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https://www.readbyqxmd.com/read/28645047/neurological-condition-assessed-with-the-hempel-examination-and-cognition-and-behaviour-at-4years
#1
Pamela Schendelaar, Jorien Seggers, Maas Jan Heineman, Mijna Hadders-Algra
AIM: To investigate associations between neurological condition, assessed with the Hempel examination, in terms of minor neurological dysfunction (MND) and neurological optimality, and cognition and behaviour at 4years. STUDY DESIGN: Cross-sectional analyses within a prospective, assessor-blinded follow-up study. SUBJECTS: Four-year-old singletons born to subfertile parents (n=235; 120 boys). OUTCOME MEASURES: Outcome parameters were complex minor neurological dysfunction (complex MND) and the neurological optimality score (NOS)...
June 20, 2017: Early Human Development
https://www.readbyqxmd.com/read/28631958/-anything-that-makes-life-s-journey-better-exploring-the-use-of-digital-technology-by-people-living-with-motor-neurone-disease
#2
Esther V Hobson, Saima Fazal, Pamela J Shaw, Christopher J McDermott
Our aim was to explore the attitudes of those living with motor neuron disease towards digital technology. Postal and online questionnaires surveyed 83 people with MND (pwMND) and 54 friends and family members (fMND). Five pwMND and five fMND underwent semi-structured interviews. 82% of pwMND and 87% of fMND use technology every day with iPads and laptops being the devices most commonly used. pwMND used technology to help them continue to participate in everyday activities such as socialising, entertainment and accessing the internet...
February 20, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28631955/cerebrospinal-fluid-neurofilament-light-concentration-in-motor-neuron-disease-and-frontotemporal-dementia-predicts-survival
#3
Tobias Skillbäck, Niklas Mattsson, Kaj Blennow, Henrik Zetterberg
OBJECTIVE: To aid diagnostics, patient stratification and studies seeking to find treatments for the related diseases motor neuron disease (MND) and frontotemporal dementia (FTD), there is a need to establish a way to assess disease severity and the amount of ongoing neurodegeneration. Previous studies have suggested that cerebrospinal fluid (CSF) neurofilament light (NFL) may serve this purpose. METHODS: We cross-referenced the Swedish mortality registry with the laboratory database at Sahlgrenska University Hospital to produce a dataset of CSF NFL concentrations and mortality information for 715 MND patients, 87 FTD patients, and 107 healthy controls...
February 6, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28616022/therapeutic-opportunities-and-challenges-of-induced-pluripotent-stem-cells-derived-motor-neurons-for-treatment-of-amyotrophic-lateral-sclerosis-and-motor-neuron-disease
#4
REVIEW
Manoj Kumar Jaiswal
Amyotrophic lateral sclerosis (ALS) and motor neuron diseases (MNDs) are progressive neurodegenerative diseases that affect nerve cells in the brain affecting upper and lower motor neurons (UMNs/LMNs), brain stem and spinal cord. The clinical phenotype is characterized by loss of motor neurons (MNs), muscular weakness and atrophy eventually leading to paralysis and death due to respiratory failure within 3-5 years after disease onset. No effective treatment or cure is currently available that halts or reverses ALS and MND except FDA approved drug riluzole that only modestly slows the progression of ALS in some patients...
May 2017: Neural Regeneration Research
https://www.readbyqxmd.com/read/28593798/nocebo-in-motor-neuron-disease-systematic-review-and-meta-analysis-of-placebo-controlled-clinical-trials
#5
Faiza Shafiq, Dimos-Dimitrios Mitsikostas, Panagiotis Zis
INTRODUCTION: Nocebo is very prevalent among neurological disorders, resulting in low adherence and treatment outcome. We sought to examine the AEs following placebo administration in placebo-controlled randomised clinical trials (RCTs) for motor neuron disease (MND). METHODS: After a systematic literature search for RCTs for MND pharmacotherapy treatments, we assessed the number of discontinuations because of placebo intolerance. RESULTS: Data were extracted from 12 RCTs fulfilling our search criteria...
June 8, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28588874/radiation-induced-tongue-myokymia-with-hypoglossal-nerve-damage-mimicker-of-motor-neuron-disease
#6
Anza B Memon, Kimberly A Playfoot
This teaching case of tongue myokymia offers clear electromyographic findings of myokymic discharges on two different sweeps. Radiation-induced tongue myokymia should be considered in the differential diagnosis for motor neuron disease (MND).
June 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28566149/clinical-profile-of-motor-neuron-disease-patients-with-lower-urinary-tract-symptoms-and-neurogenic-bladder
#7
Juan Francisco Vázquez-Costa, Salvador Arlandis, David Hervas, Esther Martínez-Cuenca, Fernando Cardona, Jordi Pérez-Tur, Enrique Broseta, Teresa Sevilla
INTRODUCTION: Lower urinary tract symptoms (LUTS) are frequent in motor neuron disease (MND) patients, but clinical factors related to them are unknown. We describe differences in LUTS among MND phenotypes and their relationship with other clinical characteristics, including prognosis. METHODS: For this study, we collected clinical data of a previously published cohort of patients diagnosed with classical amyotrophic lateral sclerosis (cALS), progressive muscular atrophy (PMA) or primary lateral sclerosis (PLS) with and without LUTS...
July 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28558552/the-patient-who-transformed-motor-neurone-disease-care
#8
Jennifer Trueland
In March 2015, Nursing Standard published a feature about a remarkable young man called Gordon Aikman. He had been diagnosed with motor neurone disease (MND) the previous year, aged 29, while working as director of research for the Better Together campaign, ahead of the Scottish independence referendum. The Scot, pictured, knew the prognosis wasn't good, and was determined to make the most of the time he had left.
May 31, 2017: Nursing Standard
https://www.readbyqxmd.com/read/28546998/uniparental-disomy-determined-by-whole-exome-sequencing-in-a-spectrum-of-rare-motoneuron-diseases-and-ataxias
#9
Dana M Bis, Rebecca Schüle, Jennifer Reichbauer, Matthis Synofzik, Tim W Rattay, Anne Soehn, Peter de Jonghe, Ludger Schöls, Stephan Züchner
BACKGROUND: The genetic causes of many rare inherited motoneuron diseases and ataxias (MND and ATX) remain largely unresolved, especially for sporadic patients, despite tremendous advances in gene discovery. Whole exome data is often available for patients, but it is rarely evaluated for unusual inheritance patterns, such as uniparental disomy (UPD). UPD is the inheritance of two copies of a chromosomal region from one parent, which may generate homozygosity for a deleterious recessive variant from only one carrier-parent...
May 2017: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/28532874/efficacy-of-kinesiology-tape-versus-postural-correction-exercises-on-neck-disability-and-axioscapular-muscles-fatigue-in-mechanical-neck-dysfunction-a-randomized-blinded-clinical-trial
#10
Aliaa M El-Abd, Abeer R Ibrahim, Haytham M El-Hafez
BACKGROUND: Mechanical neck dysfunction (MND), with axioscapular muscles fatigue, is highly prevalent worldwide. While postural correction is commonly used for its treatment, efficacy of kinesiology tape (KT) has received considerable attention. OBJECTIVES: To determine the effectiveness of KT versus correction exercises on neck disability, and axioscapular muscles fatigue in MND patients. METHODS: 46 MND patients were randomly assigned into 1 of 2 groups receiving 4 weeks treatment of either KT or correction exercises...
April 2017: Journal of Bodywork and Movement Therapies
https://www.readbyqxmd.com/read/28503469/motor-neuron-disease-presenting-with-acute-respiratory-failure-a-case-study
#11
Hyeonjun Oh, Seong Woong Kang, Won Ah Choi, Jang Woo Lee, Miri Suh, Eun Young Kim
Motor neuron diseases (MNDs) refer to a heterogeneous group of progressive neurologic disorders caused by degeneration of motor neurons. The diseases affect either the upper motor neurons, lower motor neurons, or both, and are characterized by weakness, atrophy, fasciculation, spasticity, and respiratory failure. We report a case of a 61-year-old male patient with no past history of cardiovascular or pulmonary disease, who presented with only dyspnea, and no indication of any other symptom such as muscle weakness, atrophy, or bulbar dysfunction...
April 2017: Annals of Rehabilitation Medicine
https://www.readbyqxmd.com/read/28502900/nuclear-morphometry-in-histological-specimens-of-canine-prostate-cancer-correlation-with-histological-subtypes-gleason-score-methods-of-collection-and-survival-time
#12
Guido Di Donato, Renée Laufer-Amorim, Chiara Palmieri
Ten normal prostates, 22 benign prostatic hyperplasia (BPH) and 29 prostate cancer (PC) were morphometrically analyzed with regard to mean nuclear area (MNA), mean nuclear perimeter (MNP), mean nuclear diameter (MND), coefficient of variation of the nuclear area (NACV), mean nuclear diameter maximum (MDx), mean nuclear diameter minimum (MDm), mean nuclear form ellipse (MNFe) and form factor (FF). The relationship between nuclear morphometric parameters and histological type, Gleason score, methods of sample collection, presence of metastases and survival time of canine PC were also investigated...
May 6, 2017: Research in Veterinary Science
https://www.readbyqxmd.com/read/28482893/decision-making-and-referral-processes-for-patients-with-motor-neurone-disease-a-qualitative-study-of-gp-experiences-and-evaluation-of-a-new-decision-support-tool
#13
Susan Baxter, Christopher J McDermott
BACKGROUND: The diagnosis of motor neurone disease (MND) is known to be challenging and there may be delay in patients receiving a correct diagnosis. This study investigated the referral process for patients who had been diagnosed with MND, and whether a newly-developed tool (The Red Flags checklist) might help General Practitioners (GPs) in making referral decisions. METHODS: We carried out interviews with GPs who had recently referred a patient diagnosed with MND, and interviews/surveys with GPs who had not recently referred a patient with suspected MND...
May 8, 2017: BMC Health Services Research
https://www.readbyqxmd.com/read/28461025/amyotrophic-lateral-sclerosis-or-not-keys-for-the-diagnosis
#14
REVIEW
T Lenglet, J-P Camdessanché
Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease (MND) which prognosis is poor. Early diagnosis permit to set up immediately adapted treatment and cares. Available diagnostic criteria are based on the detection of both central and peripheral motor neuron injury in bulbar, cervical, thoracic and lumbar regions. Electrodiagnostic (EDX) tests are the key tools to identify peripheral motor neuron involvement. Needle examination records abnormal activities at rest, and looks for neurogenic pattern during muscle contraction...
April 28, 2017: Revue Neurologique
https://www.readbyqxmd.com/read/28446118/motor-neurone-disease-progress-and-challenges
#15
REVIEW
Thanuja Dharmadasa, Robert D Henderson, Paul S Talman, Richard Al Macdonell, Susan Mathers, David W Schultz, Merrillee Needham, Margaret Zoing, Steve Vucic, Matthew C Kiernan
Major progress has been made over the past decade in the understanding of motor neurone disease (MND), changing the landscape of this complex disease. Through identifying positive prognostic factors, new evidence-based standards of care have been established that improve patient survival, reduce burden of disease for patients and their carers, and enhance quality of life. These factors include early management of respiratory dysfunction with non-invasive ventilation, maintenance of weight and nutritional status, as well as instigation of a multidisciplinary team including neurologists, general practitioners and allied health professionals...
May 1, 2017: Medical Journal of Australia
https://www.readbyqxmd.com/read/28440863/finger-extension-weakness-and-downbeat-nystagmus-motor-neuron-disease-syndrome-a-novel-motor-neuron-disorder
#16
Aline Delva, Nimish Thakore, Erik P Pioro, Koen Poesen, Rachel Saunders-Pullman, Inge A Meijer, Janet C Rucker, John T Kissel, Philip Van Damme
INTRODUCTION: Disturbances of eye movements are infrequently encountered in motor neuron diseases (MNDs) or motor neuropathies, and there is no known syndrome that combines progressive muscle weakness with downbeat nystagmus. METHODS: To describe the core clinical features of a syndrome of MND associated with downbeat nystagmus, clinical features were collected from 6 patients. RESULTS: All patients had slowly progressive muscle weakness and wasting in combination with downbeat nystagmus, which was clinically most obvious in downward and lateral gaze...
April 25, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28440098/early-diagnosis-of-amyotrophic-lateral-sclerosis-mimic-syndromes-pros-and-cons-of-current-clinical-diagnostic-criteria
#17
Elena Cortés-Vicente, Jesús Pradas, Juan Marín-Lahoz, Noemi De Luna, Jordi Clarimón, Janina Turon-Sans, Ellen Gelpí, Jordi Díaz-Manera, Isabel Illa, Ricard Rojas-Garcia
OBJECTIVE: To describe the frequency and clinical characteristics of patients referred to a tertiary neuromuscular clinic as having amyotrophic lateral sclerosis (ALS) but who were re-diagnosed as having an ALS mimic syndrome, and to identify the reasons that led to the revision of the diagnosis. METHODS: We reviewed the final diagnosis of all patients prospectively registered in the Sant Pau-MND register from 1 January 2004 to 31 December 2015. A detailed clinical evaluation and a clinically-guided electrophysiological study were performed at first evaluation...
April 25, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28431575/heterogeneous-ribonuclear-protein-a3-hnrnp-a3-is-present-in-dipeptide-repeat-protein-containing-inclusions-in-frontotemporal-lobar-degeneration-and-motor-neurone-disease-associated-with-expansions-in-c9orf72-gene
#18
Yvonne S Davidson, Louis Flood, Andrew C Robinson, Yoshihiro Nihei, Kohji Mori, Sara Rollinson, Anna Richardson, Bridget C Benson, Matthew Jones, Julie S Snowden, Stuart Pickering-Brown, Christian Haass, Tammaryn Lashley, David M A Mann
Frontotemporal Lobar Degeneration (FTLD) encompasses certain related neurodegenerative disorders which alter behaviour, personality and language. Heterogeneous ribonuclear proteins (hnRNPs) maintain RNA metabolism and changes in their function may underpin the pathogenesis of FTLD. Immunostaining for hnRNP A1, A2/B1 and A3 was performed on sections of temporal cortex with hippocampus from 61 patients with FTLD, stratified by pathological hallmarks into FTLD-tau and FTLD-TDP type A, B and C subtypes, and by genetics into patients with C9orf72 expansions, MAPT or GRN mutations, or those without known mutation...
April 21, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28429089/the-effectiveness-of-prophylactic-modified-neck-dissection-for-reducing-the-development-of-lymph-node-recurrence-of-papillary-thyroid-carcinoma
#19
Yasuhiro Ito, Akira Miyauchi, Takumi Kudo, Minoru Kihara, Mitsuhiro Fukushima, Akihiro Miya
AIM: The most frequent recurrence site of papillary thyroid carcinoma (PTC) is the cervical lymph nodes. The introduction of an electric linear probe for use with ultrasonography in 1996 improved preoperative lateral neck evaluations. Before 2006, however, our hospital routinely performed prophylactic modified neck dissection (p-MND) for N0 or N1a PTCs >1 cm to prevent node recurrence. In 2006, we changed our policy and the indications for p-MND to PTCs >3 cm and/or with significant extrathyroid extension...
April 20, 2017: World Journal of Surgery
https://www.readbyqxmd.com/read/28419177/interpreting-patterns-of-low-scores-on-the-nih-toolbox-cognition-battery
#20
James A Holdnack, David S Tulsky, Brian L Brooks, Jerry Slotkin, Richard Gershon, Allen W Heinemann, Grant L Iverson
Introduction: The National Institutes of Health Toolbox for Assessment of Neurological and Behavioral Function Cognition Battery is comprised of seven cognitive tests, including two tests measuring crystallized cognitive ability (i.e., vocabulary and reading) and five tests measuring fluid cognitive functioning (i.e., working memory, memory, speed of processing, and executive functioning). This study presents comprehensive base rate tables for the frequency of low scores in adults and older adults from the normative sample...
April 17, 2017: Archives of Clinical Neuropsychology: the Official Journal of the National Academy of Neuropsychologists
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