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https://www.readbyqxmd.com/read/29150977/the-cellular-composition-and-glia-neuron-ratio-in-the-spinal-cord-of-a-human-and-a-non-human-primate-comparison-with-other-species-and-brain-regions
#1
Jami Bahney, Christopher S von Bartheld
The cellular composition of brains shows largely conserved, gradual evolutionary trends between species. In the primate spinal cord, however, the glia-neuron ratio was reported to be greatly increased over that in the rodent spinal cord. Here, we re-examined the cellular composition of the spinal cord of one human and one non-human primate species by employing two different counting methods, the isotropic fractionator and stereology. We also determined whether segmental differences in cellular composition, possibly reflecting increased fine motor control of the upper extremities, may explain a sharply increased glia-neuron ratio in primates...
November 18, 2017: Anatomical Record: Advances in Integrative Anatomy and Evolutionary Biology
https://www.readbyqxmd.com/read/29148035/late-age-onset-of-amyotrophic-lateral-sclerosis-is-often-not-considered-in-elderly-people
#2
E Broussalis, S Grinzinger, A B Kunz, M Killer-Oberpfalzer, E Haschke-Becher, H-P Hartung, J Kraus
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease causing an upper and lower motor neuron loss. It is neurology textbook knowledge that the mean age of onset is about 60 years. However, recent investigations show an increasing incidence in older persons. We therefore evaluated whether ALS is potentially not considered in elderly people with ALS symptoms, respectively, not recognized. MATERIALS AND METHODS: We included retrospectively all patients with ALS diagnoses after work-up that were admitted to our neurological and geriatric departments from 2007 to 2010 and collected their clinical data...
November 17, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/29146456/is-more-always-better-how-different-doses-of-exercise-after-incomplete-spinal-cord-injury-affects-the-membrane-properties-of-deep-dorsal-horn-interneurons
#3
M M Rank, M P Galea, R Callister, R J Callister
Interneurons in the deep dorsal horn (DDH) of the spinal cord process somatosensory input, and form an important link between upper and lower motoneurons to subsequently shape motor output. Exercise training after SCI is known to improve functional motor recovery, but little is known about the mechanisms within spinal cord neurons that underlie these improvements. Here we investigate how the properties of DDH interneurons are affected by spinal cord injury (SCI) alone, and SCI in combination with different 'doses' of treadmill exercise training (3, 6, and 9wks)...
November 13, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/29138753/lesion-pattern-mechanisms-and-long-term-prognosis-in-patients-with-monoparetic-stroke-a-comparison-with-nonmonoparetic-stroke
#4
Seung-Jae Lee, Dong-Geun Lee, Hye-Jin Moon, Tae-Kyeong Lee
Background: Monoparetic stroke is rare but could be misdiagnosed as peripheral neuropathy. We investigated the prevalence, lesion pattern, stroke mechanism, and long-term prognosis in patients with monoparetic stroke. Methods: 586 acute ischemic stroke patients (including 31 with monoparesis) were studied. Monoparetic stroke was defined as a motor deficit in either an arm or a leg but without facial weakness or speech disturbance. Median follow-up period was 32...
2017: BioMed Research International
https://www.readbyqxmd.com/read/29137918/spinobulbar-muscular-atrophy-combined-with-atypical-hereditary-neuropathy-with-liability-to-pressure-palsy
#5
Kyomin Choi, So Hyun Ahn, Seol-Hee Baek, Jun-Soon Kim, Seok-Jin Choi, Je-Young Shin, Sung-Min Kim, Yoon-Ho Hong, Jung-Joon Sung
Spinobulbar muscular atrophy (SBMA) is an X-linked recessive disease, presenting motor weakness and wasting of facial, bulbar and limb muscles. Hereditary neuropathy with liability to pressure palsy (HNPP) is autosomal dominant disorder characterized by recurrent neuropathies at common entrapment sites. We report a case of co-existence of SBMA and atypical HNPP with genetic confirmation of CAG expansion in the androgen receptor (AR) gene and deletion of the peripheral myelin protein 22 (PMP22) gene. A 62-year-old man presented with progressive muscle weakness, fasciculations in upper and lower limbs and dysesthesia predominantly in the distal regions...
November 11, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29134445/ptosis-and-bulbar-onset-an-unusual-phenotype-of-familial-als
#6
Fabiola De Marchi, L Corrado, E Bersano, M F Sarnelli, V Solara, S D'Alfonso, R Cantello, L Mazzini
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of upper and lower motor neurons that usually spare the oculomotor nerves. Here, we describe a case of two siblings with a familial bulbar-onset ALS both with ptosis manifested at the onset of the disease.
November 13, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/29128155/motor-neuron-disease-in-inherited-neurometabolic-disorders
#7
REVIEW
P Victor Sgobbi de Souza, T Bortholin, F George Monteiro Naylor, M Antônio Troccoli Chieia, W Bocca Vieira de Rezende Pinto, A Souza Bulle Oliveira
Inherited neurometabolic disorders represent a growing group of inborn errors of metabolism that present with major neurological symptoms or a complex spectrum of symptoms dominated by central or peripheral nervous system dysfunction. Many neurological presentations may arise from the same metabolic defect, especially in autosomal-recessive inherited disorders. Motor neuron disease (MND), mainly represented by amyotrophic lateral sclerosis, may also result from various inborn errors of metabolism, some of which may represent potentially treatable conditions, thereby emphasizing the importance of recognizing such diseases...
November 8, 2017: Revue Neurologique
https://www.readbyqxmd.com/read/29124833/a-nonstop-variant-in-reep1-causes-peripheral-neuropathy-by-unmasking-a-3-utr-encoded-aggregation-inducing-motif
#8
Andrea S Bock, Sven Günther, Julia Mohr, Lisa V Goldberg, Amir Jahic, Cornelia Klisch, Christian A Hübner, Saskia Biskup, Christian Beetz
Single nucleotide variants that abolish the stop codon ('nonstop' alterations) are a unique type of substitution in genomic DNA. Whether they confer instability of the mutant mRNA or result in expression of a C-terminally extended protein depends on the absence or presence of a downstream in-frame stop codon, respectively. Of the predicted protein extensions, only few have been functionally characterized. In a family with autosomal dominant Charcot-Marie-Tooth disease type 2, i.e. an axonopathy affecting sensory neurons as well as lower motor neurons, we identified a heterozygous nonstop variant in REEP1...
November 9, 2017: Human Mutation
https://www.readbyqxmd.com/read/29123918/whole-genome-sequencing-identifies-a-novel-homozygous-exon-deletion-in-the-nt5c2-gene-in-a-family-with-intellectual-disability-and-spastic-paraplegia
#9
Hossein Darvish, Luis J Azcona, Abbas Tafakhori, Mona Ahmadi, Azadeh Ahmadifard, Coro Paisán-Ruiz
Hereditary spastic paraplegias are a rare group of clinically and genetically heterogeneous neurodegenerative diseases, with upper motor neuron degeneration and progressive lower limb spasticity as their main phenotypic features. Despite that 76 distinct loci have been reported and some casual genes identified, most of the underlying causes still remain unidentified. Moreover, a wide range of clinical manifestations is present in most hereditary spastic paraplegias subtypes, adding further complexity to their differential clinical diagnoses...
2017: NPJ Genomic Medicine
https://www.readbyqxmd.com/read/29122765/semiautomated-evaluation-of-the-primary-motor-cortex-in-patients-with-amyotrophic-lateral-sclerosis-at-3t
#10
G Donatelli, A Retico, E Caldarazzo Ienco, P Cecchi, M Costagli, D Frosini, L Biagi, M Tosetti, G Siciliano, M Cosottini
BACKGROUND AND PURPOSE: Amyotrophic lateral sclerosis is a neurodegenerative disease involving the upper and lower motor neurons. In amyotrophic lateral sclerosis, pathologic changes in the primary motor cortex include Betz cell depletion and the presence of reactive iron-loaded microglia, detectable on 7T MR images as atrophy and T2*-hypointensity. Our purposes were the following: 1) to investigate the signal hypointensity-to-thickness ratio of the primary motor cortex as a radiologic marker of upper motor neuron involvement in amyotrophic lateral sclerosis with a semiautomated method at 3T, 2) to compare 3T and 7T results, and 3) to evaluate whether semiautomated measurement outperforms visual image assessment...
November 9, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29114369/bimelic-symmetric-hirayama-disease-spectrum-of-magnetic-resonance-imaging-findings-and-comparative-evaluation-with-classical-monomelic-amyotrophy-and-other-motor-neuron-disease
#11
Deb Kumar Boruah, Shantiranjan Sanyal, Arjun Prakash, Sashidhar Achar, Dhabal D Dhingani, Binod Sarma
Background: The aim of the study was to evaluate the magnetic resonance imaging (MRI) findings in bilateral symmetrical Hirayama disease and find out MRI features which are probably more indicative of symmetrical Hirayama disease, thereby help in differentiating this entity from other motor neuron disease (MND). Methods: This prospective as well as retrospective study was carried out from December 2010 to September 2016 in a tertiary care center of northeast India on 92 patients with Hirayama disease. Only 19 patients having bilateral symmetric upper limb involvement at the time of presentation were included in this study sample...
July 6, 2017: Iranian Journal of Neurology
https://www.readbyqxmd.com/read/29096208/changes-in-the-soleus-h-reflex-test-and-correlations-between-its-results-and-dynamic-magnetic-resonance-imaging-abnormalities-in-patients-with-hirayama-disease
#12
Chaojun Zheng, Yu Zhu, Feizhou Lu, Dongqing Zhu, Shuo Yang, Xiaosheng Ma, Xinlei Xia, Robert Weber, Jianyuan Jiang
OBJECTIVE: To investigate changes in soleus H-reflex tests in patients with Hirayama disease (HD) and to analyse correlations between these changes and forward-shifting of the cervical cord during neck flexion. METHODS: The amplitude of the soleus H-reflex with and without vibration on the Achilles tendon was recorded bilaterally in 81 HD patients and 34 controls to measure both the vibratory inhibition index (VII) and the Hmax/Mmax ratio. The maximum forward-shifting degree of cervical cord during neck flexion was measured using dynamic magnetic resonance imaging in all HD patients...
October 3, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29081604/what-is-hyper-in-the-als-hypermetabolism
#13
REVIEW
Alberto Ferri, Roberto Coccurello
The progressive and fatal loss of upper (brain) and lower (spinal cord) motor neurons and muscle denervation concisely condenses the clinical picture of amyotrophic lateral sclerosis (ALS). Despite the multiple mechanisms believed to underlie the selective loss of motor neurons, ALS aetiology remains elusive and obscure. Likewise, there is also a cluster of alterations in ALS patients in which muscle wasting, body weight loss, eating dysfunction, and abnormal energy dissipation coexist. Defective energy metabolism characterizes the ALS progression, and such paradox of energy balance stands as a challenge for the understanding of ALS pathogenesis...
2017: Mediators of Inflammation
https://www.readbyqxmd.com/read/29076391/management-of-stress-urinary-incontinence-in-spinal-cord-injured-female-patients-with-a-mid-urethral-tape-a-single-center-experience
#14
Vasileios I Sakalis, Michael S Floyd, Philippa Caygill, Chloe Price, Ben Hartwell, Peter J Guy, Melissa C Davies
CONTEXT/OBJECTIVE: Stress urinary incontinence (SUI) affects the quality of life of females with spinal cord injury (SCI), has a negative impact on functional independence and disturbs their psychosocial interaction. Our aim was to assess the efficacy of mid-urethral tapes (MUT) in managing stress urinary incontinence in this population. DESIGN: Retrospective cohort study. PARTICIPANTS: SCI females with upper motor neuron lesion and urodynamically proven stress or mixed urinary incontinence that was treated with a mid-urethral tape and followed up for at least 12 months...
October 27, 2017: Journal of Spinal Cord Medicine
https://www.readbyqxmd.com/read/29071208/fast-progressive-lower-motor-neuron-disease-is-an-als-variant-a-two-centre-tract-of-interest-based-mri-data-analysis
#15
Hans-Peter Müller, Federica Agosta, Nilo Riva, Edoardo G Spinelli, Giancarlo Comi, Albert C Ludolph, Massimo Filippi, Jan Kassubek
BACKGROUND: The criteria for assessing upper motor neuron pathology in pure lower motor neuron disease (LMND) still remain a major issue of debate with respect to the clinical classification as an amyotrophic lateral sclerosis (ALS) variant. OBJECTIVE: The study was designed to investigate white matter damage by a hypothesis-guided tract-of-interest-based approach in patients with LMND compared with healthy controls and ´classical´ ALS patients in order to identify in vivo brain structural changes according to the neuropathologically defined ALS affectation pattern...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29059659/-post-stroke-spasticity-management-including-a-chosen-physiotherapeutic-methods-and-improvements-in-motor-control-review-of-the-current-scientific-evidence
#16
Robert Dymarek, Kuba Ptaszkowski, Lucyna Słupska, Małgorzata Paprocka-Borowicz, Jakub Taradaj, Tomasz Halski, Joanna Rosińczuk
Cerebrovascular diseases based on stroke etiology concern millions of people worldwide, and annual rates of disease are still increasing. In the era of an aging society and suffering from a number of risk factors, in particular those modifiable, strokes and muscles' spastic paresis, subsequently resulting in damage of upper motor neuron structures will become a serious problem for the entire health care system. Effective management and physiotherapy treatment for post-stroke spasticity persisted, both in the acute and chronic, is still a significant medical problem in the interdisciplinary aspect...
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29052789/computational-analysis-of-pharyngeal-swallowing-mechanics-in-patients-with-motor-neuron-disease-a-pilot-investigation
#17
K L Garand, Ryan Schwertner, Amy Chen, William G Pearson
Swallowing impairment (dysphagia) is a common sequela in patients with motor neuron disease (MND). The purpose of this retrospective, observational pilot investigation was to characterize how pharyngeal swallowing mechanics are impacted in patients with MND using a comparison with healthy, non-dysphagic control group. Computational analysis of swallowing mechanics (CASM) was used to determine covariate biomechanics of pharyngeal swallowing from videofluoroscopic assessment in 15 patients with MND and 15 age- and sex-matched healthy controls...
October 20, 2017: Dysphagia
https://www.readbyqxmd.com/read/29041999/electrodiagnostic-and-nerve-ultrasonographic-features-in-upper-limb-spasticity-an-observational-study
#18
A Picelli, S Tamburin, G Berto, E Chemello, Marialuisa Gandolfi, Leopold Saltuari, Andreas Waldner, Nicola Smania
To better understand the effects of spasticity on peripheral nerves, we evaluated the electrodiagnostic and nerve ultrasonographic features of the median and ulnar nerves in adults with upper limb spasticity. Twenty chronic stroke patients with spastic hemiparesis underwent nerve conduction study and nerve ultrasonography of the median and ulnar nerves at both upper limbs. Affected versus unaffected upper limb comparisons showed significant differences in the median and ulnar nerve distal motor latencies, compound muscle action potentials and F-wave minimal latencies...
July 2017: Functional Neurology
https://www.readbyqxmd.com/read/29037065/patterns-of-symptom-development-in-patients-with-motor-neuron-disease
#19
Renée Walhout, Esther Verstraete, Martijn P Van Den Heuvel, Jan H Veldink, Leonard H Van Den Berg
OBJECTIVE: To investigate whether symptom development in motor neuron disease (MND) is a random or organized process. METHODS: Six hundred patients with amyotrophic lateral sclerosis (ALS), upper motor neuron (UMN) or lower motor neuron (LMN) phenotypes were invited for a questionnaire concerning symptom development. A binomial test was used to examine distribution of symptoms from site of onset. Development of symptoms over time was evaluated by Kaplan-Meier analysis...
October 16, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29024655/lost-in-transportation-nucleocytoplasmic-transport-defects-in-als-and-other-neurodegenerative-diseases
#20
REVIEW
Hong Joo Kim, J Paul Taylor
Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurodegenerative disease characterized by degeneration of upper and lower motor neurons in the brain and spinal cord. The hallmark pathological feature in most cases of ALS is nuclear depletion and cytoplasmic accumulation of the protein TDP-43 in degenerating neurons. Consistent with this pattern of intracellular protein redistribution, impaired nucleocytoplasmic trafficking has emerged as a mechanism contributing to ALS pathology. Dysfunction in nucleocytoplasmic transport is also an emerging theme in physiological aging and other related neurodegenerative diseases, such as Huntington's and Alzheimer's diseases...
October 11, 2017: Neuron
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