Nervous System Diseases Commons^™

Qki-Mediated Cholesterol Biosynthesis In Eye Lens And Myelin Of The Central Nervous System, Seula Shin, Seula Shin

Dissertations and Theses (Open Access)

Cells obtain cholesterol in two ways, de novo biosynthesis and uptake from circulation. While most tissues utilize both sources, eye lens and brain depend extensively on cholesterol biosynthesis due to the limited supply from circulation. Lens cell membrane consists of highest portion of cholesterol. Brain is the most cholesterol-rich organ, which accounts for 23% of total cholesterol. Genetic mutations of cholesterol biosynthesis enzymes in humans and animal models present cataracts and hypomyelinating disorders linked to neurological impairment. Yet, it remains unclear how gene expression of cholesterol biosynthesis is regulated in lens and brain. Therefore, studying cholesterol biosynthesis in both tissues …

Qki-Mediated Cholesterol Biosynthesis In Eye Lens And Myelin Of The Central Nervous System, Seula Shin, Seula Shin

Dissertations and Theses (Open Access)

Cells obtain cholesterol in two ways, de novo biosynthesis and uptake from circulation. While most tissues utilize both sources, eye lens and brain depend extensively on cholesterol biosynthesis due to the limited supply from circulation. Lens cell membrane consists of highest portion of cholesterol. Brain is the most cholesterol-rich organ, which accounts for 23% of total cholesterol. Genetic mutations of cholesterol biosynthesis enzymes in humans and animal models present cataracts and hypomyelinating disorders linked to neurological impairment. Yet, it remains unclear how gene expression of cholesterol biosynthesis is regulated in lens and brain. Therefore, studying cholesterol biosynthesis in both tissues …

After A Horrid Year, Free Streaming Of ‘Dancing At The Vatican' Is An Inspirational Gift And A Call To Aid Huntington's Disease Families, Kenneth P. Serbin

At Risk for Huntington's Disease

No abstract provided.

Diagnosing And Managing Post-Stroke Aphasia, Shannon M. Sheppard, Rajani Sebastian

Communication Sciences and Disorders Faculty Articles and Research

Introduction: Aphasia is a debilitating language disorder and even mild forms of aphasia can negatively affect functional outcomes, mood, quality of life, social participation, and the ability to return to work. Language deficits after post-stroke aphasia are heterogeneous.

Areas covered: The first part of this manuscript reviews the traditional syndrome-based classification approach as well as recent advances in aphasia classification that incorporate automatic speech recognition for aphasia classification. The second part of this manuscript reviews the behavioral approaches to aphasia treatment and recent advances such as noninvasive brain stimulation techniques and pharmacotherapy options to augment the effectiveness of …

Happy Thanksgiving! And Hail To The Pharmaceutical And Biotech Industries – And The Scientists!, Kenneth P. Serbin

At Risk for Huntington's Disease

No abstract provided.

Efficacy And Safety Of Abobotulinumtoxina For Upper Limb Spasticity In Children With Cerebral Palsy: A Randomized Repeat-Treatment Study, Mauricio R. Delgado, Ann Tilton, Jorge Carranzo-Del Rio, Nigar Dursun, Marcin Bonikowski, Resa Aydin, Iwona Maciag-Tymecka, Joyce Oleszek, Edward Dabrowski, Anne-Sophie Grandoulier, Philippe Picaut, The Dysport In Pul Study Group

School of Medicine Faculty Publications

Aim: To assess the efficacy and safety of repeat abobotulinumtoxinA injections in reducing upper limb spasticity in children with cerebral palsy (CP). Method: This was a double-blind, repeat-cycle study (NCT02106351) in children with CP (2–17y). Children were randomized to receive 2U/kg (control), 8U/kg, or 16U/kg abobotulinumtoxinA injections into the target muscle group (wrist or elbow flexors) and additional muscles alongside occupational therapy via a home-exercise therapy program (HETP; minimum five 15min sessions/wk). Children received 8U/kg or 16U/kg plus HETP in cycles 2 to 4. Results: During cycle 1, 210 children (126 males, 84 females; mean age [SD] 9y [4y 5mo], …

Annual Huntington Study Group Meeting Reveals An Hd Community Energized To Aid Families, Develop Treatments, Kenneth P. Serbin

At Risk for Huntington's Disease

No abstract provided.

Chronic Inflammatory Demyelinating Polyneuropathy (Cidp) In A Patient With Diabetes: A Primary Care Perspective, Jordan Dever, Adam M. Franks, Laura M. Given, William Rollyson, Adrienne Mays-Kingston

Marshall Journal of Medicine

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a recurrent and progressive disease that causes proximal, symmetrical extremity weakness. The disease is diagnosed using clinical features, electrophysiologic testing, albumino-cytological disassociation in the cerebrospinal fluid, and sural nerve plexus biopsy. However, because of the low sensitivity of diagnostic criteria and other similar neuropathies, including diabetic polyneuropathy (DPN), accurate diagnosis is difficult. Differentiating between these diseases is especially important as CIDP’s changes are reversible and DPN’s are not. Making this differentiation allows for symptomatic improvement in a patient’s quality of life that would not be achieved otherwise. Early recognition and treatment, with modalities including …

Anesthetic Use In Status Epilepticus: A Concise Review, Furkan M. Yilmaz, Lauren A. Igneri, Yasir A. Yilmaz, Evren Burakgazi-Dalkilic

Cooper Rowan Medical Journal

The purpose of this review is to summarize the recent literature focusing on intravenous (IV) administration of commonly used anesthetics, including midazolam, pentobarbital/ thiopental, propofol, and ketamine in the treatment of status epilepticus (SE). Anesthetics should be reserved as third-line therapy for the treatment of SE when benzodiazepines and other second-line agents fail to terminate the seizure. We have reviewed the literature related to the treatment of status epilepticus, refractory status epilepticus, super-refractory status epilepticus, anesthetics, midazolam, pentobarbital/thiopental, propofol, and ketamine. This paper focuses on the pharmacology, dosages, major side effects, and clinical use of IV anesthetics in the treatment …

Gains Across Who Dimensions Of Function After Robot-Based Therapy In Stroke Subjects, Jennifer Wu, Lucy Dodakian, Jill See, Erin Burke Quinlan, Lisa Meng, Jeby Abraham, Ellen C. Wong, Vu Le, Alison Mckenzie, Steven C. Cramer

Physical Therapy Faculty Articles and Research

Background

Studies examining the effects of therapeutic interventions after stroke often focus on changes in loss of body function/structure (impairment). However, improvements in activities limitations and participation restriction are often higher patient priorities, and the relationship that these measures have with loss of body function/structure is unclear.

Objective

This study measured gains across WHO International Classification of Function (ICF) dimensions and examined their interrelationships.

Methods

Subjects were recruited 11 to 26 weeks after hemiparetic stroke. Over a 3-week period, subjects received 12 sessions of intensive robot-based therapy targeting the distal arm. Each subject was assessed at baseline and at 1 …

Subcutaneous Immunoglobulin Proves To Be An Effective Alternative To Intravenous Immunoglobulin In The Treatment Of Chronic Inflammatory Demyelinating Polyneuropathy, Benjamin Ross

Clinical Research in Practice: The Journal of Team Hippocrates

A clinical decision report appraising van Schaik IN, Bril V, van Geloven N, et al. Subcutaneous immunoglobulin for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (PATH): a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Neurol. 2018;17(1):35-46. https://doi.org/10.1016/S1474-4422(17)30378-2

Triplet Therapeutics Aims To Transform The Approach To Treating Huntington's Disease, Similar Disorders, Kenneth P. Serbin

At Risk for Huntington's Disease

No abstract provided.

Consequences Of A Missed History: A Case Of Antidepressant Discontinuation Syndrome, Sajid Hameed, Mukesh Kumar, Piyush Puri, Fnu Sapna, Pal Satyajit Singh Athwal

Section of Neurology

Antidepressant discontinuation syndrome (ADDS) is reported to occur in almost 30-50% of the patients who take antidepressants for a duration of at least four to six weeks and then suddenly discontinue the drug. Since there is an increase in the use of antidepressants for various reasons by general practitioners, patient education about when and how to discontinue a drug is not acknowledged enough. It is reported to occur with the use of different classes of antidepressants - selective serotonin reuptake inhibitor (SSRI), monoamineoxidase inhibitor (MAOI), tricyclic antidepressants (TCAs), and atypical antipsychotics like risperidone, trazodone, clozapine, and venlafaxine. Slow tapering off …

Effect Of Bout Length On Gait Measures In People With And Without Parkinson’S Disease During Daily Life, Vrutangkumar Shah, James Mcnames, Graham Harker, Martina Mancini, Patricia Carlson-Kuhta, John G. Nutt, Mahmoud El-Gohary, Carolin Curtze, Fay Horak

Electrical and Computer Engineering Faculty Publications and Presentations

Although the use of wearable technology to characterize gait disorders in daily life is increasing, there is no consensus on which specific gait bout length should be used to characterize gait. Clinical trialists using daily life gait quality as study outcomes need to understand how gait bout length affects the sensitivity and specificity of measures to discriminate pathological gait as well as the reliability of gait measures across gait bout lengths. We investigated whether Parkinson’s disease (PD) affects how gait characteristics change as bout length changes, and how gait bout length affects the reliability and discriminative ability of gait measures …

Plasma Proteins That May Cause Parkinson’S Disease And Multiple Sclerosis: A Mendelian Randomization Study, Brigid A. Staley

Dissertations and Theses

Multiple sclerosis (MS) and Parkinson’s disease (PD) are progressively disabling neurologic disorders that profoundly affect quality of life and shorten life expectancy. There is no cure for either disease, and current treatments only alleviate symptoms and may cause serious side effects. The causes of MS and PD are not well understood. Previous epidemiologic studies have documented numerous environmental risk factors for both diseases. However, these studies are inherently prone to bias from confounding which may generate spurious results. The lack of unbiased evidence on environmental causes of MS and PD has been a critical barrier to fully understanding their pathophysiology. …

Acute And Chronic Dosing Of A High-Affinity Rat/Mouse Chimeric Transferrin Receptor Antibody In Mice, Demi M. Castellanos, Jiahong Sun, Joshua Yang, Weijun Ou, Alexander C. Zambon, William M. Pardridge, Rachita K. Sumbria

Pharmacy Faculty Articles and Research

Non-invasive brain delivery of neurotherapeutics is challenging due to the blood-brain barrier. The revived interest in transferrin receptor antibodies (TfRMAbs) as brain drug-delivery vectors has revealed the effect of dosing regimen, valency, and affinity on brain uptake, TfR expression, and Fc-effector function side effects. These studies have primarily used monovalent TfRMAbs with a human constant region following acute intravenous dosing in mice. The effects of a high-affinity bivalent TfRMAb with a murine constant region, without a fusion partner, following extravascular dosing in mice are, however, not well characterized. Here we elucidate the plasma pharmacokinetics and safety of a high-affinity bivalent …

In This Electoral Season, Let’S Highlight Our Natural Role As Caregivers And Support Care Professionals, Kenneth P. Serbin

At Risk for Huntington's Disease

No abstract provided.

Comparison Of Machine Learning Models: Gesture Recognition Using A Multimodal Wrist Orthosis For Tetraplegics, Charlie Martin

The Journal of Purdue Undergraduate Research

Many tetraplegics must wear wrist braces to support paralyzed wrists and hands. However, current wrist orthoses have limited functionality to assist a person’s ability to perform typical activities of daily living other than a small pocket to hold utensils. To enhance the functionality of wrist orthoses, gesture recognition technology can be applied to control mechatronic tools attached to a novel fabricated wrist brace. Gesture recognition is a growing technology for providing touchless human-computer interaction that can be particularly useful for tetraplegics with limited upper-extremity mobility. In this study, three gesture recognition models were compared—two dynamic time-warping models and a hidden …

Occupational Therapy’S Role In Addressing Sex And Intimacy For Individuals With Progressive Neuromuscular Disorders, Lindsay N. Richards, Becki Cohill, Katrhyn Ellis, Susan Macdermott

San Marcos, Summer 2020

Individuals with progressive neuromuscular disorders (PND); specifically, Parkinson’s disease (PD), Multiple Sclerosis (MS), Huntington’s Disease (HD), and Amyotrophic Lateral Sclerosis (ALS) often face physical, psychological, and social challenges related to sex and intimacy. Occupational therapy (OT) practitioners are experts in activity analysis and are equipped with unique knowledge of performance skills and client factors to address deficits in occupational performance.

Though there is literature presenting the effects of PND on sexual occupations, a gap exists as it relates to qualitative data from the perspective of the individual and their partners. A mixed-methods survey was conducted examining the lived experience of …

Management Practices For West Syndrome In South Asia: A Survey Study And Meta-Analysis, Priyanka Madaan, Prem Chand, Kyaw Linn, Jithangi Wanigasinghe, Mimi Lhamu Mynak, Prakash Poudel, Raili Riikonen, Amit Kumar, Pooja Dhir, Sandeep Negi, Jitendra Kumar Sahu

Department of Paediatrics and Child Health

Objectives: Considering the dearth of literature on West syndrome (WS) from South Asian countries, this study aimed to evaluate the management practices in South Asia by an online survey and meta-analysis.
Methods: An online questionnaire was sent to 223 pediatric neurologists/pediatricians in India, Pakistan, Myanmar, Sri Lanka, Bhutan, Nepal, and Bangladesh. Their responses were evaluated and supplemented by a meta-analysis.
Results: Of 125 responses received (response rate: 56%), around 60% of responders observed male preponderance and an approximate lead-time-to-treatment (LTTT) of 4-12 weeks. The commonest etiology observed was a static structural insult (88.6% of responders). Most commonly used first-line drug …

Effect Of S100b Deletion On Membrane Properties And Localization Of Ncald And Hpca, Natasha Hesketh

Graduate School of Biomedical Sciences Theses and Dissertations

Calcium signaling is particularly important for neuronal function. Neurons utilize a wide range of calcium-binding proteins. Dysregulation of such proteins is linked to neurodegeneration. Neurocalcin delta (NCALD), hippocalcin (HPCA), and S100B are calcium sensors that are expressed in the hippocampus, a brain region essential to memory and severely damaged in Alzheimer’s disease (AD). Despite the potential importance of these proteins, we do not fully understand the physiological significance of their relationship. Because NCALD and HPCA are known to interact with S100B, we hypothesized that the loss of S100B affects NCALD and HPCA localization, and therefore electrical properties, of hippocampal neurons. …

A Fatal Case Of Primary Amoebic Meningoencephalitis (Pam) Complicated With Diabetes Insipidus (Di): A Case Report And Review Of The Literature, Zain Mushtaq, Saad Bin Zafar Mahmood, Adil Aziz

Section of Internal Medicine

Naegleria fowleri is a highly infective free-living amoeba usually isolated from soil and fresh water and is primarily found to infect the central nervous system (CNS) resulting in primary amoebic meningoencephalitis (PAM). PAM as a cause of meningitis is often overlooked for other, more common causes of meningitis. Despite all the advances in antimicrobial therapy and supportive care systems, the mortality rate of this rare infection remains above 95% with the bulk of the cases being found in developed countries. We are presenting a case of a 44-year-old male with fever, worsening headache, and generalized weakness. Lumbar puncture showed a …

Wonder If You’Ll Get Huntington’S Disease? Preparing For The Big, 'Intensely Personal' Decision To Undergo Predictive Testing, Kenneth P. Serbin

At Risk for Huntington's Disease

No abstract provided.

Free L-Glutamate-Induced Modulation In Oxidative And Neurochemical Profile Contributes To Enhancement In Locomotor And Memory Performance In Male Rats, Saiqa Tabassum, Saara Ahmad, Syeda Madiha, Sidrah Shahzad, Zehra Batool, Sadia Sadir, Saida Haider

Department of Biological & Biomedical Sciences

Glutamate (Glu), the key excitatory neurotransmitter in the central nervous system, is considered essential for brain functioning and has a vital role in learning and memory formation. Earlier it was considered as a harmful agent but later found to be useful for many body functions. However, studies regarding the effects of free L-Glu administration on CNS function are limited. Therefore, current experiment is aimed to monitor the neurobiological effects of free L-Glu in male rats. L-Glu was orally administered to rats for 5-weeks and changes in behavioral performance were monitored. Thereafter, brain and hippocampus were collected for oxidative and neurochemical …

Using A Timed Motor Task To Predict One-Year Functional Decline In Amnestic Mild Cognitive Impairment, Sydney Y. Schaefer, Andrew Hooyman, Kevin Duff

Physical Therapy Faculty Articles and Research

Affordable, noninvasive methods of predicting functional decline are needed for individuals at risk for Alzheimer’s disease. This study tested whether a timed upper-extremity motor task predicted functional decline over one year in 79 adults diagnosed with amnestic mild cognitive impairment. Participants completed subjective and objective measures of daily functioning at baseline and one year later. Motor task performance and delayed memory were also evaluated at baseline. Motor task performance was a significant predictor of one-year follow-up daily functioning, improving model fits by 18– 35%. Thus, motor behavior has potential to be an affordable enrichment strategy that is sensitive to functional …

Geriatrics In Clinical Practice And Beyond: Brain Health, The Sixth Vital Sign?, Nannette Hoffman

HCA Healthcare Journal of Medicine

Brain health in middle age and geriatric patients will take on greater importance for health care practitioners in our aging society. This article discusses various approaches clinicians can use for their patients to promote and improve brain health. These are generally the same lifestyle adoptions for cerebrovascular disease and cardiovascular disease prevention: weight management, exercise, and blood pressure and cholesterol lowering. Social stimulation and cognitive activities may also be important for the elderly.

A Case Of Disseminated Nocardia Beijingensis In An Immunocompetent Patient, Manifesting As Subcutaneous And Cns Nocardiosis., Scott Cathcart, Patricia Stogsdill, Rebecca Bell

Journal of Maine Medical Center

Introduction

Nocardiosis is an uncommon infection, typically encountered in immunocompromised patients. With only 5 cases reported thus far in the United States, we present a case of disseminated Nocardia beijingensis in an immunocompetent patient, involving the lung, subcutaneous tissue, and CNS.

Clinical Findings

A 60-year-old immunocompetent male with no significant medical history presents to our institution after a complicated medical course, beginning with vague respiratory complaints. Initial work-up revealed a large right upper lung mass of unclear etiology. A month later he had a syncopal episode followed by the development of focal neurologic deficits.

Main Diagnoses, Therapeutics Interventions, and Outcomes …

A Novel Indication For Oocyte Cryopreservation Prior To Autologous Hematopoietic Cell Transplantation For Refractory Multiple Sclerosis, Neal R. Trulock, Luke Ying, Edward Zbella, Mark Sanchez

West Florida Division GME Research Day 2020

Multiple sclerosis (MS) is an immune mediated inflammatory diseases of the central nervous system characterized by focal ovoid like lesions of demyelination seen on MRI(1,2). The etiology of MS is unknown, however inflammation followed by CNS demyelination and axonal degeneration are known to be part of the pathologic processes that lead to the disease (1). The mean age of onset of MS is 28-31 years old and tends to affect women more often then men with a ratio of 2.3:1 (3,4) . Other risk factors associated with MS include vitamin D deficiency, smoking, and certain viruses (5). The pattern of …

Challenges Of Doing Alzheimer’S Research In Costa Rica And Some Possible Solutions, Felipe Mejias

International Programs

Alzheimer's disease is a public health priority which has been researched for many years in the hope of finding a cure soon. Costa Rica has made significant progress in many areas of research; however, the research carried out in the country on Alzheimer's disease is very scarce. The present poster presents the challenges of developing scientific Alzheimer’s research in Costa Rica by reviewing current scientific research on Alzheimer's in Costa Rica and by conducting interviews with Costa Rican professionals who have conducted Alzheimer's research. The lack of investigative training of doctors, the difficulties in accessing patient data, and the lack …

Targeting The Transferrin Receptor To Develop Erythropoietin For Alzheimer’S Disease, Rachita K. Sumbria

Pharmacy Faculty Articles and Research

"Alzheimer’s disease (AD) is the sixth leading cause of death in the United States with approximately 5.8 million Americans currently living with AD. Due to the lack of a disease modifying treatment for AD and the aging baby boomer generation, this number is projected to grow to 13.8 million by 2050 (Gaugler et al., 2019). Amyloid-beta (Aβ) plaque accumulation, one of the major pathological hallmarks of AD, can begin > 20 years before clinical symptoms of AD. By the time AD is clinically diagnosed, neuronal loss and neuropathological lesions (Aβ plaques and tau tangles) have already occurred in many brain regions …