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Upper motor neuron

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https://www.readbyqxmd.com/read/27929803/an-autopsy-verified-case-of-ftld-tdp-type-a-with-upper-motor-neuron-predominant-motor-neuron-disease-mimicking-mm2-thalamic-type-sporadic-creutzfeldt-jakob-disease
#1
Yuichi Hayashi, Yasushi Iwasaki, Akira Takekoshi, Nobuaki Yoshikura, Takahiko Asano, Maya Mimuro, Akio Kimura, Katsuya Satoh, Tetsuyuki Kitamoto, Mari Yoshida, Takashi Inuzuka
Here we report an autopsy-verified case of frontotemporal lobar degeneration (FTLD)-transactivation responsive region (TAR) DNA binding protein (TDP) type A with upper motor neuron-predominant motor neuron disease mimicking MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD). A 69-year-old woman presented with an 11-month history of progressive dementia, irritability, insomnia, and gait disturbance without a family history of dementia or prion disease. Neurological examination revealed severe dementia, frontal signs, and exaggerated bilateral tendon reflexes...
November 2016: Prion
https://www.readbyqxmd.com/read/27925241/vertebral-subluxation-repair-in-a-pet-goat
#2
Sara Nannarone, Enrico Bellezza, Yves P Moens, Paula Larenza Menzies
OBJECTIVES: To describe the perioperative management, including surgery, anesthesia, metabolic derangements, and physiotherapy, in a goat referred for paraparesis secondary to a road traffic accident. STUDY DESIGN: Case report. ANIMALS: 2-year-old mixed breed dwarf 44 kg female pet goat. METHODS: Clinical examination showed symptoms of early compensatory stages of shock, paraparesis with hyperextension of the thoracic limbs, pain on palpation of the thoracolumbar spine, increased patellar reflexes of both pelvic limbs without superficial sensitivity, but preserved deep pain sensation...
December 7, 2016: Veterinary Surgery: VS
https://www.readbyqxmd.com/read/27924954/eeg-functional-network-topology-is-associated-with-disability-in-patients-with-amyotrophic-lateral-sclerosis
#3
Matteo Fraschini, Matteo Demuru, Arjan Hillebrand, Lorenza Cuccu, Silvia Porcu, Francesca Di Stefano, Monica Puligheddu, Gianluca Floris, Giuseppe Borghero, Francesco Marrosu
Amyotrophic Lateral Sclerosis (ALS) is one of the most severe neurodegenerative diseases, which is known to affect upper and lower motor neurons. In contrast to the classical tenet that ALS represents the outcome of extensive and progressive impairment of a fixed set of motor connections, recent neuroimaging findings suggest that the disease spreads along vast non-motor connections. Here, we hypothesised that functional network topology is perturbed in ALS, and that this reorganization is associated with disability...
December 7, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27919237/a-novel-ano3-variant-identified-in-a-53-year-old-woman-presenting-with-hyperkinetic-dysarthria-blepharospasm-hyperkinesias-and-complex-motor%C3%A2-tics
#4
Patrick R Blackburn, Michael T Zimmermann, Jennifer M Gass, Kimberly G Harris, Margot A Cousin, Nicole J Boczek, Owen A Ross, Eric W Klee, Paul W Brazis, Jay A Van Gerpen, Paldeep S Atwal
BACKGROUND: Cervical dystonias have a variable presentation and underlying etiology, but collectively represent the most common form of focal dystonia. There are a number of known genetic forms of dystonia (DYT1-27); however the heterogeneity of disease presentation does not always make it easy to categorize the disease by phenotype-genotype comparison. CASE PRESENTATION: In this report, we describe a 53-year-old female who presented initially with hand tremor following a total hip arthroplasty...
December 5, 2016: BMC Medical Genetics
https://www.readbyqxmd.com/read/27909242/beneficial-effects-of-rapamycin-in-a-drosophila-model-for-hereditary-spastic-paraplegia
#5
Shiyu Xu, Michael Stern, James A McNew
The locomotor deficits in the hereditary spastic paraplegias (HSPs) reflect degeneration of upper motor neurons, but the mechanisms underlying this neurodegeneration are unknown. We established a Drosophila model for the HSP atlastin (atl), which encodes an ER fusion protein. Here we show that neuronal atl loss causes degeneration of specific thoracic muscles that is preceded by other pathologies including accumulation of aggregates containing poly-ubiquitin (poly-UB), increased generation of reactive oxygen species, and activation of the JNK/Foxo stress response pathway...
December 1, 2016: Journal of Cell Science
https://www.readbyqxmd.com/read/27907012/the-signature-of-microrna-dysregulation-in-muscle-paralyzed-by-spinal-cord-injury-includes-downregulation-of-micrornas-that-target-myostatin-signaling
#6
Rita De Gasperi, Zachary A Graham, Lauren M Harlow, William A Bauman, Weiping Qin, Christopher P Cardozo
Spinal cord injury (SCI) results in muscle atrophy, reduced force generation and an oxidative-to-glycolytic fiber type shift. The mechanisms responsible for these alterations remain incompletely understood. To gain new insights regarding mechanisms involved in deterioration of muscle after SCI, global expression profiles of miRs in paralyzed gastrocnemius muscle were compared between sham-operated (Sham) and spinal cord-transected (SCI) rats. Ingenuity Pathways Analysis of the altered miRs identified signaling via insulin, IGF-1, integrins and TGF-β as being significantly enriched for target genes...
2016: PloS One
https://www.readbyqxmd.com/read/27904488/effects-of-different-frequencies-of-repetitive-transcranial-magnetic-stimulation-on-the-recovery-of-upper-limb-motor-dysfunction-in-patients-with-subacute-cerebral-infarction
#7
Jiang Li, Xiang-Min Meng, Ru-Yi Li, Ru Zhang, Zheng Zhang, Yi-Feng Du
Studies have confirmed that low-frequency repetitive transcranial magnetic stimulation can decrease the activity of cortical neurons, and high-frequency repetitive transcranial magnetic stimulation can increase the excitability of cortical neurons. However, there are few studies concerning the use of different frequencies of repetitive transcranial magnetic stimulation on the recovery of upper-limb motor function after cerebral infarction. We hypothesized that different frequencies of repetitive transcranial magnetic stimulation in patients with cerebral infarction would produce different effects on the recovery of upper-limb motor function...
October 2016: Neural Regeneration Research
https://www.readbyqxmd.com/read/27902929/necroptosis-in-amyotrophic-lateral-sclerosis-and-other-neurological-disorders
#8
REVIEW
Jessica R Morrice, Cheryl Y Gregory-Evans, Christopher A Shaw
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the progressive degeneration of upper and lower motor neurons. Cell death in ALS and in general was previously believed to exist as a dichotomy between apoptosis and necrosis. Most research investigating cell death mechanisms in ALS was conducted before the discovery of programmed necrosis thus did not use selective cell death pathway-specific markers. Recently, a new form of programmed cell death, termed "necroptosis", has been characterized and has been recently implicated in ALS as a primary mechanism driving motor neuron cell death in different forms of ALS...
November 27, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27892702/slowly-progressive-motor-neuron-disease-with-multi-system-involvement-related-to-p-e121g-sod1-mutation
#9
Guillaume Taieb, Anne Polge, Raul Juntas-Morales, Nicolas Pageot, Serge Lumbroso, Kevin Mouzat, William Camu
We report the third case of amyotrophic lateral sclerosis related to p.E121G Superoxide dismutase-1 (SOD1) mutation. Besides a sporadic presentation and a slow progressive course, as described in the 2 previously cases, our patient presented with prominent sensory and cerebellar signs. This case report strengthens that p.E121G should be considered as a causal mutation. Slowly upper and lower motor neuron degeneration, even with non-motor clinical features, should prompt a sequencing of SOD1.
November 28, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27871959/intracavernous-injections-in-spinal-cord-injured-men-with-erectile-dysfunction-a-systematic-review-and-meta-analysis
#10
REVIEW
Lucie Chochina, Florian Naudet, Clément Chéhensse, Andrea Manunta, Mireille Damphousse, Isabelle Bonan, François Giuliano
INTRODUCTION: Despite improvements in the care of patients after spinal cord injury (SCI), permanent impairment of locomotion, sensation, and autonomic function remains a major hurdle. After the acute stage of injury, recovering sexual function is a high priority. AIM: To review the efficacy of intracavernous injections (ICIs) in men with SCI and to identify prognostic factors affecting the efficacy of ICIs in this population. METHODS: Systematic review of the literature was conducted using the PubMed-Medline, Embase, EBSCO, Web of Science, and Cochrane Library databases...
July 2016: Sexual Medicine Reviews
https://www.readbyqxmd.com/read/27867347/susceptibility-of-mutant-sod1-to-form-a-destabilized-monomer-predicts-cellular-aggregation-and-toxicity-but-not-in-vitro-aggregation-propensity
#11
Luke McAlary, J Andrew Aquilina, Justin J Yerbury
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the rapid and progressive degeneration of upper and lower motor neurons in the spinal cord, brain stem and motor cortex. The first gene linked to ALS was the gene encoding the free radical scavenging enzyme superoxide dismutase-1 (SOD1) that currently has over 180, mostly missense, ALS-associated mutations identified. SOD1-associated fALS patients show remarkably broad mean survival times (<1 year to ~17 years death post-diagnosis) that are mutation dependent...
2016: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/27867060/the-role-of-efr3a-in-age-related-hearing-loss
#12
Haixia Hu, Yan Ma, Bin Ye, Quan Wang, Tao Yang, Jingrong Lv, Jun Shi, Hao Wu, Mingliang Xiang
Efr3a has been found to be involved in the functional maintenance and structural degeneration of sensory and motor nervous tissues. Our previous data have suggested that Efr3a may be associated with the initiation of the degeneration of spiral ganglion neurons (SGNs). In this study, we used Efr3a knockdown (Efr3a KD) and Efr3a overexpression (Efr3a OE) mice to determine the role of Efr3a in age-related hearing loss. Measurements of hearing thresholds showed that Efr3a had little or no influence on the hearing threshold at all frequencies in adult mice, whereas in an early stage of senescence, Efr3a reduction resulted in better hearing function, especially at 10 and 12months of age...
November 17, 2016: Neuroscience
https://www.readbyqxmd.com/read/27863507/transgenic-mice-overexpressing-the-als-linked-protein-matrin-3-develop-a-profound-muscle-phenotype
#13
Christina Moloney, Sruti Rayaprolu, John Howard, Susan Fromholt, Hilda Brown, Matt Collins, Mariela Cabrera, Colin Duffy, Zoe Siemienski, Dave Miller, Maurice S Swanson, Lucia Notterpek, David R Borchelt, Jada Lewis
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of upper and lower motor neurons. Mutations in the gene encoding the nuclear matrix protein Matrin 3 have been found in familial cases of ALS, as well as autosomal dominant distal myopathy with vocal cord and pharyngeal weakness. We previously found that spinal cord and muscle, organs involved in either ALS or distal myopathy, have relatively lower levels of Matrin 3 compared to the brain and other peripheral organs in the murine system...
November 18, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27863479/myasthenic-symptoms-in-anti-low-density-lipoprotein-receptor-related-protein-4-antibody-seropositive-amyotrophic-lateral-sclerosis-two-case-reports
#14
Hisashi Takahashi, Yu-Ichi Noto, Naoki Makita, Yukie Kushimura-Okada, Ryotaro Ishii, Akihiro Tanaka, Tomoyuki Ohara, Shunya Nakane, Osamu Higuchi, Masanori Nakagawa, Toshiki Mizuno
BACKGROUND: Myasthenic symptoms can be present in patients with amyotrophic lateral sclerosis (ALS). These symptoms have been considered to be caused by the degeneration of distal motor neurons and the neuromuscular junction (NMJ). Recent studies suggested that antibody to low-density lipoprotein receptor-related protein 4 (LRP4) was a pathogenic agent of myasthenia gravis (MG), and it was also detected in ALS patients. CASE PRESENTATION: Patient 1: A 58-year-old Japanese man developed progressive weakness and subsequent myasthenic symptoms including oculomotor disturbance...
November 18, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27858953/the-concept-and-diagnostic-criteria-of-primary-lateral-sclerosis
#15
Verena Wais, Angela Rosenbohm, Susanne Petri, Katja Kollewe, Andreas Hermann, Alexander Storch, Frank Hanisch, Stephan Zierz, Gabriele Nagel, Jan Kassubek, Patrick Weydt, Johannes Brettschneider, Jochen H Weishaupt, Albert C Ludolph, Johannes Dorst
OBJECTIVES: Primary lateral sclerosis (PLS) is commonly considered as a motor neuron disease (MND) variant which almost exclusively affects upper motor neurons (UMN). There is still no consensus whether PLS should be regarded as an independent disease entity separate from amyotrophic lateral sclerosis (ALS) or as a comparatively slowly progressive variant of ALS. Given these different views, clinical diagnosis of PLS is a challenge. In this multicenter study, we analyzed clinical features of patients diagnosed with PLS in four specialized MND centers...
November 15, 2016: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/27837005/glial-activation-colocalizes-with-structural-abnormalities-in-amyotrophic-lateral-sclerosis
#16
Mohamad J Alshikho, Nicole R Zürcher, Marco L Loggia, Paul Cernasov, Daniel B Chonde, David Izquierdo Garcia, Julia E Yasek, Oluwaseun Akeju, Ciprian Catana, Bruce R Rosen, Merit E Cudkowicz, Jacob M Hooker, Nazem Atassi
OBJECTIVE: In this cross-sectional study, we aimed to evaluate brain structural abnormalities in relation to glial activation in the same cohort of participants. METHODS: Ten individuals with amyotrophic lateral sclerosis (ALS) and 10 matched healthy controls underwent brain imaging using integrated MR/PET and the radioligand [(11)C]-PBR28. Diagnosis history and clinical assessments including Upper Motor Neuron Burden Scale (UMNB) were obtained from patients with ALS...
November 11, 2016: Neurology
https://www.readbyqxmd.com/read/27830492/transcranial-magnetic-stimulation-for-the-assessment-of-neurodegenerative-disease
#17
REVIEW
Steve Vucic, Matthew C Kiernan
Transcranial magnetic stimulation (TMS) is a noninvasive technique that has provided important information about cortical function across an array of neurodegenerative disorders, including Alzheimer's disease, frontotemporal dementia, Parkinson's disease, and related extrapyramidal disorders. Application of TMS techniques in neurodegenerative diseases has provided important pathophysiological insights, leading to the development of pathogenic and diagnostic biomarkers that could be used in the clinical setting and therapeutic trials...
November 9, 2016: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/27821773/reduced-high-frequency-motor-neuron-firing-emg-fractionation-and-gait-variability-in-awake-walking-als-mice
#18
Muhamed Hadzipasic, Weiming Ni, Maria Nagy, Natalie Steenrod, Matthew J McGinley, Adi Kaushal, Eleanor Thomas, David A McCormick, Arthur L Horwich
Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease prominently featuring motor neuron (MN) loss and paralysis. A recent study using whole-cell patch clamp recording of MNs in acute spinal cord slices from symptomatic adult ALS mice showed that the fastest firing MNs are preferentially lost. To measure the in vivo effects of such loss, awake symptomatic-stage ALS mice performing self-initiated walking on a wheel were studied. Both single-unit extracellular recordings within spinal cord MN pools for lower leg flexor and extensor muscles and the electromyograms (EMGs) of the corresponding muscles were recorded...
November 22, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27818809/neurobrucellosis-a-case-report-from-himachal-pradesh-india-and-review-of-the-literature
#19
Sujeet Raina, Ashish Sharma, Rajesh Sharma, Amit Bhardwaj
Human brucellosis is a multisystem disease that commonly presents as a febrile illness along with variable spectrum of clinical manifestations. Neurological complications include encephalitis, meningoencephalitis, radiculitis, myelitis, peripheral and cranial neuropathies, subarachnoid hemorrhage, and psychiatric manifestations. We report a case diagnosed as neurobrucellosis who presented with fever and bilateral upper motor neuron symptoms and signs along with bilateral sensorineural deafness. Diagnosis was confirmed by Rose Bengal Test (RBT) and standard tube agglutination test (SAT)...
2016: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/27815397/new-strategy-that-delays-progression-of-amyotrophic-lateral-sclerosis-in-g1h-g93a-transgenic-mice-oral-administration-of-xanthine-oxidoreductase-inhibitors-that-are-not-substrates-for-the-purine-salvage-pathway
#20
Shinsuke Kato, Masako Kato, Teruo Kusano, Takeshi Nishino
Amyotrophic lateral sclerosis (ALS), Lou Gehrig's disease, is a progressive fatal neurodegenerative disease that involves both upper and lower motor neurons. We orally administered 4 xanthine oxidoreductase (XOR) inhibitors to G1H-G93A mice carrying 25 transgene copy numbers of human mutant G93A superoxide dismutase 1, from 80 days of age. Three nonpurine-analogue inhibitors (TEI-6720: Febuxostat, Y-700 and FYX-051), but not allopurinol with a purine analogue ring (pyrazolo pyrimidine ring), significantly delayed disease onset, prolonged survival and the duration of disease stages, improved clinical signs, and alleviated weight loss...
November 4, 2016: Journal of Neuropathology and Experimental Neurology
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