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https://www.readbyqxmd.com/read/29767817/-motor-neuron-diseases-clinical-and-genetic-differential-diagnostics
#1
REVIEW
M Regensburger, N Weidner, Z Kohl
The causes of degenerative disease of the upper and lower motor neurons are incompletely understood. In this review the current concepts in the clinical and genetic differential diagnostics of motor neuron diseases are presented. Hereditary spastic paraplegia, primary lateral sclerosis, spinal muscular atrophy and amyotrophic lateral sclerosis are explained, structured according to the affection of the upper and/or lower motor neuron. The substantial variability in the presentation and course of motor neuron diseases as well as the lack of specific laboratory tests hinder an early diagnosis...
May 16, 2018: Der Nervenarzt
https://www.readbyqxmd.com/read/29766929/sleep-disorders-in-amyotrophic-lateral-sclerosis-a-questionnaire-based-study-from-india
#2
Samhita Panda, Mandaville Gourie-Devi, Ankkita Sharma
Background: Amyotrophic lateral sclerosis (ALS) is a relatively rare neurological disorder affecting upper and lower motor neurons in the brain and spinal cord with survival for 3-5 years and rarely beyond 10 years. Sleep disturbances in ALS are underreported and undertreated and there is no related data from India. This study aimed to assess the frequency of sleep disorders in patients of ALS and their determinants. Methods: Patients with definite and probable ALS as per the El Escorial criteria were recruited from May 2014 to April 2016...
May 2018: Neurology India
https://www.readbyqxmd.com/read/29765873/characteristics-of-cricopharyngeal-dysphagia-after-ischemic-stroke
#3
Hyuna Yang, Youbin Yi, Yong Han, Hyun Jung Kim
Objective: To evaluate the characteristics of cricopharyngeal dysfunction (CPD), the frequency, and correlation with a brain lesion in patients with first-ever ischemic stroke, and to provide basic data for developing a therapeutic protocol for dysphagia management. Methods: We retrospectively reviewed the medical records of a series of subjects post-stroke who underwent a videofluoroscopic swallowing study (VFSS) from January 2009 to December 2015. VFSS images were recorded on videotape and analyzed...
April 2018: Annals of Rehabilitation Medicine
https://www.readbyqxmd.com/read/29760466/two-photon-imaging-of-neuronal-activity-in-motor-cortex-of-marmosets-during-upper-limb-movement-tasks
#4
Teppei Ebina, Yoshito Masamizu, Yasuhiro R Tanaka, Akiya Watakabe, Reiko Hirakawa, Yuka Hirayama, Riichiro Hira, Shin-Ichiro Terada, Daisuke Koketsu, Kazuo Hikosaka, Hiroaki Mizukami, Atsushi Nambu, Erika Sasaki, Tetsuo Yamamori, Masanori Matsuzaki
Two-photon imaging in behaving animals has revealed neuronal activities related to behavioral and cognitive function at single-cell resolution. However, marmosets have posed a challenge due to limited success in training on motor tasks. Here we report the development of protocols to train head-fixed common marmosets to perform upper-limb movement tasks and simultaneously perform two-photon imaging. After 2-5 months of training sessions, head-fixed marmosets can control a manipulandum to move a cursor to a target on a screen...
May 14, 2018: Nature Communications
https://www.readbyqxmd.com/read/29753924/converging-cellular-themes-for-the-hereditary-spastic-paraplegias
#5
REVIEW
Craig Blackstone
Hereditary spastic paraplegias (HSPs) are neurologic disorders characterized by prominent lower-extremity spasticity, resulting from a length-dependent axonopathy of corticospinal upper motor neurons. They are among the most genetically-diverse neurologic disorders, with >80 distinct genetic loci and over 60 identified genes. Studies investigating the molecular pathogenesis underlying HSPs have emphasized the importance of converging cellular pathogenic themes in the most common forms of HSP, providing compelling targets for therapy...
May 10, 2018: Current Opinion in Neurobiology
https://www.readbyqxmd.com/read/29740862/integrated-mri-and-11-c-pbr28-pet-imaging-in-amyotrophic-lateral-sclerosis
#6
Mohamad J Alshikho, Nicole R Zürcher, Marco L Loggia, Paul Cernasov, Beverly Reynolds, Olivia Pijanowski, Daniel B Chonde, David Izquierdo Garcia, Caterina Mainero, Ciprian Catana, James Chan, Suma Babu, Sabrina Paganoni, Jacob M Hooker, Nazem Atassi
OBJECTIVE: To characterize [11 C]-PBR28 brain uptake using positron emission tomography (PET) in people with amyotrophic lateral sclerosis (ALS), and primary lateral sclerosis (PLS). We have previously shown increased [11 C]-PBR28 uptake in the precentral gyrus in a small group of ALS patients. Herein, we confirm our initial finding, study the longitudinal changes, and characterize the gray vs. white matter distribution of [11 C]-PBR28 uptake in a larger cohort of patients with ALS and PLS...
May 8, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29725842/motor-neuron-disease-of-paraneoplastic-origin-a-rare-but-treatable-condition
#7
Nicolas Mélé, Giulia Berzero, Thierry Maisonobe, François Salachas, Guillaume Nicolas, Nicolas Weiss, Guillemette Beaudonnet, Francois Ducray, Dimitri Psimaras, Timothée Lenglet
Paraneoplastic motor neuron disorders (MND) are rare conditions; their exact clinical and electrophysiological phenotype have not been exhaustively described yet. The purpose of this study is to depict the main characteristics of paraneoplastic MND to highlight the features that may allow its diagnosis. Based on the description of eight original cases, and on the revision of 21 patients identified from a systematic review of the literature, the main features of paraneoplastic MND can be summarized as follows: (1) subacute; (2) lower motor neuron syndrome, associated or not with upper motor neuron involvement; (3) predominant asymmetric upper limb involvement; (4) presence of other non-motor neurological manifestations, including sensory neuronopathy; (5) signs of inflammation in the cerebrospinal fluid (CSF); (6) neurological improvement or stabilization after immunotherapy and tumor treatment...
May 3, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29709651/sensory-nerves-disturbance-in-amyotrophic-lateral-sclerosis
#8
REVIEW
Qing-Qing Tao, Qiao Wei, Zhi-Ying Wu
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disorder, characterized by the selective degeneration of upper and lower motor neurons. The common clinical symptoms of ALS are caused by the degeneration and dysfunction of motor neurons. With the progression of our understanding of the pathogenesis of the disease, an increasing number of extramotor phenotypes have been linked to ALS. It has long been believed that sensory neurons localized in the dorsal root ganglia are not involved in ALS...
April 27, 2018: Life Sciences
https://www.readbyqxmd.com/read/29707616/rostrocaudal-areal-patterning-of-human-psc-derived-cortical-neurons-by-fgf8-signaling
#9
Kent Imaizumi, Koki Fujimori, Seiji Ishii, Asako Otomo, Yasushi Hosoi, Hiroaki Miyajima, Hitoshi Warita, Masashi Aoki, Shinji Hadano, Wado Akamatsu, Hideyuki Okano
The cerebral cortex is subdivided into distinct areas that have particular functions. The rostrocaudal (R-C) gradient of fibroblast growth factor 8 (FGF8) signaling defines this areal identity during neural development. In this study, we recapitulated cortical R-C patterning in human pluripotent stem cell (PSC) cultures. Modulation of FGF8 signaling appropriately regulated the R-C markers, and the patterns of global gene expression resembled those of the corresponding areas of human fetal brains. Furthermore, we demonstrated the utility of this culture system in modeling the area-specific forebrain phenotypes [presumptive upper motor neuron (UMN) phenotypes] of amyotrophic lateral sclerosis (ALS)...
March 2018: ENeuro
https://www.readbyqxmd.com/read/29705168/stenosis-and-neurological-level-discrepancies-in-cervical-spondylotic-myelopathy
#10
Young-Ah Choi, Keewon Kim
BACKGROUND: Cervical spondylotic myelopathy (CSM) is the most common cause of cord dysfunction in older individuals. CSM involves either the upper motor neuron or lower motor neuron (LMN) in the cervical spine. The neurological level of the LMN lesion does not usually correspond with the structural level detected via magnetic resonance imaging (MRI) OBJECTIVE: To examine the relationship between the structural level of stenosis and the neurological level of LMN involvement in patients with CSM DESIGN: Retrospective descriptive study SETTING: Tertiary hospital PATIENTS: Patients with cervical spondylotic myelopathy, aged 41 to 79 years METHODS: We reviewed electromyography (EMG) and magnetic resonance imaging (MRI) findings from 17 patients with CSM to objectively show the relationship between the structural level of stenosis and the neurological level with lower motor neuron involvement...
April 26, 2018: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/29698489/altered-distribution-of-atg9a-and-accumulation-of-axonal-aggregates-in-neurons-from-a-mouse-model-of-ap-4-deficiency-syndrome
#11
Raffaella De Pace, Miguel Skirzewski, Markus Damme, Rafael Mattera, Jeffrey Mercurio, Arianne M Foster, Loreto Cuitino, Michal Jarnik, Victoria Hoffmann, H Douglas Morris, Tae-Un Han, Grazia M S Mancini, Andrés Buonanno, Juan S Bonifacino
The hereditary spastic paraplegias (HSP) are a clinically and genetically heterogeneous group of disorders characterized by progressive lower limb spasticity. Mutations in subunits of the heterotetrameric (ε-β4-μ4-σ4) adaptor protein 4 (AP-4) complex cause an autosomal recessive form of complicated HSP referred to as "AP-4 deficiency syndrome". In addition to lower limb spasticity, this syndrome features intellectual disability, microcephaly, seizures, thin corpus callosum and upper limb spasticity...
April 26, 2018: PLoS Genetics
https://www.readbyqxmd.com/read/29697388/-transformation-of-myasthenia-gravis-into-amyotrophic-lateral-sclerosis-or-their-concomitance-case-review
#12
N Kvirkvelia, R Shakarishvili, T Kanashvili
The authors present a case of 75-year-old male patient with typical clinical and electroneuromyographic signs of Amyotrophic Lateral Sclerosis (ALS), manifested in 4 years after a diagnosis of generalized Myasthenia Gravis (MG) had been made. The aim of the article is to assess the possibility of pathogenetic integrated comorbidity of MG and ALS, which may have resulted from a common aberrant immune process and to emphasize the importance of detailed clinical analysis and adequate diagnostic methods essential for correct diagnosis and treatment...
March 2018: Georgian Medical News
https://www.readbyqxmd.com/read/29659159/spinal-accessory-motor-neurons-in-the-mouse-a-special-type-of-branchial-motor-neuron
#13
Charles Watson, Petr Tvrdik
The spinal accessory nerve arises from motor neurons in the upper cervical spinal cord. The axons of these motor neurons exit dorsal to the ligamentum denticulatum and form the spinal accessory nerve. The nerve ascends in the spinal subarachnoid space to enter the posterior cranial fossa through the foramen magnum. The spinal accessory nerve then turns caudally to exit through the jugular foramen alongside the vagus and glossopharyngeal nerves, and then travels to supply the sternomastoid and trapezius muscles in the neck...
April 16, 2018: Anatomical Record: Advances in Integrative Anatomy and Evolutionary Biology
https://www.readbyqxmd.com/read/29656927/pontocerebellar-hypoplasia-type-1-for-the-neuropediatrician-genotype-phenotype-correlations-and-diagnostic-guidelines-based-on-new-cases-and-overview-of-the-literature
#14
I Ivanov, D Atkinson, I Litvinenko, L Angelova, S Andonova, H Mumdjiev, I Pacheva, M Panova, R Yordanova, V Belovejdov, A Petrova, M Bosheva, T Shmilev, A Savov, A Jordanova
Pontocerebellar hypoplasia type 1 (PCH1) is a major cause of non-5q spinal muscular atrophy (SMA). We screened 128 SMN1-negative SMA patients from Bulgaria for a frequent mutation -p.G31A in EXOSC3, and performed a literature review of all genetically verified PCH1 cases. Homozygous p.G31A/EXOSC3 mutation was identified in 14 Roma patients, representing three fourths of all our SMN1-negative Roma SMA cases. The phenotype of the p.G31A/EXOSC3 homozygotes was compared to the clinical presentation of all reported to date genetically verified PCH1 cases...
April 3, 2018: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29621978/high-frequency-of-the-tardbp-p-m337-v-mutation-among-south-eastern-chinese-patients-with-familial-amyotrophic-lateral-sclerosis
#15
Guo-Rong Xu, Wei Hu, Ling-Ling Zhan, Chong Wang, Liu-Qing Xu, Min-Ting Lin, Wan-Jin Chen, Ning Wang, Qi-Jie Zhang
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a devastating motor neuron disease characterized by substantial clinical and genetic heterogeneity. Thus far, only a few TARDBP-ALS families have been reported in China, and no mutation analysis has been reported in south-eastern China. METHODS: Seven index cases from ALS families negative for SOD1 and FUS mutations were screened by Sanger sequencing for TARDBP gene exons 2-6. TARDBP exon 6 was analysed in 215 sporadic ALS patients...
April 5, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29615862/serotonergic-dysfunction-in-amyotrophic-lateral-sclerosis-and-parkinson-s-disease-similar-mechanisms-dissimilar-outcomes
#16
Yannick Vermeiren, Jana Janssens, Debby Van Dam, Peter P De Deyn
Amyotrophic lateral sclerosis (ALS) and Parkinson's disease (PD) share similar pathophysiological mechanisms. From a neurochemical point of view, the serotonin (5-hydroxytryptamine; 5-HT) dysfunction in both movement disorders-related to probable lesioning of the raphe nuclei-is profound, and, therefore, may be partially responsible for motor as well as non-motor disturbances. More specifically, in ALS, it has been hypothesized that serotonergic denervation leads to loss of its inhibitory control on glutamate release, resulting into glutamate-induced neurotoxicity in lower and/or upper motor neurons, combined with a detrimental decrease of its facilitatory effects on glutamatergic motor neuron excitation...
2018: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/29590745/high-frequency-stimulation-of-the-subthalamic-nucleus-activates-motor-cortex-pyramidal-tract-neurons-by-a-process-involving-local-glutamate-gaba-and-dopamine-receptors-in-hemi-parkinsonian-rats
#17
Chi-Fen Chuang, Chen-Wei Wu, Ying Weng, Pei-San Hu, Shin-Rung Yeh, Yen-Chung Chang
Deep brain stimulation (DBS) is widely used to treat advanced Parkinson’s disease. Here, we investigated how DBS applied on the subthalamic nucleus (STN) influenced the neural activity in the motor cortex. Rats, which had the midbrain dopaminergic neurons partially depleted unilaterally, called the hemi-Parkinsonian rats, were used as a study model. c-Fos expression in the neurons was used as an indicator of neural activity. Application of high-frequency stimulation (HFS) upon the STN was used to mimic the DBS treatment...
March 29, 2018: Chinese Journal of Physiology
https://www.readbyqxmd.com/read/29582019/truncating-slc5a7-mutations-underlie-a-spectrum-of-dominant-hereditary-motor-neuropathies
#18
Claire G Salter, Danique Beijer, Holly Hardy, Katy E S Barwick, Matthew Bower, Ines Mademan, Peter De Jonghe, Tine Deconinck, Mark A Russell, Meriel M McEntagart, Barry A Chioza, Randy D Blakely, John K Chilton, Jan De Bleecker, Jonathan Baets, Emma L Baple, David Walk, Andrew H Crosby
Objective: To identify the genetic cause of disease in 2 previously unreported families with forms of distal hereditary motor neuropathies (dHMNs). Methods: The first family comprises individuals affected by dHMN type V, which lacks the cardinal clinical feature of vocal cord paralysis characteristic of dHMN-VII observed in the second family. Next-generation sequencing was performed on the proband of each family. Variants were annotated and filtered, initially focusing on genes associated with neuropathy...
April 2018: Neurology. Genetics
https://www.readbyqxmd.com/read/29577934/selective-spatio-temporal-vulnerability-of-central-nervous-system-neurons-to-pathological-tar-dna-binding-protein-43-in-aged-transgenic-mice
#19
Annika van Hummel, Gabriella Chan, Julia van der Hoven, Marco Morsch, Stefania Ippati, Lisa Suh, Mian Bi, Prita R Asih, Wei Siang Lee, Troy A Butler, Magdalena Przybyla, Glenda M Halliday, Olivier Piguet, Matthew C Kiernan, Roger S Chung, Lars M Ittner, Yazi D Ke
Amyotrophic lateral sclerosis (ALS) is a rapidly progressing and fatal disease characterized by muscular atrophy due to loss of upper and lower motor neurons. Histopathologically, the majority of ALS cases present with abnormal cytoplasmic accumulation and aggregation of the nuclear RNA-regulating protein TAR DNA-binding protein 43 (TDP-43). Pathogenic mutations in the TARDBP gene that encodes TDP-43 have been identified in familial ALS. We have previously reported transgenic mice with neuronal expression of human TDP-43 carrying the pathogenic A315T mutation (iTDP-43A315T mice), presenting with early-onset motor deficits in adolescent animals...
March 22, 2018: American Journal of Pathology
https://www.readbyqxmd.com/read/29564498/beyond-fractional-anisotropy-in-amyotrophic-lateral-sclerosis-the-value-of-mean-axial-and-radial-diffusivity-and-its-correlation-with-electrophysiological-conductivity-changes
#20
Ana Filipa Geraldo, João Pereira, Pedro Nunes, Sofia Reimão, Rita Sousa, Miguel Castelo-Branco, Susana Pinto, Jorge Guedes Campos, Mamede de Carvalho
PURPOSE: This paper aims to analyze the contribution of mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) in the detection of microstructural abnormalities in amyotrophic lateral sclerosis (ALS) and to evaluate the degree of agreement between structural and functional changes through concomitant diffusion tensor imaging (DTI), transcranial magnetic stimulation (TMS), and clinical assessment. METHODS: Fourteen patients with ALS and 11 healthy, age- and gender-matched controls were included...
May 2018: Neuroradiology
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