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Trends in Research Literature Describing Dysphagia in Motor Neuron Diseases (MND): A Scoping Review

AbstractDysphagia in motor neuron diseases (MNDs) is highly complex, affecting all stages of swallowing and leading to impaired swallowing safety and efficiency. In order to explore the degree to which research is capturing the symptom of dysphagia in MND, we conducted a scoping review of the existing literature. The primary aims of this review were to identify common themes within the literature on dysphagia in MND, explore patterns and trends in research focus, and identify if any imbalances exist between the research themes related to dysphagia description and management. A comprehensive search strategy yielded 1690 unique articles for review. Following relevance screening, a total of 157 articles were included in the synthesis. Relevant data and keywords were extracted from each article and grouped into themes. Frequency estimates were calculated for each theme to identify trends across research literature. Swallowing impairment in MNDs is described in a variety of ways across current research. The most commonly reported theme wasAspiration/Penetration, mentioned in 73.2% of all included articles; a significant imbalance was identified between reports of swallowing safety and efficiency (p = 0.008). The most frequently reported theme related to dysphagia management wasEnteral Nutrition, and very few studies have reported on the efficacy ofRehabilitation/Compensatory recommendations. It is suggested that researchers and clinicians remain mindful of imbalances and...
Source: Dysphagia - Category: Speech-Language Pathology Source Type: research

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Authors: D'Cruz RF, Murphy PB, Kaltsakas G Abstract 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 uppe...
Source: Journal of Thoracic Disease - Category: Respiratory Medicine Tags: J Thorac Dis Source Type: research
The interface between work and health is complex and poses a variety of issues for health professionals. Health factors may be congenital or inherited (or acquired at an early age) or present later in life. They may recover fully, improve partially, leave fluctuating levels of impairment (e.g. multiple sclerosis or rheumatoid arthritis) or progress inexorably (e.g. motor neurone disease) [1]. The end result of these differing health pathways may lead to difficulties working —and the numbers are huge. Government estimates indicate that there are over 7 million people with disabilities of working age in the UK, of whom...
Source: Occupational Medicine - Category: Respiratory Medicine Source Type: research
Source: Muscle and Nerve - Category: Internal Medicine Authors: Tags: Editorial Source Type: research
We present a case of a cervical intramedullary schwannoma surgically treated in a 19-year-old male patient who initially presented with motor neuron disease.Case Rep Neurol 2018;10:18 –24
Source: Case Reports in Neurology - Category: Neurology Source Type: research
A family from Clydach, Wales, have lost five members of their family to motor neurone disease. However, Mandy Duguid is determined to aid in funding for research into the disease
Source: the Mail online | Health - Category: Consumer Health News Source Type: news
Discussion: Mean‐MUNIX and NI were more sensitive than clinical measures at detecting LMN loss in presymptomatic limbs from patients with slowly progressive ALS. Therefore, these electrophysiological biomarkers should be included in early study phases as meaningful outcome measures. Muscle Nerve, 2018
Source: Muscle and Nerve - Category: Internal Medicine Authors: Tags: Clinical Research Article Source Type: research
AbstractMutations in fused in sarcoma (FUS) cause amyotrophic lateral sclerosis (ALS). FUS is a multifunctional protein involved in the biogenesis and activity of several types of RNAs, and its role in the pathogenesis of ALS may involve both direct effects of disease-associated mutations through gain- and loss-of-function mechanisms and indirect effects due to the cross talk between different classes of FUS-dependent RNAs. To explore how FUS mutations impinge on motor neuron-specific RNA-based circuitries, we performed transcriptome profiling of small and long RNAs of motor neurons (MNs) derived from mouse embryonic stem ...
Source: Molecular Neurobiology - Category: Neurology Source Type: research
AbstractCo-culture models of neurons and Schwann cells have been utilized for the study of myelination and demyelination in the peripheral nervous system; in most of the previous studies, however, these cells were obtained by primary culture with embryonic or neonatal animals. A spontaneously immortalized Schwann cell line IFRS1 from long-term cultures of adult Fischer rat peripheral nerves has been shown to retain fundamental ability to myelinate neurites in co-cultures with adult rat dorsal root ganglion neurons and nerve growth factor-primed PC12 cells. Our current investigation focuses on the establishment of stable co...
Source: Histochemistry and Cell Biology - Category: Biomedical Science Source Type: research
Authors: Cortes CJ, La Spada AR Abstract Spinal and Bulbar Muscular Atrophy (SBMA) is an inherited neuromuscular disorder caused by a CAG-polyglutamine (polyQ) repeat expansion in the androgen receptor (AR) gene. Unlike other polyQ diseases, where the function of the native causative protein is unknown, the biology of AR is well understood, and this knowledge has informed our understanding of how native AR function interfaces with polyQ-AR dysfunction. Furthermore, ligand-dependent activation of AR has been linked to SBMA disease pathogenesis, and has led to a thorough study of androgen-mediated effects on polyQ-AR...
Source: Advances in Experimental Medicine and Biology - Category: Research Tags: Adv Exp Med Biol Source Type: research
Abstract The BCR consists of the contraction of the bulbocavernosus muscle in response to squeezing the glans penis or clitoris, and is mediated through the pudendal nerve. In case of a complete lesion, the presence of BCR is indicative of intact S2-S4 spinal reflex arcs and loss of supraspinal inhibition, determining an upper motor neuron (UMN) lesion, its absence a lower motor neuron (LMN) lesion. The BCR further helps distinguish conus medullaris from cauda equina syndromes. Sensory or motor function in the most caudal sacral segments, not BCR, defines the sacral sparing as part of the International Standards f...
Source: Anal Sci - Category: Chemistry Authors: Tags: Spinal Cord Ser Cases Source Type: research
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