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Upper motor neuron

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https://www.readbyqxmd.com/read/28226823/properties-of-primary-motor-cortical-local-field-potentials-in-the-leg-and-trunk-representations-during-arm-movements
#1
Adil A Tobaa, Matthew D Best, Karthikeyan Balasubramanian, Kazutaka Takahashi, Nicholas G Hatsopoulos, Adil A Tobaa, Matthew D Best, Karthikeyan Balasubramanian, Kazutaka Takahashi, Nicholas G Hatsopoulos, Adil A Tobaa, Matthew D Best, Karthikeyan Balasubramanian, Kazutaka Takahashi, Nicholas G Hatsopoulos
Large, spatially-distributed populations of motor cortical neurons are recruited during upper limb movements. Here, we examined how beta attenuation, a mesoscopic reflection of unit engagement, varies across a spatially expansive sampling of primary motor cortex in a non-human primate (macaca mulatta). We found that electrodes in both the trunk and leg representation of motor cortex exhibit qualitatively similar behavior to electrodes in the arm representation during a planar reaching task, despite the fact that there were no overt movements of the trunk or leg...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28223914/decoding-lower-limb-muscle-activity-and-kinematics-from-cortical-neural-spike-trains-during-monkey-performing-stand-and-squat-movements
#2
Xuan Ma, Chaolin Ma, Jian Huang, Peng Zhang, Jiang Xu, Jiping He
Extensive literatures have shown approaches for decoding upper limb kinematics or muscle activity using multichannel cortical spike recordings toward brain machine interface (BMI) applications. However, similar topics regarding lower limb remain relatively scarce. We previously reported a system for training monkeys to perform visually guided stand and squat tasks. The current study, as a follow-up extension, investigates whether lower limb kinematics and muscle activity characterized by electromyography (EMG) signals during monkey performing stand/squat movements can be accurately decoded from neural spike trains in primary motor cortex (M1)...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28211850/subthalamic-neurons-encode-both-single-and-multi-limb-movements-in-parkinson-s-disease-patients
#3
Ariel Tankus, Ido Strauss, Tanya Gurevich, Anat Mirelman, Nir Giladi, Itzhak Fried, Jeffrey M Hausdorff
The subthalamic nucleus (STN) is the main target for neurosurgical treatment of motor signs of Parkinson's disease (PD). Despite the therapeutic effect on both upper and lower extremities, its role in motor control and coordination and its changes in Parkinson's disease are not fully clear. We intraoperatively recorded single unit activity in ten patients with PD who performed repetitive feet or hand movements while undergoing implantation of a deep brain stimulator. We found both distinct and overlapping representations of upper and lower extremity movement kinematics in subthalamic units and observed evidence for re-routing to a multi-limb representation that participates in limb coordination...
February 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28184211/disrupted-saccade-control-in-chronic-cerebral-injury-upper-motor-neuron-like-disinhibition-in-the-ocular-motor-system
#4
John-Ross Rizzo, Todd E Hudson, Andrew Abdou, Yvonne W Lui, Janet C Rucker, Preeti Raghavan, Michael S Landy
Saccades rapidly direct the line of sight to targets of interest to make use of the high acuity foveal region of the retina. These fast eye movements are instrumental for scanning visual scenes, foveating targets, and, ultimately, serve to guide manual motor control, including eye-hand coordination. Cerebral injury has long been known to impair ocular motor control. Recently, it has been suggested that alterations in control may be useful as a marker for recovery. We measured eye movement control in a saccade task in subjects with chronic middle cerebral artery stroke with both cortical and substantial basal ganglia involvement and in healthy controls...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28182676/the-swimming-test-is-effective-for-evaluating-spasticity-after-contusive-spinal-cord-injury
#5
Youngjae Ryu, Toru Ogata, Motoshi Nagao, Taku Kitamura, Kazuhito Morioka, Yoshinori Ichihara, Toru Doi, Yasuhiro Sawada, Masami Akai, Ryohei Nishimura, Naoki Fujita
Spasticity is a frequent chronic complication in individuals with spinal cord injury (SCI). However, the severity of spasticity varies in patients with SCI. Therefore, an evaluation method is needed to determine the severity of spasticity. We used a contusive SCI model that is suitable for clinical translation. In this study, we examined the feasibility of the swimming test and an EMG for evaluating spasticity in a contusive SCI rat model. Sprague-Dawley rats received an injury at the 8th thoracic vertebra...
2017: PloS One
https://www.readbyqxmd.com/read/28162871/a-young-female-with-urinary-retention-a-case-report-of-hashimoto-s-encephalopathy
#6
Benjamin L Cooper, Shane E Appel, Hussam M Ammar
Hashimoto's Encephalopathy (HE) is a rare form of autoimmune encephalopathy associated with Hashimoto's thyroiditis in which patients experience cognitive impairment and various neurologic symptoms. We present a case of a young female that presented to the emergency department with urinary retention, and was ultimately diagnosed with HE. Examination was significant for direction-changing and vertical nystagmus (direction-changing nystagmus describes a phenomenon where the fast beat changes with the direction of gaze), hyperreflexia, clonus, and Babinski and Hoffman's reflexes (all upper motor neuron (UMN) signs)...
January 26, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28131894/gaba-and-glutamate-levels-correlate-with-mtr-and-clinical-disability-insights-from-multiple-sclerosis
#7
Julia C Nantes, Sébastien Proulx, Jidan Zhong, Scott A Holmes, Sridar Narayanan, Robert A Brown, Richard D Hoge, Lisa Koski
Converging areas of research have implicated glutamate and γ-aminobutyric acid (GABA) as key players in neuronal signalling and other central functions. Further research is needed, however, to identify microstructural and behavioral links to regional variability in levels of these neurometabolites, particularly in the presence of demyelinating disease. Thus, we sought to investigate the extent to which regional glutamate and GABA levels are related to a neuroimaging marker of microstructural damage and to motor and cognitive performance...
January 25, 2017: NeuroImage
https://www.readbyqxmd.com/read/28124177/tfg-associated-hereditary-spastic-paraplegia-an-addition-to-the-phenotypic-spectrum
#8
Huma Tariq, Sadaf Naz
Hereditary spastic paraplegias (HSPs) constitute movement disorders with extreme lower limb spasticity caused by axonopathies of the upper motor neurons. We describe two siblings affected with a recessive form of movement disorder. Whole-exome sequencing revealed a homozygous missense mutation c.64 C>T (p.Arg22Trp) in TFG as cause of the disorder. Comparison of the phenotype of the patients of this study, with that reported previously, revealed differences in the severity of the disorder as well as new clinical findings...
January 25, 2017: Neurogenetics
https://www.readbyqxmd.com/read/28120243/flow-metabolism-uncoupling-in-the-cervical-spinal-cord-of-als-patients
#9
Toru Yamashita, Tetsuhiro Hatakeyama, Kota Sato, Yusuke Fukui, Nozomi Hishikawa, Yasuyuki Ohta, Yoshihiro Nishiyama, Nobuyuki Kawai, Takashi Tamiya, Koji Abe
Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease. In ALS, both glucose consumption and neuronal intensity reportedly decrease in the cerebral motor cortex when measured by positron emission tomography (PET). In this study, we evaluated cervical spinal glucose metabolism, blood flow, and neuronal intensity of 10 ALS patients with upper extremity (U/E) atrophy both with (18)F-2-fluoro-2-deoxy-D-glucose ((18)F-FDG) PET and (11)C-flumazenil ((11)C-FMZ) PET. On the ipsilateral side of C5 and T1 levels, (18)F-FDG uptake increased significantly (*p < 0...
January 24, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28099355/hereditary-spastic-paraplegia-due-to-a-novel-mutation-of-the-reep1-gene-case-report-and-literature-review
#10
Sébastien Richard, Julie Lavie, Guillaume Banneau, Nathalie Voirand, Karine Lavandier, Marc Debouverie
RATIONALE: Hereditary spastic paraplegia (HSP) is a heterogeneous group of diseases little known in clinical practice due to its low prevalence, slow progression, and difficult diagnosis. This results in an underestimation of HSP leading to belated diagnosis and management. In depth diagnosis is based on clinical presentation and identification of genomic mutations. We describe the clinical presentation and pathogeny of HSP through a report of a case due to a novel mutation of the REEP1 gene (SPG31)...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28078312/biallelic-scn10a-mutations-in-neuromuscular-disease-and-epileptic-encephalopathy
#11
Marios Kambouris, Julien Thevenon, Ariane Soldatos, Allison Cox, Joshi Stephen, Tawfeg Ben-Omran, Yasser Al-Sarraj, Hala Boulos, William Bone, James C Mullikin, Alice Masurel-Paulet, Judith St-Onge, Yannis Dufford, Corrine Chantegret, Christel Thauvin-Robinet, Jamil Al-Alami, Laurence Faivre, Jean Baptiste Riviere, William A Gahl, Alexander G Bassuk, May Christine V Malicdan, Hatem El-Shanti
OBJECTIVES: Two consanguineous families, one of Sudanese ethnicity presenting progressive neuromuscular disease, severe cognitive impairment, muscle weakness, upper motor neuron lesion, anhydrosis, facial dysmorphism, and recurrent seizures and the other of Egyptian ethnicity presenting with neonatal hypotonia, bradycardia, and recurrent seizures, were evaluated for the causative gene mutation. METHODS AND RESULTS: Homozygosity mapping and whole exome sequencing (WES) identified damaging homozygous variants in SCN10A, namely c...
January 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/28069760/axotomized-corticospinal-neurons-increase-supra-lesional-innervation-and-remain-crucial-for-skilled-reaching-after-bilateral-pyramidotomy
#12
Alice C Mosberger, Jenifer C Miehlbradt, Nadja Bjelopoljak, Marc P Schneider, Anna-Sophia Wahl, Benjamin V Ineichen, Miriam Gullo, Martin E Schwab
Skilled upper limb function heavily depends on the corticospinal tract. After bilateral lesions to this tract, motor control is disrupted but can be partially substituted by other motor systems to allow functional recovery. However, the remaining roles of motor cortex and especially of axotomized corticospinal neurons (CSNs) are not well understood. Using the single pellet retrieval task in adult rats, we induced significant recovery of skilled reaching after bilateral pyramidotomy by rehabilitative reaching training, and show that reach-related motor cortex activity, recorded in layer V, topographically reappeared shortly after axotomy...
January 8, 2017: Cerebral Cortex
https://www.readbyqxmd.com/read/28068522/motor-evoked-potential-gain-is-a-helpful-test-for-the-detection-of-corticospinal-tract-dysfunction-in-amyotrophic-lateral-sclerosis
#13
Y Duclos, A M Grapperon, E Jouve, R Truillet, C Zemmour, A Verschueren, J Pouget, S Attarian
OBJECTIVE: The detection of upper motor neuron (UMN) dysfunction is necessary for the diagnosis of amyotrophic lateral sclerosis (ALS). However, signs of UMN dysfunction may be difficult to establish. This study aimed to determine whether motor-evoked potential (MEP) gain (MEP area/background electromyographic activity) represents an efficient alternative to assess UMN dysfunction. METHODS: MEP area, MEP/compound muscle action potential (CMAP) area ratio, and MEP gain were tested at different force levels in healthy control subjects and ALS patients...
February 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28058507/als-ftld-experimental-models-and-reality
#14
REVIEW
Rachel H Tan, Yazi D Ke, Lars M Ittner, Glenda M Halliday
Amyotrophic lateral sclerosis is characterised by a loss of upper and lower motor neurons and characteristic muscle weakness and wasting, the most common form being sporadic disease with neuronal inclusions containing the tar DNA-binding protein 43 (TDP-43). Frontotemporal lobar degeneration is characterised by atrophy of the frontal and/or temporal lobes, the most common clinical form being the behavioural variant, in which neuronal inclusions containing either TDP-43 or 3-repeat tau are most prevalent. Although the genetic mutations associated with these diseases have allowed various experimental models to be developed, the initial genetic forms identified remain the most common models employed to date...
January 5, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28057298/amyotrophic-lateral-sclerosis-pathogenesis-converges-on-defects-in-protein-homeostasis-associated-with-tdp-43-mislocalization-and-proteasome-mediated-degradation-overload
#15
G Lin, D Mao, H J Bellen
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder that affects upper and/or lower motor neurons. It usually affects people between the ages of 40-70. The average life expectancy is about 3-5 years after diagnosis and there is no effective cure available. Identification of variants in more than 20 different loci has provided insight into the pathogenic molecular mechanisms mediating disease pathogenesis. In this review, we focus on seven ALS-causing genes: TDP-43, FUS, C9orf72, VCP, UBQLN2, VAPB and SOD-1, which encompass about 90% of the variants causing familial ALS...
2017: Current Topics in Developmental Biology
https://www.readbyqxmd.com/read/28051077/fig4-variants-in-central-european-patients-with-amyotrophic-lateral-sclerosis-a-whole-exome-and-targeted-sequencing-study
#16
Alma Osmanovic, Isolde Rangnau, Anne Kosfeld, Susanne Abdulla, Claas Janssen, Bernd Auber, Peter Raab, Matthias Preller, Susanne Petri, Ruthild G Weber
We aimed to identify the genetic cause of the devastating neurodegenerative disease amyotrophic lateral sclerosis (ALS) in a German family with two affected individuals, and to assess the prevalence of variants in the identified risk gene, FIG4, in a central European ALS cohort. Whole-exome sequencing (WES) and an overlapping data analysis strategy were performed in an ALS family with autosomal dominant inheritance and incomplete penetrance. Additionally, 200 central European ALS patients were analyzed using whole-exome or targeted sequencing...
February 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28044945/recognizing-and-treating-pseudobulbar-affect
#17
William M Sauvé
Pseudobulbar affect, thought by many to be a relatively newly described condition, is in fact a very old one, described as early as the 19th century. It refers to those who experience inappropriate affect, disconnected from internal state, or mood, generally thought to be the result of an upper motor neuron injury or illness. One possible explanation for this condition's relative obscurity is the dearth of treatment options; clinical medicine is not typically in the habit of identifying conditions that cannot be modified...
December 2016: CNS Spectrums
https://www.readbyqxmd.com/read/28040732/mutant-profilin1-transgenic-mice-recapitulate-cardinal-features-of-motor-neuron-disease
#18
Daniel Fil, Abigail DeLoach, Shilpi Yadav, Duah Alkam, Melanie MacNicol, Awantika Singh, Cesar M Compadre, Joseph J Goellner, Charles A O'Brien, Tariq Fahmi, Alexei G Basnakian, Noel Y Calingasan, Jodi L Klessner, M Flint Beal, Owen M Peters, Jake Metterville, Robert H Brown, Karen K Y Ling, Frank Rigo, P Hande Ozdinler, Mahmoud Kiaei
The recent identification of profilin1 mutations in 25 familial ALS cases has linked altered function of this cytoskeleton-regulating protein to the pathogenesis of motor neuron disease. To investigate the pathological role of mutant profilin1 in motor neuron disease, we generated transgenic lines of mice expressing human profilin1 with a mutation at position 118 (hPFN1(G118V)). One of the mouse lines expressing high levels of mutant human PFN1 protein in the brain and spinal cord exhibited many key clinical and pathological features consistent with human ALS disease...
December 30, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/28036062/an-unusual-case-of-post-traumatic-headache-complicated-by-intracranial-hypotension
#19
Sara Siavoshi, Carrie Dougherty, Jessica Ailani, Kaustubh Yadwadkar, Frank Berkowitz
We present a case of post-traumatic headache complicated by intracranial hypotension resulting in an acquired Chiari malformation and myelopathy with syringomyelia. This constellation of findings suggest a possible series of events that started with a traumatic cerebral spinal fluid (CSF) leak, followed by descent of the cerebellar tonsils and disruption of CSF circulation that caused spinal cord swelling and syrinx. This unusual presentation of post-traumatic headache highlights the varying presentations and the potential sequelae of intracranial hypotension...
December 29, 2016: Brain Sciences
https://www.readbyqxmd.com/read/28035186/rapid-progression-of-sporadic-als-in-a-patient-carrying-sod1-p-gly13arg-mutation
#20
Myung-Jin Kim, Jae-Han Bae, Jeong-Min Kim, Hye Ryoun Kim, Byung-Nam Yoon, Jung-Joon Sung, Suk-Won Ahn
Amyotrophic lateral sclerosis (ALS), the most common adult onset motor neuron disease, is pathologically characterized by progressive loss of the upper and lower motor neurons. Mutations in the Cu/Zn superoxide dismutase gene (SOD1) account for about 20% of familial ALS cases and a small percentage of sporadic ALS (SALS) cases, and have revealed a validated genotype-phenotype correlation. Herein, we report a p.Gly13Arg mutation in SOD1 exon 1 in a patient with SALS who presented with a rapidly progressive course, predominantly affecting the lower motor neurons...
December 2016: Experimental Neurobiology
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