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Steffen T Simon, Irene J Higginson, Sara Booth, Richard Harding, Vera Weingärtner, Claudia Bausewein
BACKGROUND: This is an updated version of the original Cochrane review published in Issue 1, 2010, on 'Benzodiazepines for the relief of breathlessness in advanced malignant and non-malignant diseases in adults'. Breathlessness is one of the most common symptoms experienced in the advanced stages of malignant and non-malignant disease. Benzodiazepines are widely used for the relief of breathlessness in advanced diseases and are regularly recommended in the literature. At the time of the previously published Cochrane review, there was no evidence for a beneficial effect of benzodiazepines for the relief of breathlessness in people with advanced cancer and chronic obstructive pulmonary disease (COPD)...
October 20, 2016: Cochrane Database of Systematic Reviews
Nazem Atassi, Ettore Beghi, Miguel Blanquer, Nicholas M Boulis, Roberto Cantello, Claudia Caponnetto, Adriano Chiò, Stephen B Dunnett, Eva L Feldman, Angelo Vescovi, Letizia Mazzini
Intraspinal stem cell (SC) transplantation represents a new therapeutic approach for amyotrophic lateral sclerosis (ALS) clinical trials. There are considerable difficulties in designing future efficacy trials, some related to the field of ALS and some that are specific to SCs or the mode of delivery. In October 2015, the most controversial points on SC transplantation were addressed during an international workshop intended to bring together international SC and ALS researchers in a public discussion on a topic for which expertise is limited...
October 6, 2016: Cytotherapy
Massimiliano Godani, Marco Zoccarato, Alessandro Beronio, Luigi Zuliani, Luana Benedetti, Bruno Giometto, Massimo Del Sette, Elisa Raggio, Roberta Baldi, Angela Vincent
BACKGROUND: The spectrum of autoimmune neurological diseases associated with voltage-gated potassium channel (VGKC)-complex antibodies (Abs) ranges from peripheral nerve disorders to limbic encephalitis. Recently, low titers of VGKC-complex Abs have also been reported in neurodegenerative disorders, but their clinical relevance is unknown. OBJECTIVE: The aim of the study was to explore the prevalence of VGKC-complex Abs in slow-progression motor neuron disease (MND)...
October 7, 2016: Neuro-degenerative Diseases
Jacob I Ayers, Jeffrey Diamond, Adriana Sari, Susan Fromholt, Ahmad Galaleldeen, Lyle W Ostrow, Jonathan D Glass, P John Hart, David R Borchelt
Evidence of misfolded wild-type superoxide dismutase 1 (SOD1) has been detected in spinal cords of sporadic ALS (sALS) patients, suggesting an etiological relationship to SOD1-associated familial ALS (fALS). Given that there are currently a number of promising therapies under development that target SOD1, it is of critical importance to better understand the role of misfolded SOD1 in sALS. We previously demonstrated the permissiveness of the G85R-SOD1:YFP mouse model for MND induction following injection with tissue homogenates from paralyzed transgenic mice expressing SOD1 mutations...
October 4, 2016: Acta Neuropathologica
Paul Talman, Thi Duong, Steve Vucic, Susan Mathers, Svetha Venkatesh, Robert Henderson, Dominic Rowe, David Schultz, Robert Edis, Merrilee Needham, Richard Macdonnell, Pamela McCombe, Carol Birks, Matthew Kiernan
OBJECTIVE: To capture the clinical patterns, timing of key milestones and survival of patients presenting with amyotrophic lateral sclerosis/motor neuron disease (ALS/MND) within Australia. METHODS: Data were prospectively collected and were timed to normal clinical assessments. An initial registration clinical report form (CRF) and subsequent ongoing assessment CRFs were submitted with a completion CRF at the time of death. DESIGN: Prospective observational cohort study...
September 30, 2016: BMJ Open
Gérard Manière, Anna B Ziegler, Flore Geillon, David E Featherstone, Yael Grosjean
Dietary leucine has been suspected to play an important role in insulin release, a hormone that controls satiety and metabolism. The mechanism by which insulin-producing cells (IPCs) sense leucine and regulate insulin secretion is still poorly understood. In Drosophila, insulin-like peptides (DILP2 and DILP5) are produced by brain IPCs and are released in the hemolymph after leucine ingestion. Using Ca(2+)-imaging and ex vivo cultured larval brains, we demonstrate that IPCs can directly sense extracellular leucine levels via minidiscs (MND), a leucine transporter...
September 27, 2016: Cell Reports
Stavroula Tsitkanou, Paul A Della Gatta, Aaron P Russell
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is a fatal motor neuron disorder. It results in progressive degeneration and death of upper and lower motor neurons, protein aggregation, severe muscle atrophy and respiratory insufficiency. Median survival with ALS is between 2 and 5 years from the onset of symptoms. ALS manifests as either familial ALS (FALS) (~10% of cases) or sporadic ALS (SALS), (~90% of cases). Mutations in the copper/zinc (CuZn) superoxide dismutase (SOD1) gene account for ~20% of FALS cases and the mutant SOD1 mouse model has been used extensively to help understand the ALS pathology...
2016: Frontiers in Physiology
Leah T Le, Serena S Spudich
Since the advent of combination antiretroviral therapy (cART), HIV has transformed from a fatal disease to a chronic illness that often presents with milder central nervous system (CNS) symptoms laced with related confounders. The immune recovery associated with access to cART has led to a new spectrum of immune-mediated presentations of infection, phenotypically distinct from the conditions observed in advanced disease.HIV-associated neurocognitive disorder (HAND) entails a categorized continuum of disorders reflecting an array of clinical presentation, outcome, and increasing level of severity: asymptomatic neurocognitive impairment (ANI), mild neurocognitive disorder (MND), and HIV-associated dementia (HAD)...
August 2016: Seminars in Neurology
Roger Pamphlett, Stephen Kum Jew
Toxic heavy metals have been implicated in the loss of spinal motoneurons in amyotrophic lateral sclerosis/motor neuron disease (ALS/MND). Motoneuron loss in the spinal anterior horn is severe in ALS/MND at the time of death, making this tissue unsuitable for examination. We therefore examined spinal cords of people without muscle weakness to look for any presence of heavy metals that could make these neurons susceptible to damage. Spinal cord samples from 50 individuals aged 1-95 y who had no clinical or histopathological evidence of spinal motoneuron loss were studied...
2016: PloS One
Phillip Smethurst, Jia Newcombe, Claire Troakes, Roberto Simone, Yun-Ru Chen, Rickie Patani, Katie Sidle
Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease (MND), and >95% of familial and sporadic cases involve the deposition of insoluble aggregated, phosphorylated and cleaved TDP-43 protein. Accumulating clinical and biological evidence now indicates that ALS bears a number of similarities to the prion diseases, with TDP-43 acting as a misfolded 'prion-like' protein demonstrating similar underlying pathobiology. Here we systematically address the hypothesis that ALS is a prion-like disorder...
August 30, 2016: Neurobiology of Disease
Benjamin Gaastra, Aleksey Shatunov, Sara Pulit, Ashley R Jones, William Sproviero, Alexandra Gillett, Zhongbo Chen, Janine Kirby, Isabella Fogh, John F Powell, P Nigel Leigh, Karen E Morrison, Pamela J Shaw, Christopher E Shaw, Leonard H van den Berg, Jan H Veldink, Cathryn M Lewis, Ammar Al-Chalabi
Our objective was to identify whether rare genetic variation in amyotrophic lateral sclerosis (ALS) candidate survival genes modifies ALS survival. Candidate genes were selected based on evidence for modifying ALS survival. Each tail of the extreme 1.5% of survival was selected from the UK MND DNA Bank and all samples available underwent whole genome sequencing. A replication set from the Netherlands was used for validation. Sequences of candidate survival genes were extracted and variants passing quality control with a minor allele frequency ≤0...
September 1, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
R A Armstrong
Factors associated with survival were studied in 84 neuropathologically documented cases of the pre-senile dementia frontotemporal dementia lobar degeneration (FTLD) with transactive response (TAR) DNA-binding protein of 43 kDa (TDP-43) proteinopathy (FTLD-TDP). Kaplan-Meier survival analysis estimated mean survival as 7.9 years (range: 1-19 years, SD = 4.64). Familial and sporadic cases exhibited similar survival, including progranulin (GRN) gene mutation cases. No significant differences in survival were associated with sex, disease onset, Braak disease stage, or disease subtype, but higher survival was associated with lower post-mortem brain weight...
2016: Folia Neuropathologica
Patricia A M van Iersel, Saskia C M Bakker, Arnold J H Jonker, Mijna Hadders-Algra
AIM: To evaluate in term infants associations between quality of general movements and developmental outcome in term infants at 6 years with either cerebral palsy (CP) or limited mobility without CP. METHOD: Participants of this prospective study were 145 term infants (86 male, 59 female). Their general movements quality was assessed at 'writhing' and 'fidgety' general movements age (3wks and 13wks post term). The assessment at 6 years consisted of a neurological examination, including assessment of minor neurological dysfunction (MND), evaluation of mobility with the Movement Assessment Battery for Children, and of behaviour and learning problems with questionnaires...
August 13, 2016: Developmental Medicine and Child Neurology
Tinka Kurpershoek, Eva S Potharst-Sirag, Cornelieke S H Aarnoudse-Moens, Aleid G van Wassenaer-Leemhuis
BACKGROUND: Minor neurological dysfunction (MND) is present in one quarter to one third of children born very preterm (VP). The more severe form, complex (c)-MND has been associated with learning disabilities, behavioural and motor problems. OBJECTIVE: To study the association between c-MND and neurocognitive and motor disabilities at age five in VP children without CP. METHODS: Ninety-four children born with gestational age<30weeks and/or a birth weight<1000g were assessed at five years corrected age...
August 8, 2016: Early Human Development
René Günther, Nicole Richter, Anna Sauerbier, Kallol Ray Chaudhuri, Pablo Martinez-Martin, Alexander Storch, Andreas Hermann
BACKGROUND: The recently postulated "disease spreading hypothesis" has gained much attention, especially for Parkinson's disease (PD). The various non-motor symptoms (NMS) in neurodegenerative diseases would be much better explained by this hypothesis than by the degeneration of disease-specific cell populations. Motor neuron disease (MND) is primarily known as a group of diseases with a selective loss of motor function. However, recent evidence suggests disease spreading into non-motor brain regions also in MND...
2016: Frontiers in Neurology
Swati Tiwari, Adrianne Hontz, Catherine Terrell, Paritha Arumugam, Marlene Carmo, Kimberly Risma, Michael Jordan, Punam Malik
Perforin-1 mutations result in a potentially fatal hemophagocytic lymphohistiocytosis (HLH) with heightened immune activation, hypercytokinemia, pancytopenia and end-organ damage. Currently, hematopoietic stem cell (HSC) transplant is curative, but limited by donor availability and associated mortality, making gene therapy an attractive alternative approach for HLH. We recently reported that perforin expression driven by cellular promoters in lentiviral vectors (LV) resulted in significant, albeit partial, correction of the inflammatory features in a murine model of HLH...
July 29, 2016: Human Gene Therapy
Paola Cerutti, Valentina Solara, Silvia Rossi Ferrario
The approach to patients affected by motor neuron disease (MND) and their caregivers requires specific training for the care-team. In fact, the progression of the disease, with the decline of physical--and sometimes cognitive--function, the increasing difficulties in speaking, breathing, and swallowing and the need of invasive choices, as the artificial nutrition and tracheostomy, constitute a challenge for the health professionals, often generating distress. For this reason, their cohesion and sharing abilities are fundamental...
April 2016: Giornale Italiano di Medicina del Lavoro Ed Ergonomia
Rachel L Fazio, John H Denning, Robert L Denney
OBJECTIVE: To determine the effectiveness of the Test of Memory Malingering Trial 1 (TOMM1) as a freestanding Performance Validity Test (PVT) as compared to the full TOMM in a criminal forensic sample. METHOD: Participants included 119 evaluees in a Midwestern forensic hospital. Criterion groups were formed based on passing/failing scores on other freestanding PVTs. This resulted in three groups: +MND (Malingered Neurocognitive Dysfunction), who failed two or more freestanding PVTs; possible MND (pMND), who failed one freestanding PVT; and -MND, who failed no other freestanding PVTs...
July 26, 2016: Clinical Neuropsychologist
Thanuja Dharmadasa, José M Matamala, Matthew C Kiernan
PURPOSE OF REVIEW: Although there is no cure for motor neurone disease (MND), the advent of multidisciplinary care and neuroprotective agents has improved treatment interventions and enhanced quality of life for MND patients and their carers. RECENT FINDINGS: Evidence-based multidisciplinary care, respiratory management and disease-modifying therapy have improved the outcomes of patients diagnosed with MND. Supportive approaches to nutritional maintenance and optimization of symptomatic treatments, including management of communication and neuropsychiatric issues, improve the quality of life for MND patients...
October 2016: Current Opinion in Neurology
Bronwyn Hemsley, Stuart Palmer
To date, there is no research examining how adults with Amyotrophic Lateral Sclerosis (ALS) or Motor Neurone Disease (MND) and severe communication disability use Twitter, nor the use of Twitter in relation to ALS/MND beyond its use for fundraising and raising awareness. In this paper we (a) outline a rationale for the use of Twitter as a method of communication and information exchange for adults with ALS/MND, (b) detail multiple qualitative and quantitative methods used to analyse Twitter networks and tweet content in the our studies, and (c) present the results of two studies designed to provide insights on the use of Twitter by an adult with ALS/MND and by #ALS and #MND hashtag communities in Twitter...
2016: Studies in Health Technology and Informatics
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