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myasthenia gravis cognition

Berit Jordan, Tabea L K Schweden, Theresa Mehl, Uwe Menge, Stephan Zierz
INTRODUCTION: Cognitive fatigue has frequently been reported in myasthenia gravis (MG). However, objective assessment of cognitive fatigability has never been evaluated. METHODS: Thirty-three MG patients with stable generalized disease and 17 healthy controls underwent a test battery including repeated testing of attention and concentration (d2-R) and PASAT (Paced Auditory Serial Addition Test). Fatigability was based on calculation of linear trend (LT) reflecting dynamic performance within subsequent constant time intervals...
December 29, 2016: Muscle & Nerve
Sen Hee Tay, Anna-Marie Fairhurst, Anselm Mak
BACKGROUND/PURPOSE: Neuropsychiatric (NP) events are found in patients with rheumatic diseases, commonly in systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS). The standard nomenclature and case definitions for 19 NPSLE syndromes by the American College of Rheumatology (ACR) Committee on Research cover a wide range of NP events seen in both SLE and SS. Despite advances in the understanding of SLE and SS, NP syndromes continue to pose diagnostic challenges. Correct attribution of NP events is critical in determining the correct treatment and prognosis...
February 2017: Autoimmunity Reviews
Katerina Markopoulou, Bruce A Chase, Piotr Robowski, Audrey Strongosky, Ewa Narożańska, Emilia J Sitek, Mariusz Berdynski, Maria Barcikowska, Matt C Baker, Rosa Rademakers, Jarosław Sławek, Christine Klein, Katja Hückelheim, Meike Kasten, Zbigniew K Wszolek
Olfactory dysfunction is associated with normal aging, multiple neurodegenerative disorders, including Parkinson's disease, Lewy body disease and Alzheimer's disease, and other diseases such as diabetes, sleep apnea and the autoimmune disease myasthenia gravis. The wide spectrum of neurodegenerative disorders associated with olfactory dysfunction suggests different, potentially overlapping, underlying pathophysiologies. Studying olfactory dysfunction in presymptomatic carriers of mutations known to cause familial parkinsonism provides unique opportunities to understand the role of genetic factors, delineate the salient characteristics of the onset of olfactory dysfunction, and understand when it starts relative to motor and cognitive symptoms...
2016: PloS One
Elaine Walklet, Kate Muse, Jane Meyrick, Tim Moss
Quality of life and well-being are frequently restricted in adults with neuromuscular disorders. As such, identification of appropriate interventions is imperative. The objective of this paper was to systematically review and critically appraise quantitative studies (RCTs, controlled trials and cohort studies) of psychosocial interventions designed to improve quality of life and well-being in adults with neuromuscular disorders. A systematic review of the published and unpublished literature was conducted. Studies meeting inclusion criteria were appraised using a validated quality assessment tool and results presented in a narrative synthesis...
August 30, 2016: Journal of Neuromuscular Diseases
Antonia Kaltsatou, Dimitris Fotiou, Dimitris Tsiptsios, Anastasios Orologas
BACKGROUND: The purpose of this study was to investigate with neurophysiological and neuropsychological methods such as pupillometry, cognitive test and Hamilton Depression Rating Scale (HAM-D) the hypothesis of Central Nervous System (CNS) cholinergic involvement in patients with Myasthenia Gravis (MG). METHODS: Thirty-two patients (32) with MG and a mean age of 51.1 ± 17.2 volunteered to participate in this investigation, while thirty-three (33) healthy subjects with a mean age of 50...
June 2015: BBA Clinical
Suresh Kumar
OBJECTIVES: The brain of mammals contains two major form of cholinesterase enzymes, acetylcholinesterase (AChE) and butyrylcholinesterase (BuChE). The dual inhibition of these enzymes is considered as a promising strategy for the treatment of neurological disorder such as Alzheimer's disease (AD), senile dementia, ataxia, and myasthenia gravis. The present study was undertaken to explore the anticholinesterase inhibition property of allicin. MATERIALS AND METHODS: An assessment of cholinesterase inhibition was carried out by Ellman's assay...
July 2015: Indian Journal of Pharmacology
Lien-Ying Lin, Ming-Hong Chang, Wei-Ju Lee
PURPOSE: Paraneoplastic limbic encephalitis (PLE) is a rare, immune-mediated entity. We present an unusual case of a patient who has double cancers and two different paraneoplastic neurological syndromes. CASE REPORT: A 58-year-old gentleman has histories of adenocarcinoma of lung and malignant thymoma associated with myasthenia gravis, which underwent surgery and chemotherapy 3 years ago. This time, he presented to our ward with rapidly progressive memory decline and myoclonic jerks in his limbs for two weeks...
September 2014: Acta Neurologica Taiwanica
Zhifeng Mao, Junjie Yin, Zhengqi Lu, Xueqiang Hu
The course of myasthenia gravis (MG) is complicated by increased reports of cognitive defects in both human and animal models, which suggests potential central nervous system (CNS) damage. We conducted a systematic review of the relationships between MG and cognitive function. This systematic review followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Major databases were searched to examine the neuropsychological studies of adults with MG. Weighted effect sizes were pooled by cognitive domain...
April 2015: Annals of Indian Academy of Neurology
Wenli Chen, Xiansi Zeng, Fucheng Luo, Tao Lv, Xiaoshuang Zhou, Jie Bai
Myasthenia gravis (MG) is an autoimmune disease caused by circulating antibodies that block acetylcholine receptor (AchR) at the neuromuscular junction. There is the cognitive and memory impairment in patients with MG. However, the molecular mechanisms underlying the alteration of central nervous system in MG remain unknown. In the present study, we found that the level of malondialdehyde (MDA) was increased in the brain of experimental autoimmune myasthenia gravis (EAMG). Furthermore, the expression of thioredoxin-1 (Trx-1) and the activity of cAMP response element-binding protein (CREB) were significantly decreased in frontal lobe and hippocampus of mice with EAMG...
August 2014: Neuromuscular Disorders: NMD
Philippe Couratier, Benoît Marin, Géraldine Lautrette, Marie Nicol, Pierre-Marie Preux
Amyotrophic Lateral Sclerosis (ALS) is the most common motor neuron disease in adults. Its incidence in France is estimated at 2.5 per 100,000 population and its prevalence between 5 and 8 per 100,000 inhabitants. Good prognostic factors are age of early onset, a longer time to diagnosis, initial damage to the spinal onset, early management of undernutrition and restrictive respiratory failure. The diagnosis of ALS is primarily clinical and is based on the evidence of involvement of the central motor neuron and peripheral neuron (NMP) in different territories or spinal or bulbar...
May 2014: La Presse Médicale
Daniel B Park, Joseph V Dobson, Joseph D Losek
OBJECTIVE: The objectives of this study were to highlight the intimate role that cognitive biases play in clinical decision making in the pediatric emergency department and to recommend strategies to limit their negative impact on patient care outcomes. METHODS: This was a descriptive study of 3 cases of presumed asthma exacerbation evolving into alternate diagnoses. RESULTS: The role cognitive biases played in either delay to diagnosis or missed diagnosis contributing to patient morbidity are illustrated in each case...
February 2014: Pediatric Emergency Care
Filipa Teixeira, Isabel Moreira, Ana Martins Silva, Carlos Vasconcelos, Fátima Farinha, Ernestina Santos
OBJECTIVES: To perform an observational retrospective cross-sectional case-control study to evaluate prevalence, clinical patterns and outcomes of neurological involvement in a cohort of Primary Sjögren Syndrome (pSS) patients followed up in a single center. MATERIAL AND METHODS: From a total of 93 pSS patients, diagnosed according to the 2002 criteria proposed by the American-European Consensus Group, we reviewed the clinical data of those with neurological complaints that were referred to observation by Neuroimmunology doctors...
January 2013: Acta Reumatológica Portuguesa
Shi Hui Wong, Jennifer C Nitz, Katrina Williams, Sandra G Brauer
INTRODUCTION: Studies of exercise in patients with myasthenia gravis (MG) are sparse. Balance strategy training (BST) multimodal exercise has proven efficacy in adults for enhancing balance and functional mobility. This prospective study aims to determine if BST improves functional ability and balance in people with MG. METHODS: Seven individuals with MG participated in a 16-session workstation intervention. Repeated measures (pre/post-intervention and 4-week follow-up) consisting of quantitative myasthenia gravis score (QMG), 6-minute walk test (6MWT), timed up and go (TUG) with dual task (TUG(manual) and TUG(cognitive)), and standing stability on foam with eyes closed (foamEC) were assessed...
May 2014: Muscle & Nerve
Rakez Kayed, George R Jackson, D Mark Estes, Alan D T Barrett
Alzheimer's disease (AD) is the most common neurodegenerative disorder. Currently available therapies are symptomatic but do not alter underlying disease progression. Immunotherapeutic approaches such as anti Aβ peptide active vaccination trials have had limited success to date. Intravenous immunoblobulin (IVIg) is widely used in immune-mediated neurological disorders such myasthenia gravis and Guillain-Barre syndrome. These preparations have been obtained from the pooled plasma of healthy human donors and contain natural anti-amyloid antibodies and are well tolerated...
2011: Journal of Central Nervous System Disease
Elliot D Freeman, Alberta Ipser, Austra Palmbaha, Diana Paunoiu, Peter Brown, Christian Lambert, Alex Leff, Jon Driver
The sight and sound of a person speaking or a ball bouncing may seem simultaneous, but their corresponding neural signals are spread out over time as they arrive at different multisensory brain sites. How subjective timing relates to such neural timing remains a fundamental neuroscientific and philosophical puzzle. A dominant assumption is that temporal coherence is achieved by sensory resynchronisation or recalibration across asynchronous brain events. This assumption is easily confirmed by estimating subjective audiovisual timing for groups of subjects, which is on average similar across different measures and stimuli, and approximately veridical...
November 2013: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
Fidias E Leon-Sarmiento, Edgardo A Bayona, Jaime Bayona-Prieto, Allen Osman, Richard L Doty
In this study we demonstrate that myasthenia gravis, an autoimmune disease strongly identified with deficient acetylcholine receptor transmission at the post-synaptic neuromuscular junction, is accompanied by a profound loss of olfactory function. Twenty-seven MG patients, 27 matched healthy controls, and 11 patients with polymiositis, a disease with peripheral neuromuscular symptoms analogous to myasthenia gravis with no known central nervous system involvement, were tested. All were administered the University of Pennsylvania Smell Identification Test (UPSIT) and the Picture Identification Test (PIT), a test analogous in content and form to the UPSIT designed to control for non-olfactory cognitive confounds...
2012: PloS One
S Lanfranconi, S Corti, P Baron, G Conti, L Borellini, N Bresolin, A Bersano
Muscle-specific tyrosine kinase- (MuSK-) antibodies-positive Myasthenia Gravis accounts for about one third of Seronegative Myasthenia Gravis and is clinically characterized by early onset of prominent bulbar, neck, shoulder girdle, and respiratory weakness. The response to medical therapy is generally poor. Here we report a case of late-onset MuSK-antibodies-positive Myasthenia Gravis presenting with signs of cognitive impairment and parkinsonism in addition to bulbar involvement and external ophthalmoplegia...
2011: Neurology Research International
P K Mukherjee, N Satheeshkumar, P Venkatesh, M Venkatesh
Acetylcholinesterase (AChE) inhibitors are considered as promising therapeutic agents for the treatment of several neurological disorders such as Alzheimer's disease (AD), senile dementia, ataxia and myasthenia gravis. There are only few synthetic medicines with adverse effects, available for treatment of cognitive dysfunction and memory loss associated with these diseases. A variety of plants has been reported to possess AChE inhibitory activity and so may be relevant to the treatment of neurodegenerative disorders such as AD...
March 2011: Mini Reviews in Medicinal Chemistry
Anna Sadnicka, Mary M Reilly, Cath Mummery, Sebastian Brandner, Nicholas Hirsch, Michael P T Lunn
A 76-year-old man with a pre-existing diagnosis of myasthenia gravis was admitted to an intensive care unit with pneumonia and type II respiratory failure. In addition, muscle weakness, widespread myokymia, neuropsychiatric disturbance and autonomic disturbance were present. Antivoltage gated potassium channel antibodies, antistriated muscle antibodies and antiacetylcholine receptor antibodies were positive. Nerve-conduction studies demonstrated findings consistent with patchy demyelination. Electromyography confirmed widespread myokymia, and there was evidence of diffuse encephalopathy on electroencephalography...
February 2011: Journal of Neurology, Neurosurgery, and Psychiatry
Michelle H Cameron, Eve L Klein
UNLABELLED: NARRATIVE REVIEW: In general, nervous system disorders present with changes in sensation, strength, and cognitive function that must be recognized early for the timely referral often needed for optimal outcome. This article summarizes screening for nervous system disorders in patients who present to the hand therapist and the typical findings associated with common neurologic disorders. Recommendations for referral by the hand therapist of patients with a screening examination consistent with a nervous system disorder are also presented...
April 2010: Journal of Hand Therapy: Official Journal of the American Society of Hand Therapists
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