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Huan Yi, Bingbing Xie, Ben Liu, Xuan Wang, Li Xu, Jia Liu, Min Li, Xiufeng Zhong, Fuhua Peng
Induced pluripotent stem cells (iPSCs) have provided new opportunities for motor neuron disease (MND) modeling, drug screening, and cellular therapeutic development. Among the various types of iPSCs, urine-derived iPSCs have become a promising source of stem cells because they can be safely and noninvasively isolated and easily reprogrammed. Here, for the first time, we differentiated urine-derived iPSCs (urine-iPSCs) into motor neurons (MNs) and compared the capacity of urine-iPSCs and cord-blood-derived iPSCs (B-iPSCs) to differentiate into MNs...
2018: Stem Cells International
Denise Andrea Harris, Kirsten Jack, Christopher Wibberley
AIM: The aim of this study was to explore the meaning of living with uncertainty for people diagnosed with motor neurone disease (MND). BACKGROUND: MND is a progressive neurodegenerative condition resulting in multiple needs, arising from the complex nature of the disease trajectory. People with MND are often required to make decisions for symptom management and end-of-life care. Research into the lived experience of MND has previously highlighted: the shock of receiving such a diagnosis and prognosis; subsequent concerns relating to the future and loss; and the existential suffering for a person with MND...
March 8, 2018: Journal of Clinical Nursing
Lieke H H Meeter, Everard G Vijverberg, Marta Del Campo, Annemieke J M Rozemuller, Laura Donker Kaat, Frank Jan de Jong, Wiesje M van der Flier, Charlotte E Teunissen, John C van Swieten, Yolande A L Pijnenburg
OBJECTIVE: To examine the clinical value of neurofilament light chain (NfL) and the phospho-tau/total tau ratio (p/t-tau) across the entire frontotemporal dementia (FTD) spectrum in a large, well-defined cohort. METHODS: CSF NfL and p/t-tau levels were studied in 361 patients with FTD: 179 behavioral variant FTD, 17 FTD with motor neuron disease (FTD-MND), 36 semantic variant primary progressive aphasia (PPA), 19 nonfluent variant PPA, 4 logopenic variant PPA (lvPPA), 42 corticobasal syndrome, and 64 progressive supranuclear palsy...
March 7, 2018: Neurology
Bárbara Carolina Brandão, Alline de Sousa Galdino, Luciano Garcia Lourenção, Glaucia Santana Trindade, Magali Aparecida Orate Menezes da Silva, Roberta Gonçalves da Silva
OBJECTIVE: Describe and correlate bulbar functionality with laryngeal penetration and/or laryngotracheal aspiration for different food consistencies in Motor Neuron Disease (MND). METHODS: Study participants were 18 individuals diagnosed with MND regardless of the type and time of onset of disease. The Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised/BR (ALSFRS-R/BR) was applied, and only the bulbar parameter, which includes speech, salivation and swallowing, was analyzed, with scores raging from 0 (disability) to 12 (normal functionality)...
March 5, 2018: CoDAS
Philippe Corcia, Patrick Vourc'h, Helene Blasco, Philippe Couratier, Audrey Dangoumau, Remi Bellance, Claude Desnuelle, Fausto Viader, Vivien Pautot, Stephanie Millecamps, Salah Bakkouche, FranÇois Salachas, Christian R Andres, Vincent Meininger, William Camu
BACKGROUND: Amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA) are the most frequent motor neuron disorders in adulthood and infancy, respectively. There is a growing literature supporting common pathophysiological patterns between those disorders. One important clinical issue for that is the co-occurrence of both diseases within a family. OBJECTIVES: To collect families in which ALS and SMA patients co-exist and describe the phenotype and the genotype of ALS patients...
March 1, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Martin Crivello, Saidhbhe L O'Riordan, Ina Woods, Sarah Cannon, Luise Halang, Karen S Coughlan, Marion C Hogg, Sebastian A Lewandowski, Jochen H M Prehn
Loss-of-function mutations in the angiogenin (ANG) gene have been identified in familial and sporadic ALS patients. Previous work from our group identified human ANG (huANG) to protect motoneurons in vitro, and provided proof-of-concept that daily intraperitoneal (i.p.) huANG injections post-symptom onset increased lifespan and delayed disease progression in SOD1G93A mice. huANG's mechanism of action remains less well understood. Here, we implemented a preclinical in vivo design to validate our previous results, provide pharmacokinetic and protein distribution data after systemic administration, and explore potential pleiotropic activities of huANG in vivo...
February 24, 2018: Neuropharmacology
David Morley, Sarah Dummett, Laura Kelly, Ray Fitzpatrick, Crispin Jenkinson
BACKGROUND: Comparisons between neurological conditions have the potential to inform service providers by identifying particular areas of difficulty experienced by affected individuals. This study aimed to identify predictors of activity and participation in people with motor neurone disease (MND), people with multiple sclerosis (MS) and people with Parkinson's Disease (PD). METHODS: The Oxford Participation and Activities Questionnaire (Ox-PAQ) and Medical Outcomes Study 36-Item Short Form Survey (MOS SF-36) were administered by postal survey to 386 people with a confirmed diagnosis of MND, MS or PD...
February 17, 2018: BMC Neurology
Elena Lobo, Guillermo Marcos, Javier Santabárbara, Luis Lobo-Escolar, Helena Salvador-Rosés, Concepción De la Cámara, Raul Lopez-Anton, Patricia Gracia-García, Antonio Lobo-Escolar
OBJECTIVES: To test the hypothesis that differences by gender will be observed in the association of hip fracture risk with stages of cognitive impairment; and to explore the association between Petersen's "mild cognitive impairment" (MCI) and DSM-5 "mild neurocognitive disorder" (MND). STUDY DESIGN: A community sample of 4803 individuals aged 55+ years was assessed in a two-phase case-finding enquiry in Zaragoza, Spain, and was followed up for 16 years...
March 2018: Maturitas
Enrica Bersano, Maria Francesca Sarnelli, Valentina Solara, Fabiola De Marchi, Gian Mauro Sacchetti, Alessandro Stecco, Lucia Corrado, Sandra D'alfonso, Roberto Cantello, Letizia Mazzini
We describe a 64-year-old woman, suffering from late-onset obsessive-compulsive disorder (OCD) from the age of 57, who developed dysarthria and dysphagia, spastic diplegic, and proximal muscles weakness. Needle electromyography showed no active denervation. Neuropsychological evaluation showed intact cognitive functioning. We diagnosed upper motor neuron disease (MND), with no known genetic correlates. Brain magnetic resonance (MRI) detected bilateral hippocampal atrophy with sclerosis of right hippocampus...
February 16, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Esther V Hobson, Wendy O Baird, Rebecca Partridge, Cindy L Cooper, Susan Mawson, Ann Quinn, Pamela J Shaw, Theresa Walsh, Daniel Wolstenholme, Christopher J Mcdermott
OBJECTIVES: Attendance at a specialist multidisciplinary motor neurone disease (MND) clinic is associated with improved survival and may also improve quality of life and reduce hospital admissions. However, patients struggle to travel to clinic and may experience difficulties between clinic visits that may not be addressed in a timely manner. We wanted to explore how we could improve access to specialist MND care. METHODS: We adopted an iterative, user-centered co-design approach, collaborating with those with experience of providing and receiving MND care including patients, carers, clinicians, and technology developers...
February 16, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Rebecca F D'Cruz, Patrick B Murphy, Georgios Kaltsakas
Motor neurone disease (MND) is a neurodegenerative disease defined by axonal loss and gliosis of upper and lower motor neurones in the motor cortex, lower brainstem nuclei and ventral horn of the spinal cord. MND is currently incurable and has a poor prognosis, with death typically occurring 3 to 5 years after disease onset. The disease is characterised by rapidly progressive weakness leading to paralysis, fasciculations, bulbar symptoms (including dysarthria and dysphagia) and respiratory compromise. Respiratory complications arise as a result of weakness of upper airway (pharyngeal and laryngeal) muscles and respiratory muscles (diaphragm, intercostal and accessory muscles) leading to respiratory failure...
January 2018: Journal of Thoracic Disease
Zengcai Guo, Yuming Guan, Chengxiang Dai, Jingbo Mu, Hongwei Che, Guangshuo Wang, Xiaoliang Zhang, Zhixiao Zhang, Xiliang Zhang
A one-dimensional hierarchical Ag nanoparticle (AgNP)/MnO2 nanorod (MND) nanocomposite was synthesized by combining a simple solvothermal method and a facile reduction approach in situ. Owing to its high electrical conductivity, the resulting AgNP/MND nanocomposite displayed a high specific capacitance of 314 F g-1 at a current density of 2 A g-1, which was much higher than that of pure MNDs (178 F g-1). Resistances of the electrolyte (Rs) and charge transportation (Rct) of the nanocomposite were much lower than that of pure MNDs...
July 1, 2018: Journal of Nanoscience and Nanotechnology
Yeong Jeong Jeon, Yong Soo Choi, Kyung Jong Lee, Se Hoon Lee, Hongryull Pyo, Joon Young Choi
Background: We evaluated the feasibility and outcomes of pulmonary resection and mediastinal node dissection (MND) by video-assisted thoracoscopic surgery (VATS) following neoadjuvant therapy for stage IIIA N2 non-small cell lung cancer (NSCLC). Methods: From November 2009 to December 2013, a total of 35 consecutive patients with pathologically or radiologically confirmed stage IIIA N2 lung cancer underwent pulmonary resection and MND, performed by a single surgeon, following neoadjuvant chemoradiation...
February 2018: Korean Journal of Thoracic and Cardiovascular Surgery
Geak Poh Tan, Nigel McArdle, Satvinder Singh Dhaliwal, Jane Douglas, Clare Siobhan Rea, Bhajan Singh
Home mechanical ventilation (HMV) is used in a wide range of disorders associated with chronic hypoventilation. We describe the patterns of use, survival and predictors of death in Western Australia. We identified 240 consecutive patients (60% male; mean age 58 years and body mass index 31 kg m-2) referred for HMV between 2005 and 2010. The patients were grouped into four categories: motor neurone disorders (MND; 39%), pulmonary disease (PULM; 25%, mainly chronic obstructive pulmonary disease), non-MND neuromuscular and chest wall disorders (NMCW; 21%) and the obesity hypoventilation syndrome (OHS; 15%)...
January 1, 2018: Chronic Respiratory Disease
Alfonso Zamudio-Rodríguez, Pablo F Belaunzaran-Zamudio, Juan Sierra-Madero, Jennifer Margarita Cuellar-Rodríguez, Brenda Crabtree-Ramírez, Juan Luis Alcalá-Zermeno, Hélène Amieva, José Alberto Avila-Funes
INTRODUCTION: The population of aging adults living with Human Immunodeficiency Virus (HIV) is growing worldwide and evidence suggests that frailty occurs prematurely among them. In turn, frailty has been associated with cognitive decline. It is unknown, however, if people with both frailty and HIV-infection have higher risk of cognitive impairment compared with non-frail HIV-infected persons. Therefore, the main objective of this study was to determine the association between the phenotype of frailty and HIV-associated neurocognitive disorders (HAND) among adults aged 50 years or older living with HIV/AIDS...
February 1, 2018: AIDS Research and Human Retroviruses
Maize C Cao, Andrew Chancellor, Alison Charleston, Mike Dragunow, Emma L Scotter
BACKGROUND: We determined the mortality rates of motor neuron disease (MND) in New Zealand over 22 years from 1992 to 2013. Previous studies have found an unusually high and/or increasing incidence of MND in certain regions of New Zealand; however, no studies have examined MND rates nationwide to corroborate this. METHODS: Death certificate data coded G12.2 by International Classification of Diseases (ICD)-10 coding, or 335.2 by ICD-9 coding were obtained. These codes specify amyotrophic lateral sclerosis, progressive bulbar palsy, or other motor neuron diseases as the underlying cause of death...
January 30, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Miriam Galvin, Sile Carney, Bernie Corr, Iain Mays, Niall Pender, Orla Hardiman
OBJECTIVES: Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is a debilitating terminal condition. Informal caregivers are key figures in ALS care provision. The physical, psychological and emotional impact of providing care in the home requires appropriate assistance and support. The objective of this analysis is to explore the needs of informal ALS caregivers across the caregiving course. DESIGN: In an open-ended question as part of a semistructured interview, caregivers were asked what would help them in their role...
January 27, 2018: BMJ Open
Rachel L Fazio, Robert L Denney
Objective: To compare the efficacy of the Validity Indicator Profile (VIP) and Word Memory Test (WMT) in a criminal forensic population. Method: Potential participants included 225 male evaluees from a maximum-security Federal prison referred for neuropsychological evaluation for either forensic purposes or for suspected neurocognitive dysfunction as part of a medical evaluation. Examinees were included in the analysis if administered the VIP (Verbal, Nonverbal, or both tests) and WMT along with at least two other freestanding PVTs; 74 satisfied these criteria...
January 24, 2018: Archives of Clinical Neuropsychology: the Official Journal of the National Academy of Neuropsychologists
Margaret O'Connor, Samar M Aoun, Lauren J Breen
While the experiences of family members supporting a person with a terminal illness are well documented, less is known about the needs of carers of people with neurological diseases, in particular, Motor Neurone Disease (MND). This paper describes the qualitative data from a large Australian survey of family carers of people with MND, to ascertain their experiences of receiving the diagnosis. The aim of the study was to describe the experiences of family carers of people with MND in receiving the diagnosis in order to inform and improve ways in which the diagnosis is communicated...
January 22, 2018: Health & Social Care in the Community
David Brenner, Rüstem Yilmaz, Kathrin Müller, Torsten Grehl, Susanne Petri, Thomas Meyer, Julian Grosskreutz, Patrick Weydt, Wolfgang Ruf, Christoph Neuwirth, Markus Weber, Susana Pinto, Kristl G Claeys, Berthold Schrank, Berit Jordan, Antje Knehr, Kornelia Günther, Annemarie Hübers, Daniel Zeller, Christian Kubisch, Sibylle Jablonka, Michael Sendtner, Thomas Klopstock, Mamede de Carvalho, Anne Sperfeld, Guntram Borck, Alexander E Volk, Johannes Dorst, Joachim Weis, Markus Otto, Joachim Schuster, Kelly Del Tredici, Heiko Braak, Karin M Danzer, Axel Freischmidt, Thomas Meitinger, Tim M Strom, Albert C Ludolph, Peter M Andersen, Jochen H Weishaupt
Heterozygous missense mutations in the N-terminal motor or coiled-coil domains of the kinesin family member 5A (KIF5A) gene cause monogenic spastic paraplegia (HSP10) and Charcot-Marie-Tooth disease type 2 (CMT2). Moreover, heterozygous de novo frame-shift mutations in the C-terminal domain of KIF5A are associated with neonatal intractable myoclonus, a neurodevelopmental syndrome. These findings, together with the observation that many of the disease genes associated with amyotrophic lateral sclerosis disrupt cytoskeletal function and intracellular transport, led us to hypothesize that mutations in KIF5A are also a cause of amyotrophic lateral sclerosis...
January 12, 2018: Brain: a Journal of Neurology
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