophia Dang, Sage Rahm, Nohra Chalouhi, Md, Stavropoula Tjoumakaris Md, Robert H. Rosenwasswer Md, Pascal Jabbour Md

Department of Neurosurgery Posters

Spinal arachnoiditis (SA) is an extremely rare and delayed complication of intracranial subarachnoid hemorrhage (SAH). SA is an inflammatory process leading to chronic fibrosis of the spinal cord. Possible pathophysiology is a two-staged disease of initial inflammatory reaction secondary to SAH, followed by a “free interval phase” prior to delayed adhesive phase (i.e. SA). The clinical course can be complicated and is the cause of major morbidity.

Cortical Thinning And Neuropsychiatric Outcomes In Children Exposed To Prenatal Adversity: A Role For Placental Crh?, Curt A. Sandman, Megan M. Curran, Elyssia Poggi Davis, Laura M. Glynn, Kevin Head, Tallie Z. Baram

Psychology Faculty Articles and Research

Exposure to early life adversity may disrupt the development and maturation of neurons and brain circuits, which, in turn, underlie neurodevelopment and mental illnesses. During fetal life, maternal adversity is conveyed to the developing brain via several molecular signals, including the stress hormone corticotropin releasing hormone (CRH). Employing a large well characterized prospective cohort, we find that fetal exposure to placental-origin CRH levels predicts structural and functional brain outcomes in children. Specifically, elevated placental CRH levels portend thinning of selective cortical regions of exposed individuals, with commensurate cognitive and emotional deficits. Notably, the relations of placental-origin CRH to cortical thinning …

Impact Of Supplemental Oxygen On Obstructive Sleep Apnea Of Infants, Piyush Das, R Kashyap, S Kotagal

Articles

Abstract: Treatment options may be limited for infants with obstructive sleep apnea when there is no surgically correctable upper airway lesion. We therefore evaluated, retrospectively, the efficacy of low-flow oxygen as a therapeutic option for infant obstructive sleep apnea. We reviewed the medical charts of 23 infants who had undergone a therapeutic trial of low-flow oxygen during polysomnography. Split-night polysomnography was used in 21/23 subjects while 2/23 had undergone two separate, full-night polysomnography sleep architecture and respiratory findings on the baseline polysomnogram segment that was obtained in room air were compared with the segment on low-flow oxygen (0.25–1 L/min). Wilcoxon …

Factors Associated With End-Of-Life Planning In Huntington Disease., Nancy R Downing, Siera Goodnight, Sena Chae, Joel S Perlmutter, Michael Mccormack, Elizabeth Hahn, Stacey K Barton, Noelle Carlozzi

Rowan-Virtua School of Osteopathic Medicine Departmental Research

OBJECTIVE: Knowledge of one's gene status for adult onset conditions provides opportunity to make advance end-of-life (EOL) plans. The purposes of these analyses were to (1) determine the prevalence of EOL plans, including advance directives (ADs) among persons across 3 stages of Huntington disease (HD) and (2) examine factors associated with having ADs in this sample.

METHODS: Data are from 503 participants in the HD Quality of Life study. Participants completed an online health-related quality-of-life survey that included questions regarding EOL planning and self-reported HD symptoms. Frequencies were calculated for EOL planning by the HD stage. Bivariate analysis and logistic …

Evaluating Cognition In Individuals With Huntington Disease: Neuroqol Cognitive Functioning Measures, Jin-Shei Lai, Siera Goodnight, Nancy R Downing, Rebecca E Ready, Jane S Paulsen, Anna L Kratz, Julie C Stout, Michael K Mccormack, David Cella, Christopher Ross, Jenna Russell, Noelle E Carlozzi

Rowan-Virtua School of Osteopathic Medicine Departmental Research

PURPOSE: Cognitive functioning impacts health-related quality of life (HRQOL) for individuals with Huntington disease (HD). The Neuro-QoL includes two patient-reported outcome (PRO) measures of cognition-Executive Function (EF) and General Concerns (GC). These measures have not previously been validated for use in HD. The purpose of this analysis is to evaluate the reliability and validity of the Neuro-QoL Cognitive Function measures for use in HD.

METHODS: Five hundred ten individuals with prodromal or manifest HD completed the Neuro-QoL Cognition measures, two other PRO measures of HRQOL (WHODAS 2.0 and EQ5D), and a depression measure (PROMIS Depression). Measures of functioning The Total …

Acute Disseminated Encephalomyelitis In A Middle Age Female, Hina Mushtaq, Maria Qureshi, Irfan Ahsan

Pakistan Journal of Neurological Sciences (PJNS)

Acute disseminated encephalomyelitis (ADEM) is an autoimmune condition that predominately involve demyelination of the white matter of the brain and spinal cord. It resembles other acute demyelinating syndromes like multiple sclerosis and most often presents in childhood.This is a case of a 52-year woman, presented with lower limb weakness, confusion and spasticity throughout the body with no preceding illness. CSF showed increased proteins with no oligoclonal bands. MRI Brain showed abnormal high intensity areas in bilateral parietal, periventricular region, with no contrast enhancement. She was given 1gram iv methylprednisolone based on the diagnosis of ADEM and responded well.

Conserved Brain Myelination Networks Are Altered In Alzheimer's And Other Neurodegenerative Diseases., Mariet Allen, Xue Wang, Jeremy D Burgess, Jens Watzlawik, Daniel J Serie, Curtis S Younkin, Thuy Nguyen, Kimberly G Malphrus, Sarah Lincoln, Minerva M Carrasquillo, Charlotte Ho, Paramita Chakrabarty, Samantha Strickland, Melissa E Murray, Vivek Swarup, Daniel H Geschwind, Nicholas T Seyfried, Eric B Dammer, James J Lah, Allan I Levey, Todd E Golde, Cory Funk, Hongdong Li, Nathan D Price, Ronald C Petersen, Neill R Graff-Radford, Steven G Younkin, Dennis W Dickson, Julia R Crook, Yan W Asmann, Nilüfer Ertekin-Taner

Articles, Abstracts, and Reports

INTRODUCTION: Comparative transcriptome analyses in Alzheimer's disease (AD) and other neurodegenerative proteinopathies can uncover both shared and distinct disease pathways.

METHODS: We analyzed 940 brain transcriptomes including patients with AD, progressive supranuclear palsy (PSP; a primary tauopathy), and control subjects.

RESULTS: We identified transcriptional coexpression networks implicated in myelination, which were lower in PSP temporal cortex (TCX) compared with AD. Some of these associations were retained even after adjustments for brain cell population changes. These TCX myelination network structures were preserved in cerebellum but they were not differentially expressed in cerebellum between AD and PSP. Myelination networks were downregulated in …

The Role Of The Leukemia Inhibitory Factor Receptor In Neuroprotective Signaling, Stephanie M. Davis, Keith R. Pennypacker

Center for Advanced Translational Stroke Science Faculty Publications

Several neurotropic cytokines relay their signaling through the leukemia inhibitory factor receptor. This 190kDa subunit couples with the 130kDa gp130 subunit to transduce intracellular signaling in neurons and oligodendrocytes that leads to expression of genes associated with neurosurvival. Moreover, activation of this receptor alters the phenotype of immune cells to an anti-inflammatory one. Although cytokines that activate the leukemia inhibitory factor receptor have been studied in the context of neurodegenerative disease, therapeutic targeting of the specific receptor subunit has been understudied in by comparison. This review examines the role of this receptor in the CNS and immune system, and its …

Biopsy Proven Case Of Creutzfeldt- Jacob Disease From Pakistan, Rizwana Shahid, Saad Shafqat

Pakistan Journal of Neurological Sciences (PJNS)

Creutzfeldt-Jakob disease (CJD) is a rare type of prion disease leading to a rapidly progressive dementia (RPD) and other associated neurological features specially startle myoclonus. The annual incidence of CJD has been reported to be 1 per million or 271 people in USA2, but from Asia especially from Pakistan, only few case reports have been published. The probable cause behind the rarity of this disease in our society is still unclear.The diagnosis of CJD requires a high level of suspicion and lack of the availability of specific markers like CSF 14-3-3 in our setup makes it more difficult. Similarly brain …

Migraine And Vascular Disease Biomarkers: A Population-Based Case-Control Study., Gretchen E. Tietjen, Jagdish Khubchandani, Nabeel Herial, Inge H. Palm-Meinders, Hille Koppen, Gisela M. Terwindt, Mark A. Van Buchem, Lenore J. Launer, Michel D. Ferrari, Mark C. Kruit

Department of Neurosurgery Faculty Papers

Background The underpinnings of the migraine-stroke association remain uncertain, but endothelial activation is a potential mechanism. We evaluated the association of migraine and vascular disease biomarkers in a community-based population. Methods Participants (300 women, 117 men) were recruited as a part of the Dutch CAMERA 1 (Cerebral Abnormalities in Migraine, an Epidemiologic Risk Analysis) study. Participants were aged 30-60 (mean 48) years, 155 migraine had with aura (MA), 128 migraine without aura (MO), and 134 were controls with no severe headaches. Plasma concentrations of fibrinogen, Factor II, D-dimer, high sensitivity C-reactive protein (hs-CRP), and von Willebrand factor antigen were compared …

Efficacy And Safety Of Eslicarbazepine Acetate Monotherapy In Patients Converting From Carbamazepine., Ladislav Pazdera, Michael R. Sperling, Jay H. Harvey, Maria C. Sam, Laura A. Strom, David Blum, Todd Grinnell, Hailong Cheng

Department of Neurology Faculty Papers

OBJECTIVE: To evaluate the influence of prior use of carbamazepine (CBZ) and other antiepileptic drugs (AEDs) with a putatively similar mechanism of action (inhibition of voltage-gated sodium channels; VGSCs) on seizure outcomes and tolerability when converting to eslicarbazepine acetate (ESL), using data pooled from 2 controlled conversion-to-ESL monotherapy trials (studies: 093-045, 093-046).

METHODS: Adults with treatment-resistant focal (partial-onset) seizures were randomized 2:1 to ESL 1600 or 1200 mg once daily. The primary efficacy endpoint was study exit (meeting predefined exit criteria related to worsening seizure control) versus an historical control group. Other endpoints included change in seizure frequency, responder rate, …

Cigarette Smoke Initiates Oxidative Stress-Induced Cellular Phenotypic Modulation Leading To Cerebral Aneurysm Pathogenesis., Robert M. Starke, John W. Thompson, Muhammad S. Ali, Crissey L. Pascale, Alejandra Martinez Lege, Dale Ding, Nohra Chalouhi, David M. Hasan, Pascal Jabbour, Gary K Owens, Michal Toborek, Joshua M. Hare, Aaron S. Dumont

Department of Neurosurgery Faculty Papers

OBJECTIVE: Cigarette smoke exposure (CSE) is a risk factor for cerebral aneurysm (CA) formation, but the molecular mechanisms are unclear. Although CSE is known to contribute to excess reactive oxygen species generation, the role of oxidative stress on vascular smooth muscle cell (VSMC) phenotypic modulation and pathogenesis of CAs is unknown. The goal of this study was to investigate whether CSE activates a NOX (NADPH oxidase)-dependent pathway leading to VSMC phenotypic modulation and CA formation and rupture.

APPROACH AND RESULTS: In cultured cerebral VSMCs, CSE increased expression of NOX1 and reactive oxygen species which preceded upregulation of proinflammatory/matrix remodeling genes …

Phosphaturic Mesenchymal Tumor Of The Nasal Cavity: Clinicopathologic Correlation Is Essential For Diagnosis, Aidan Kerr, Ryan Rimmer, Marc Rosen, James J. Evans, Madalina Tuluc, Stacey K. Mardekian

Department of Pathology, Anatomy, and Cell Biology Faculty Papers

Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm in which the tumor cells produce fibroblast growth factor 23 (FGF23), leading to oncogenic osteomalacia and thus a distinct clinical presentation. However, the pathologic findings of PMT are often non-specific and variable, especially in tumors occurring in the head and neck. We present a case of a 66-year-old female who presented with osteomalacia-related symptoms and was found to have a nasal cavity mass. Histopathologic examination was suggestive of PMT but certain characteristic features were lacking, requiring confirmation of the diagnosis by chromogenic in situ hybridization (CISH) assay for FGF23 mRNA. The patient's …

The Advocacy Role Of The World Federation Of Neurology, Wolfgang Grisold, Mohammad Wasay

Section of Neurology

No abstract provided.

Acute Onset Of Quadriplegia Secondary To Hypoparathyroidism: Mimicker Of Aman Variant Of Gbs, Syeda Saba Zaidi, Tahira Siddiqui, Sarwar Jamil Siddiqui

Section of Neurology

Acute onset of quadriplegia is a rare phenomenon seen with hypocalcemia due to hypoparathyroidism. We describe a 33-year gentleman who presented with weakness of all four limbs and areflexia. ECG showed QT abnormality. Nerve conduction study revealed normal sensory and significantly low motor CMAP amplitudes in both the upper and lower extremities. This nerve conduction study can be seen in acute motor neuropathy of various etiologies, among which GBS is the most worrisome. Our patient found to have low calcium and parathyroid hormone level. His symptoms improved after calcium replacement. Occurrence of quadriplegia in hypoparathyroidism, and its improvement after correction …

The Loose End: Neurology Without Rehabilitation, Ahmad Zaheer Qureshi, Farooq Azam Rathore

Pakistan Journal of Neurological Sciences (PJNS)

In recent years, successive therapies for previously untreatable neurological conditions have been introduced in clinical practice and these advance interventions have remarkably changed the landscape of neurosciences1,2 .But, rehabilitation of the functional impairments caused by neurological diseases is often overlooked or sometimes even neglected. In other words, the role of a comprehensive and early multidisciplinary rehabilitation in neurological disease is unrecognized and poorly understood even among neurologists and neurosurgeons. Usually, lifesaving neurosurgical management or solving a diagnostic riddle in neurology is attributed to successful patient management; however in reality, this is the first step in establishing a care plan for …

Importance Of Bioethics In Healthcare, Rizwan Taj, Asima Khan

Pakistan Journal of Neurological Sciences (PJNS)

Healthcare ethics deals with ethical issues in health care, medicine and science.Ethical codes and its application have long history from ancient era. In presentscenario, both advancement in medical field and the challenges are increasing in same pace for healthcare providers to deal with ethical issues. Therefore, the undoubtedly important role of bioethics emerges here. Bioethics in healthcare brings understanding and knowledge among healthcare professionals about medical practice.Stressing upon the ethical aspects of bioethics, medical professionals are capable oftagging along ethical codes while practicing especially while dealing with issues. As ethical tribulations are linked with issues in medical care, so by …

Effect Of Minor Surgery Under Propofol Anaesthesia On Brain Bdnf And Cognition, Muhammad Rafiq, Zahid Mahmood, Sajed Ali, Laure Pain

Pakistan Journal of Neurological Sciences (PJNS)

Brain Derived Neurotrophic Factor (BDNF) is a brain protein implicated in learning, memory and other cognitive functions. Changes in cellular brain functions as well as cognitive defects have been observed the days following anaesthesia, even for short-duration anaesthesia with/without surgery. Despites the role of neurotrophic factors in cognition, no data are still available on brain effects after anaesthesia. Purpose: To study the effect of minor surgery under short duration anaesthesia on cognition by investigating BDNF levels in plasma, hippocampus and cortex. METHODS: Male rats received an intra-peritoneal injection of either 120 mg/kg of propofol or intralipids solution or minor surgery …

Identification Of Different Personality Traits Among Teenagers And Their Effect On Behavioural Conduct, Muhammad Yasir Tarar, Umair Sharif, Muhammad Waqar Abbas

Pakistan Journal of Neurological Sciences (PJNS)

To identify different personality traits among teenagers To give recommendations to help teenagers create a balance in personality development. METHODS: This research is based on a cross sectional study conducted at Services Institute of Medical Sciences, catering to the freshmen of MBBS. The tenure for the research lasted 8 months starting March 2017- October 2017.The estimated target population accounted to 100 teenage students who were incorporated for the research through convenient sampling. Selection criteria for the chosen sample was based on their age, institute and mental well-being. Any student over and above 19 years, not enrolled in SIMS or suffering …

Risk Factors For Stroke And Tia's In Pakistan: A Community-Based Study, Abdul Malik, Farrukh Malik, Ismail Khatri, Sadaf Nasir

Pakistan Journal of Neurological Sciences (PJNS)

OBJECTIVES: To estimate the frequency of risk factors for stroke and transient ischemic attacks in Pakistan; a multi-city community-based study. METHOD: This cross-sectional community-based study was conducted in various health care facilities including government funded District Head Quarters hospitals, private hospitals and medical centers, and community-based clinics across 27 cities all over Pakistan over a period of three years between 2013 and 2015. Nearly 900 patients were screened during stroke (Falij) screening camps at these facilities and the data regarding past stroke and transient ischemic attack (TIA) symptoms were recorded along with relevant neurological examinations. A 25 item self-administered questionnaire …