Neurology Commons^™

Cdc Grand Rounds: National Amyotrophic Lateral Sclerosis (Als) Registry Impact, Challenges, And Future Directions, Paul Mehta, D. Kevin Horton, Edward J. Kasarskis, Ed Tessaro, M. Shira Eisenberg, Susan Laird, John Iskander

Neurology Faculty Publications

Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig’s disease, is a rapidly progressive fatal neurologic disease. Currently, there is no cure for ALS and the available treatments only extend life by an average of a few months. The majority of ALS patients die within 2–5 years of diagnosis, though survival time varies depending on disease progression (1,2). For approximately 10% of patients, ALS is familial, meaning it and has a genetic component; the remaining 90% have sporadic ALS, where etiology is unknown, but might be linked to environmental factors such as chemical exposures (e.g., heavy …

Lacosamide And Levetiracetam Have No Effect On Sharp-Wave Ripple Rate, Jan Kudlacek, Jan Chvojka, Antonin Posusta, Lubica Kovacova, Seung Bong Hong, Shennan A. Weiss, Md, Phd, Kamila Volna, Petr Marusic, Jakub Otahal, Premysl Jiruska

Department of Neurology Faculty Papers

Pathological high-frequency oscillations are a novel marker used to improve the delineation of epileptogenic tissue and, hence, the outcome of epilepsy surgery. Their practical clinical utilization is curtailed by the inability to discriminate them from physiological oscillations due to frequency overlap. Although it is well documented that pathological HFOs are suppressed by antiepileptic drugs (AEDs), the effect of AEDs on normal HFOs is not well known. In this experimental study, we have explored whether physiological HFOs (sharp-wave ripples) of hippocampal origin respond to AED treatment. The results show that application of a single dose of levetiracetam or lacosamide does not …

Iron Deficiency Anemia As A Risk Factor For Simple Febrile Seizures, Tipu Sultan, Abdul Islam Hanif Hanif, Shaila Ali

Pakistan Journal of Neurological Sciences (PJNS)

To determine the association of iron deficiency anemia with simple febrile seizures in children presenting in a tertiary care hospital.

A Silver Lining In The Dark Clouds: Medical Marijuana, In The Neurological Disorders And Its Future In Pakistan., Qamar Zaman, Maimoona Siddiqui

Pakistan Journal of Neurological Sciences (PJNS)

Marijuana has been included in the list of illicit drugs for a long time despite its use as a remedy for several medical problems. However this use has been on the basis of the individual experiences with no concrete scientific evidence. Its positive effects have been mostly overshadowed by its recreational use worldwide. It has recently become the topic of hot debate due to several studies showing its role in various medical and especially neurological disorders. These new findings have created a controversy and world is still divided on its legalization for medicinal purpose. Several countries and states have legalized …

Ependymomas Overexpress Chemoresistance And Dna Repairrelated Proteins, Sherise D. Ferguson, Shouhao Zhou, Joanne Xiu, Yuuri Hashimoto, Nader Sanai, Lyndon Kim, Santosh Kesari, John De Groot, David Spetzler, Amy B. Heimberger

Department of Neurosurgery Faculty Papers

Background: After surgery and radiation, treatment options for ependymoma are few making recurrence a challenging issue. Specifically, the efficacy of chemotherapy at recurrence is limited. We performed molecular profiling on a cohort of ependymoma cases in order to uncover therapeutic targets and to elucidate the molecular mechanisms contributing to treatment resistance. Results: This ependymoma cohort showed minimal alterations in gene amplifications and mutations but had high expression rates of DNA synthesis and repair enzymes such as RRM1 (47%), ERCC1 (48%), TOPO1 (62%) and class III β-tublin (TUBB3) (57%), which are also all associated with chemoresistance. This cohort also had high …

Interhospital Transfer Before Thrombectomy Is Associated With Delayed Treatment And Worse Outcome In The Stratis Registry (Systematic Evaluation Of Patients Treated With Neurothrombectomy Devices For Acute Ischemic Stroke)., Michael T Froehler, Jeffrey L Saver, Osama O Zaidat, Reza Jahan, Mohammad Ali Aziz-Sultan, Richard P Klucznik, Diogo C Haussen, Frank R Hellinger, Dileep R Yavagal, Tom L Yao, David S Liebeskind, Ashutosh P Jadhav, Rishi Gupta, Ameer E Hassan, Coleman O Martin, Hormozd Bozorgchami, Ritesh Kaushal, Raul G Nogueira, Ravi H Gandhi, Eric C Peterson, Shervin R Dashti, Curtis A Given, Brijesh P Mehta, Vivek Deshmukh, Sidney Starkman, Italo Linfante, Scott H Mcpherson, Peter Kvamme, Thomas J Grobelny, Muhammad S Hussain, Ike Thacker, Nirav Vora, Peng Roc Chen, Stephen J Monteith, Robert D Ecker, Clemens M Schirmer, Eric Sauvageau, Alex Abou-Chebl, Colin P Derdeyn, Lucian Maidan, Aamir Badruddin, Adnan H Siddiqui, Travis M Dumont, Abdulnasser Alhajeri, M Asif Taqi, Khaled Asi, Jeffrey Carpenter, Alan Boulos, Gaurav Jindal, Ajit S Puri, Rohan Chitale, Eric M Deshaies, David H Robinson, David F Kallmes, Blaise W Baxter, Mouhammad A Jumaa, Peter Sunenshine, Aniel Majjhoo, Joey D English, Shuichi Suzuki, Richard D Fessler, Josser E Delgado Almandoz, Jerry C Martin, Nils H Mueller-Kronast

Articles, Abstracts, and Reports

BACKGROUND: Endovascular treatment with mechanical thrombectomy (MT) is beneficial for patients with acute stroke suffering a large-vessel occlusion, although treatment efficacy is highly time-dependent. We hypothesized that interhospital transfer to endovascular-capable centers would result in treatment delays and worse clinical outcomes compared with direct presentation.

METHODS: STRATIS (Systematic Evaluation of Patients Treated With Neurothrombectomy Devices for Acute Ischemic Stroke) was a prospective, multicenter, observational, single-arm study of real-world MT for acute stroke because of anterior-circulation large-vessel occlusion performed at 55 sites over 2 years, including 1000 patients with severe stroke and treated within 8 hours. Patients underwent MT with or …

Structural Insights Into The Potency Of Sk Channel Positive Modulators, Young-Woo Nam, Razan Orfali, Tingting Liu, Kunqian Yu, Meng Cui, Heike Wulff, Miao Zhang

Pharmacy Faculty Articles and Research

Small-conductance Ca2+-activated K+ (SK) channels play essential roles in the regulation of cellular excitability and have been implicated in neurological and cardiovascular diseases through both animal model studies and human genetic association studies. Over the past two decades, positive modulators of SK channels such as NS309 and 1-EBIO have been developed. Our previous structural studies have identified the binding pocket of 1-EBIO and NS309 that is located at the interface between the channel and calmodulin. In this study, we took advantage of four compounds with potencies varying over three orders of magnitude, including 1-EBIO, NS309, SKS-11 (6-bromo-5-methyl-1H-indole-2,3-dione-3-oxime) and …

The Climate Of Neurofeedback: Scientific Rigour And The Perils Of Ideology, Robert T. Thibault, Michael Lifshitz, Amir Raz

Psychology Faculty Articles and Research

Over the last six decades, an in-group with ideological and financial stakes has been conducting sub-par research to develop an ostensibly effective clinical intervention: EEG-neurofeedback. More recently, however, a string of independent studies featuring increased scientific rigour and tighter experimental controls has challenged the foundation on which EEG-neurofeedback stands. Earlier this year, Brain published one of the most robust EEG-neurofeedback experiments to date (Schabus et al., 2017), which sparked a flurry of correspondence concerning the therapeutic value of neurofeedback (Fovet et al., 2017; Schabus, 2017); notably, a parallel discussion continues in Lancet Psychiatry …

Inhibitory Or Excitatory? Optogenetic Interrogation Of The Functional Roles Of Gabaergic Interneurons In Epileptogenesis, Hui Ye

Biology: Faculty Publications and Other Works

Alteration in the excitatory/inhibitory neuronal balance is believed to be the underlying mechanism of epileptogenesis. Based on this theory, GABAergic interneurons are regarded as the primary inhibitory neurons, whose failure of action permits hyperactivity in the epileptic circuitry. As a consequence, optogenetic excitation of GABAergic interneurons is widely used for seizure suppression. However, recent evidence argues for the context-dependent, possibly “excitatory” roles that GABAergic cells play in epileptic circuitry. We reviewed current optogenetic approaches that target the “inhibitory” roles of GABAergic interneurons for seizure control. We also reviewed interesting evidence that supports the “excitatory” roles of GABAergic interneurons in epileptogenesis. …

Reorganization Of Thalamic Intrinsic Connectivity Following Anterior Temporal Lobectomy, Xiaosong He, Chaitanya Ganne, Michael R. Sperling, Ashwini Sharan, Joseph I. Tracy

Department of Neurosurgery Posters

The thalamus has played a crucial role in determining surgical outcome following anterior temporal lobectomy (ATL) in temporal lobe epilepsy (TLE) (He, et al., 2017). Before successfully quantifying the impact of the thalamus on ATL outcomes, this structure’s own intrinsic connectivity must be understood. Accordingly, we characterize thalamic intrinsic connectivity through resting-state fMRI (rsfMRI) longitudinally, exploring its change pre- to post-surgery.

Intermediate-Term Risk Of Stroke Following Cardiac Procedures In A Nationally Representative Data Set., Laura Stein, Alison Thaler, John W. Liang, Stanley Tuhrim, Amit S. Dhamoon, Mandip S. Dhamoon

Department of Neurology Faculty Papers

BACKGROUND: Studies on stroke risk following cardiac procedures addressed only perioperative and long-term risk following limited higher-risk procedures, were poorly generalizable, and often failed to stratify by stroke type. We calculated stroke risk in the intermediate risk period following cardiac procedures compared with common noncardiac surgeries and medical admissions.

METHODS AND RESULTS: The Nationwide Readmissions Database contains readmission data for 49% of US admissions in 2013. We compared age-adjusted stroke readmission rates up to 90 days postdischarge. We used Cox regression to calculate hazard ratios, up to 1 year, of stroke risk comparing transcatheter aortic valve replacement versus surgical aortic …

X-Linked Hypomyelination With Spondylometaphyseal Dysplasia (H-Smd) Associated With Mutations In Aifm1., Noriko Miyake, Nicole I Wolf, Ferdy K Cayami, Joanna Crawford, Annette Bley, Dorothy Bulas, Ryan J Taft, Adeline Vanderver, +Several Additional Authors

Neurology Faculty Publications

An X-linked condition characterized by the combination of hypomyelinating leukodystrophy and spondylometaphyseal dysplasia (H-SMD) has been observed in only four families, with linkage to Xq25-27, and recent genetic characterization in two families with a common AIFM1 mutation. In our study, 12 patients (6 families) with H-SMD were identified and underwent comprehensive assessment accompanied by whole-exome sequencing (WES). Pedigree analysis in all families was consistent with X-linked recessive inheritance. Presentation typically occurred between 12 and 36 months. In addition to the two disease-defining features of spondylometaphyseal dysplasia and hypomyelination on MRI, common clinical signs and symptoms included motor deterioration, spasticity, tremor, …

Survival Benefit Of Surgery In Recurrent Glioblastoma Multiforme, Usama Khalid Choudry, Saad Akhtar Khan, Muhammad Shahzad Shamim

Section of Neurosurgery

There is an ongoing debate regarding role of surgery for recurrent glioblastoma multiforme (GBM). Older literature hinted at only modest survival benefits with surgery and a high rate of morbidity. However, more recent literature suggests better survival that may be attributed to better surgical techniques and better options in adjuvant treatment. Herein the authors review recent literature with regards to the possible role of surgery in recurrent GBM and also look into the key factors impacting second surgery.

Incidence Of Cognitively Defined Late-Onset Alzheimer's Dementia Subgroups From A Prospective Cohort Study., Paul K Crane, Emily Trittschuh, Shubhabrata Mukherjee, Andrew J Saykin, R Elizabeth Sanders, Eric B Larson, Susan M Mccurry, Wayne Mccormick, J D Bowen, Thomas Grabowski, Mackenzie Moore, Julianna Bauman, Alden L Gross, C Dirk Keene, Thomas D Bird, Laura E Gibbons, Jesse Mez

Articles, Abstracts, and Reports

INTRODUCTION: There may be biologically relevant heterogeneity within typical late-onset Alzheimer's dementia.

METHODS: We analyzed cognitive data from people with incident late-onset Alzheimer's dementia from a prospective cohort study. We determined individual averages across memory, visuospatial functioning, language, and executive functioning. We identified domains with substantial impairments relative to that average. We compared demographic, neuropathology, and genetic findings across groups defined by relative impairments.

RESULTS: During 32,286 person-years of follow-up, 869 people developed Alzheimer's dementia. There were 393 (48%) with no domain with substantial relative impairments. Some participants had isolated relative impairments in memory (148, 18%), visuospatial functioning (117, 14%), …

Neurological Burden In Baluchistan Province, Pakistan, Mohammad Saleem Barech

Pakistan Journal of Neurological Sciences (PJNS)

Total population of Balochistan is about13.16 million, while there are only nine qualified Neurologists in the whole province who look after not only neurological disorders of the province, but also patients from nearby countries like Afghanistan and Iran. The common neurological disorders are Stroke, Neuropathies, Myopathies, Epilepsy, Migraine, SSPE, Multiple Sclerosis, Meningitis, Myasthenia Gravis, Parkinson's disease and infectious diseases especially Tuberculosis, which is very common.

Correlation Of Duration Of Diabetes With Upper Limb Musculoskeletal Problems, Hazim Brohi, Rajesh Kumar, Aqiba Surfaraz, Tahira Parveen, M Usman, Syed Ijlal Ahmed

Pakistan Journal of Neurological Sciences (PJNS)

Long standing uncontrolled diabetes is associated with many clinical problems and it frequently involves hands and shoulder causing stenosing tenosynovitis (trigger finger), Dupuytren's contracture, carpal tunnel syndrome, limited joint mobility and shoulder capsulitis. The prevalence of hand symptoms has been variable in different studies. Data from our part of world is scares. Our study aimed at looking at frequency of these hand symptoms with duration of the diabetes.

Depression In Stroke Patients; A Cross Sectional Study Highlighting The Association Of Stroke On Age And Gender Basis., Saira Abbas, Naila Naeem Shahbaz, Muhammad Amir Umer, Sumera Rafat Umer, Asfia Irfan, Adnan Anwar

Pakistan Journal of Neurological Sciences (PJNS)

Stroke is the second leading cause of death. Depressive disorder is characterized by period of sad mood and anhedonia occurring for two consecutive weeks. Among stroke survivors, the consequence of physical and psychological changes can be devastating. One of those psychological changes is post-stroke depression (PSD). Stroke itself has debilitating morbidity and superimposed PSD further decreases the quality of life of patients and impairs recovery. This study leads us to know the magnitude of post stroke depression.

Dopamine Responsive Dystonia(Drd) In A 25 Year Old Lady, Samar Iltaf Pechuho, Abdul Rahman Soomro, Alam Ibrahim Siddiqui, Tufail Ahmed Pechuho, Imran Ali Gopang

Pakistan Journal of Neurological Sciences (PJNS)

Dopamine responsive dystonia (DRD) is a dystonic syndrome of childhood, usually affecting gait. It presents with walking difficulties, spasticity or dystonia with a characteristic diurnal variation and MRI brain scan is normal in it. It may however develop into Parkinsonism later, which shows dramatic therapeutic response to levodopa. A 25 year old lady presented with difficulty in walking along with weakness and stiffness of both legs at Neurology OPD of Chandka Medical College Hospital,Larkana. After reaching at proper diagnosis of DRD, treatment was started. As a result of which, she showed dramatic improvement and was able to return back to …

A Rare Case Of Becker Disease In A 7 Year Old Boy, Prem Chand, Rozmeen Badruddin Husein, Fazal M. Arain

Pakistan Journal of Neurological Sciences (PJNS)

Becker Disease is an autosomal recessive version of the rare congenital disorder called Myotonia Congenita. Due to the rarity of Becker Disease, the genetic and pathological basis of this disease have not been studied well and possible diagnostic methods and techniques are yet to be explored. The existing method of diagnosis of such a case is predominantly dependent on the clinical examination. More work and studies need to be done on the diagnostic aspect of this disease to discover newer diagnostic methods for this disease, which are more reliable and specific. In order to develop better diagnostic methods for the …

Epidemiological Data Of Neurological Disorders In Pakistan And Neighboring Countries: A Review, Ghulam Hussain, Azhar Rasul, Haseeb Anwar, Muhammad Umar Sohail, Syed Kashif Shahid Kamran, Shahid Mahmood Baig, Asghar Shabbir, Javed Iqbal

Pakistan Journal of Neurological Sciences (PJNS)

Neurological disorders are the impairments of nervous system and are an important and growing cause of morbidity, mortality, and disability. In addition to health costs, those suffering from these conditions are also frequently victimized of stigmatization and discrimination. Stigmatization further minimizes the patients' access to treatment and social activities. These disorders, therefore, require special attention particularly in developing countries where unfortunately, the burden of these disorders remains largely unrecognized. Moreover, the burden imposed by such chronic neurological conditions in general can be expected to be particularly devastating in poor populations. These conditions are emerging as severe public health concerns in …

5th National Headache Update, Abdul Malik

Pakistan Journal of Neurological Sciences (PJNS)

Headache is one of the most common disorders worldwide. On the World Health Organization's ranking of causes of disability, this would bring headache disorders into the 10 most disabling conditions for both genders, and into the five most disabling for women. The prevalence of headache disorders in Pakistan is high, and would be at least on par with that reported in other parts of the world, although a recent epidemiological survey suggests that the prevalence of migraines could be as high as 22%, almost twice that noted in the international literature. Over the years, the Pakistan Headache Society (PHS) has …

Trends In Spinal Surgery For Pott's Disease (2000-2016): An Overview And Bibliometric Study., Christian Fisahn, Fernando Alonso, Ghazwan A Hasan, R Shane Tubbs, Joseph R Dettori, Thomas A Schildhauer, Tarush Rustagi

Articles, Abstracts, and Reports

Study Design: Systematic review.

Objectives: (1) What are the surgical indications? Have they changed over time since the year 2000? (2) What is the current surgical approaches of choice? Have they changed over time since the year 2000? Do they vary by geographical region? (3) What are the most common outcome measures following surgery?

Methods: Electronic databases and reference lists of key articles were searched from database inception from January 1, 2000 to December 31, 2016 to identify studies specifically evaluating surgical indications, current surgical approaches, and outcome measures for spinal tuberculosis.

Results: Six randomized controlled trials were identified from …

Editorial Perspective., Jens R Chapman

Articles, Abstracts, and Reports

No abstract provided.

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

Articles, Abstracts, and Reports

No abstract provided.

Influence Of Dietary Salt Knowledge, Perceptions, And Beliefs On Consumption Choices After Stroke In Uganda, Martin N. Kaddumukasa, Elly Katabira, Martha Sajatovic, Svetlana Pundik, Mark Kaddumukasa, Larry B. Goldstein

Neurology Faculty Publications

Background

Previous research on Uganda's poststroke population revealed that their level of dietary salt knowledge did not lead to healthier consumption choices.

Purpose

Identify barriers and motivators for healthy dietary behaviors and evaluate the understanding of widely accepted salt regulation mechanisms among poststroke patients in Uganda.

Methods

Convergent parallel mixed methods triangulation design comprised a cross-sectional survey (n = 81) and 8 focus group discussions with 7-10 poststroke participants in each group. We assessed participant characteristics and obtained insights into their salt consumption attitudes, perceptions, and knowledge. Qualitative responses were analyzed using an inductive approach with thematic analytic procedures. Relationships …

Cognitive Trajectory Changes Over 20 Years Before Dementia Diagnosis: A Large Cohort Study., Ge Li, Eric B Larson, Jane B Shofer, Paul K Crane, Laura E Gibbons, Wayne Mccormick, J D Bowen, Mary Lou Thompson

Articles, Abstracts, and Reports

BACKGROUND/OBJECTIVES: Longitudinal studies have shown an increase in cognitive decline many years before clinical diagnosis of dementia. We sought to estimate changes, relative to "normal" aging, in the trajectory of scores on a global cognitive function test-the Cognitive Abilities Screening Instrument (CASI).

DESIGN: A prospective cohort study.

SETTING: Community-dwelling members of a U.S. health maintenance organization.

PARTICIPANTS: Individuals aged 65 and older who had no dementia diagnosis at baseline and had at least two visits with valid CASI test score (N = 4,315).

MEASUREMENTS: Average longitudinal trajectories, including changes in trajectory before clinical diagnosis in those who would be diagnosed …

Lefort 1 Access For Juvenile Nasopharyngeal Angiofibroma Treated Without Angiographic Embolization., Shaheen Ahmed, Muhammad Imran, Atiq Ahmed Khan, Anwar Ali, Syed Ijlal Ahmed, Asim Rehmani, Muhammad Faaiq Ali

Pakistan Journal of Neurological Sciences (PJNS)

To assess and analyze the clinical presentation of Juvenile nasopharyngeal angiofibroma and analyze the complications associated with JNA resection without pre surgical embolization using LeFort 1.

Pharygeal Cervicobrachial Variant Of Guillian -Barre Syndrome, Hazim Brohi, Marium Mustaqeem, Sundus Khan

Pakistan Journal of Neurological Sciences (PJNS)

Guilain-Barre Syndrome (GBS) is a common cause of Neuro-muscular paralysis with a spectrum of clinical and electrophysiology variants. We present a case of thePharyneo-cervico-brachial type. A 25 yr old male came to the Medical OPD with the complains of one day history of upper limb weakness and decreased flexion of the neck , which was progressively worsening, along-with associated shortness of breath. His systemic inquiry and past medical/surgical histories were insignificant. Physical exam correlated withthe diagnostic criteria of PCB variant of GBS. Diagnosis was confirmed on EMG/NCS,which showed axonal neuropathy involving axillay and musculocutaneousnerve, along with absent F –waves latencies …

New Oral Anticoagulants (Noacs) For Cerebral Venous Thrombosis, Maria Khan, Mohammad Wasay

Pakistan Journal of Neurological Sciences (PJNS)

Cerebral venous thrombosis (CVT) is a relatively rare condition that results from thrombosis of the superficial or deep venous sinuses of the brain. The first ever reported case of the condition was back in 1825 and this was diagnosed at autopsy. It took more than 150 years before imaging modalities were developed that could diagnose cases earlier. With the advent of CT and MR venogram, the diagnosis has become much easier and is contributing to a larger number of cases being picked up. Despite that the incidence in Western literature stays low at 1.3-1.6 cases per 100,000 people. Middle Eastern …

Early Electrophysiological Findings In Guillain Barre Syndrome, Hazim Brohi, Rajesh Kumar, Sadia Mubarak, Muhammmad Anees Mumtaz

Pakistan Journal of Neurological Sciences (PJNS)

Early electrophysiological testing is more important as early abnormalities can be obtained even in the first week. However the electrical abnormalities may not be that prominently evident for definitive diagnosis in the first 2 weeks. However at times the decision of treatment has to be taken early. Research is required to look at different electrophysiological parameter which could give clue for an earlier diagnosis. The aim of our study is to determine early electrophysiological parameters with in first week to identify GBS at earliest.