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https://www.readbyqxmd.com/read/28766957/immunohistochemical-detection-of-c9orf72-protein-in-frontotemporal-lobar-degeneration-and-motor-neurone-disease-patterns-of-immunostaining-and-an-evaluation-of-commercial-antibodies
#1
Yvonne S Davidson, Andrew C Robinson, Sara Rollinson, Stuart Pickering-Brown, Shangxi Xiao, Janice Robertson, David M A Mann
We have employed as 'gold standards' two in-house, well-characterised and validated polyclonal antibodies, C9-L and C9-S, which detect the longer and shorter forms of C9orf72, and have compared seven other commercially available antibodies with these in order to evaluate the utility of the latter as credible tools for the demonstration of C9orf72. C9-L and C9-S antibodies immunostained cytoplasmic 'speckles', and the nuclear membrane, respectively, in cerebellar Purkinje cells of the cerebellum in patients with behavioural variant frontotemporal dementia (bvFTD) with amyotrophic lateral sclerosis (ALS), and in patients with ALS alone...
August 2, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28761434/brain-injury-in-combination-with-tacrolimus-promotes-the-regeneration-of-injured-peripheral-nerves
#2
Xin-Ze He, Jian-Jun Ma, Hao-Qi Wang, Tie-Min Hu, Bo Sun, Yun-Feng Gao, Shi-Bo Liu, Wei Wang, Pei Wang
Both brain injury and tacrolimus have been reported to promote the regeneration of injured peripheral nerves. In this study, before transection of rat sciatic nerve, moderate brain contusion was (or was not) induced. After sciatic nerve injury, tacrolimus, an immunosuppressant, was (or was not) intraperitoneally administered. At 4, 8 and 12 weeks after surgery, Masson's trichrome, hematoxylin-eosin, and toluidine blue staining results revealed that brain injury or tacrolimus alone or their combination alleviated gastrocnemius muscle atrophy and sciatic nerve fiber impairment on the experimental side, simultaneously improved sciatic nerve function, and increased gastrocnemius muscle wet weight on the experimental side...
June 2017: Neural Regeneration Research
https://www.readbyqxmd.com/read/28761431/synaptosomal-associated-protein-25-may-be-an-intervention-target-for-improving-sensory-and-locomotor-functions-after-spinal-cord-contusion
#3
Zhan-Qiong Zhong, Yang Xiang, Xi Hu, You-Cui Wang, Xi Zeng, Xiao-Meng Wang, Qing-Jie Xia, Ting-Hua Wang, Xiao Zhang
Synaptosomal-associated protein 25 kDa (SNAP-25) is localized on the synapse and participates in exocytosis and neurotransmitter release. Decreased expression of SNAP-25 is associated with Alzheimer's disease and attention deficit/hyperactivity disorder. However, the expression of SNAP-25 in spinal cord contusion injury is still unclear. We hypothesized that SNAP-25 is associated with sensory and locomotor functions after spinal cord injury. We established rat models of spinal cord contusion injury to detect gene changes with a gene array...
June 2017: Neural Regeneration Research
https://www.readbyqxmd.com/read/28749553/myelopathy-in-beh%C3%A3-et-s-disease-the-bagel-sign
#4
Ugur Uygunoglu, Burcu Zeydan, Yesim Ozguler, Serdal Ugurlu, Emire Seyahi, Naci Kocer, Civan Islak, Kejal Kantarci, Sabahattin Saip, Aksel Siva, Orhun H Kantarci
OBJECTIVE: To describe the clinical and distinctive imaging features of myelopathy associated with Behçet's disease (BD). METHODS: We evaluated the records of patients meeting the following criteria: (1) fulfillment of the International Study Group criteria for BD; (2) clinically suggestive of myelopathy; (3) simultaneous spinal cord and brain magnetic resonance images (MRIs) within 1 month of acute worsening of myelopathy; and (4) follow-up duration ≥ 1 year after initial MRI evaluation...
July 27, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28747261/enterovirus-d68-infection-in-a-cluster-of-children-with-acute-flaccid-myelitis-buenos-aires-argentina-2016
#5
Victor Ruggieri, Maria Ivana Paz, María Gabriela Peretti, Carlos Rugilo, Rosa Bologna, Cecilia Freire, Silvana Vergel, Andrea Savransky
OBJECTIVE: To report a outbreak of 11 cases of acute asymmetric flaccid myelopathy due to spinal motor neuron injury. MATERIAL AND METHODS: Eleven children, six male, with a mean age of 3 years presented with acute flaccid myelitis. We analyzed clinical features, etiology, neuroradiological images, treatment, and outcome. RESULTS: Nine children had bilateral and asymmetric flaccid myelitis of the upper limbs, 1 had upper limb monoplegia, and 1 presented with hemiparesis...
July 19, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28737865/unusual-increase-of-cases-of-myelitis-in-a-pediatric-hospital-in-argentina
#6
Guadalupe Pérez, María T Rosanova, María C Freire, María I Paz, Silvina Ruvinsky, Carlos Rugilo, Víctor Ruggieri, Daniel Cisterna, Soledad Martiren, Cristina Lema, Andrea Savransky, Soledad González, Leila Martínez, Diana Viale, Rosa Bologna
INTRODUCTION: The association between enterovirus D68 and acute flaccid myelitis was first described in the United States in 2014. Since then, sporadic cases have been reported in Canada and Europe. This study describes a series of cases of acute flaccid myelitis at Hospital de Pediatría "Prof. Dr. Juan P. Garrahan," in Buenos Aires, Argentina, during 2016. METHODS: Descriptive, retrospective study. All patients with acute flaccid myelitis and lesions in the spinal cord involving the gray matter, as observed in the magnetic resonance imaging (MRI) scan, hospitalized from 04/01/2016 to 07/01/2016, were included in the study...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28669296/a-neurophysiological-approach-to-nerve-transfer-to-restore-upper-limb-function-in-cervical-spinal-cord-injury
#7
Ross M Mandeville, Justin M Brown, Geoffrey L Sheean
A successful nerve transfer surgery can provide a wealth of benefits to a patient with cervical spinal cord injury. The process of surgical decision making ideally uses all pertinent information to produce the best functional outcome. Reliance on clinical examination and imaging studies alone can miss valuable information on the state of spinal cord health. In this regard, neurophysiological evaluation has the potential to effectively gauge the neurological status of even select pools of anterior horn cells and their axons to small nerve branches in question to determine the potential efficacy of their use in a transfer...
July 2017: Neurosurgical Focus
https://www.readbyqxmd.com/read/28662631/a-case-of-neuropsychiatric-lupus-erythematosus-characterized-by-the-owl-s-eye-sign-a-case-report
#8
Bolin Hu, Pengcheng Wu, Yibiao Zhou, Yan Peng, Xiaoping Tang, Weijiang Ding, Ming Zhang, Xueliang Qi
BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disorder characterized by multiple affected systems. More than half of SLE patients will suffer from neuropsychiatric lupus erythematosus (NPSLE) during the course of their disease. Although nearly half of the NPSLE patients have normal MRI manifestations, the abnormalities found in the remainder can be located anywhere in the brain, and especially in the subcortical white matter of the frontal and temporal lobe...
June 29, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28632899/novel-intracytoplasmic-inclusions-immunoreactive-for-phosphorylated-tdp43-and-cystatin-c-in-anterior-horn-cells-in-a-case-of-sporadic-amyotrophic-lateral-sclerosis
#9
Masayuki Shintaku, Daita Kaneda, Kiyomitsu Oyanagi
Novel intracytoplasmic inclusions immunoreactive for phosphorylated transactivation response DNA-binding protein 43 (p-TDP43), cystatin C, and transferrin were found in anterior horn cells in a case of sporadic amyotrophic lateral sclerosis (ALS). The patient was a 59-year-old woman, who died of ALS after a clinical course of 8 years. She had been receiving mechanical support for respiration for 6 years and in a "totally locked-in" state for 4 years prior to death. The spinal cord showed severe degeneration involving the anterior and lateral funiculi, whereas the posterior funiculus was preserved...
June 20, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28607524/development-of-a-modified-model-of-spinal-cord-ischemia-injury-by-selective-ligation-of-lumbar-arteries-in-rabbits
#10
W Xiao, J Wen, Y-C Huang, B-S Yu
STUDY DESIGN: Experimental study. OBJECTIVE: The aim of this study is to develop a modified model of spinal cord ischemia in rabbits. SETTINGS: Shenzhen Key Laboratory of Spine Surgery, Shenzhen, China. METHODS: In total, 20 New Zealand rabbits were divided into the following four groups according to the level of ligation of bilateral lumbar arteries: (1) group A, sham group, no ligation, n=5; (2) group B, ligation of bilateral lumbar arteries at three levels (L2-L4, n=5); (3) group C, ligation of bilateral lumbar arteries at four levels (L2-L5, n=5); and (4) group D, ligation of bilateral lumbar arteries at five levels (L1-L5, n=5)...
June 13, 2017: Spinal Cord
https://www.readbyqxmd.com/read/28583855/characterization-of-aromatase-expression-in-the-spinal-cord-of-an-animal-model-of-familial-als
#11
Can Sun, Yuanyuan Liu, Yaling Liu, Mei Zhao, Jingxu Zhai, Pengli Hao, Ying Wang, Yingxiao Ji
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease involving motor neurons in the motor cortex, brainstem and spinal cord. ALS leads to progressive, aggravated muscle weakness and paralysis. Although the precise pathogenesis remains unknown, several studies have shown that estrogens exert neuroprotective effects during the course of the disease. Aromatase is the key enzyme in estrogen synthesis. In the present study, we used immunohistochemistry, immunofluorescence and western blotting to observe the characteristics of aromatase expression in the spinal cords of copper-zinc superoxide dismutase-1 (SOD1)-G93A transgenic mice...
June 2017: Brain Research Bulletin
https://www.readbyqxmd.com/read/28570645/how-do-sma-linked-mutations-of-smn1-lead-to-structural-functional-deficiency-of-the-sma-protein
#12
Wei Li
Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease with dysfunctional α-motor neurons in the anterior horn of the spinal cord. SMA is caused by loss (∼95% of SMA cases) or mutation (∼5% of SMA cases) of the survival motor neuron 1 gene SMN1. As the product of SMN1, SMN is a component of the SMN complex, and is also involved in the biosynthesis of the small nuclear ribonucleoproteins (snRNPs), which play critical roles in pre-mRNA splicing in the pathogenesis of SMA. To investigate how SMA-linked mutations of SMN1 lead to structural/functional deficiency of SMN, a set of computational analysis of SMN-related structures were conducted and are described in this article...
2017: PloS One
https://www.readbyqxmd.com/read/28566141/bibrachial-plegia-due-to-lyme-radiculopoliomyelitis-myelitis
#13
Feras Akbik, Marcelo Matiello, Amanda Piquet, Tracey Cho, Adam Cohen, Nagagopal Venna
Nervous system involvement occurs in up to 15% of patients with Lyme disease, most commonly manifested as cranial neuropathy, lymphocytic meningitis, and or radiculoneuritis. We describe a patient with subacute radiculopoliomyelitis-myelitis matching the selective involvement of the anterior horns and roots of the cervical spinal cord seen on MRI and on electrodiagnostic studies. We demonstrate positive CSF Lyme antibodies and document a near-complete recovery with antibiotics. This case highlights the importance of recognizing an atypical presentation of Lyme disease in the setting of initial radiculitis and or myelitis, particularly given the potential for favorable outcomes with appropriate treatment...
July 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28549966/lentivirus-mediated-inhibition-of-aqp4-accelerates-motor-function-recovery-associated-with-ngf-in-spinal-cord-contusion-rats
#14
Jianmin Chen, Xi Zeng, Shengnan Li, Zhanqiong Zhong, Xi Hu, Hongyu Xiang, Ying Rao, Lixin Zhang, Xiuya Zhou, Qingjie Xia, Tinghua Wang, Xiao Zhang
Aquaporin-4 (AQP4) is a water channel protein in spinal cord and plays a critical role in the pathophysiological process of spinal cord injury (SCI). However, little is known about the molecular mechanism of AQP4 involved in SCI. The present study was performed to investigate the possible molecules regulated by AQP4 after SCI by use of lentiviral-mediated RNA interference (RNAi). First, the motor function was evaluated by Basso, Beattie, Bresnahan (BBB) scale and the expression of AQP4 was measured by western blot, immunohistochemical staining and immunofluorescence in rats after contusion spinal cord injury (cSCI)...
May 23, 2017: Brain Research
https://www.readbyqxmd.com/read/28539831/aldehyde-dehydrogenases-1a2-expression-and-distribution-are-potentially-associated-with-neuron-death-in-spinal-cord-of-tg-sod1-g93a-1gur-mice
#15
Huiting Liang, Chengsi Wu, Youqing Deng, Lei Zhu, Jie Zhang, Weiming Gan, Chunyan Tang, Renshi Xu
The pathogenesis of amyotrophic lateral sclerosis (ALS) has not been unclear yet, it might be associated with the abnormal expression and distribution of certain proteins. Aldehyde dehydrogenases 1A2 (ALDH1A2) was thought to be one of potential candidates. Therefore, in this study we observed and analyzed the alteration of the expression and distribution of ALDH1A2 in the spinal cord of wild-type (WT) and Tg(SOD1*G93A)1Gur mice. We compared the expression and distribution of ALDH1A2 in the different segments, anatomic regions and neural cells of spinal cord at the different stages of WT and Tg(SOD1*G93A)1Gur mice applied the methods of fluorescent immunohistochemistry and western blot...
2017: International Journal of Biological Sciences
https://www.readbyqxmd.com/read/28503323/severe-cervical-flexion-myelopathy-with-long-tract-signs-a-case-report-and-a-review-of-literature
#16
Takahito Fujimori, Akiko Tamura, Toshitada Miwa, Motoki Iwasaki, Takenori Oda
INTRODUCTION: Hirayama disease, a type of cervical flexion myelopathy, is a rare neurological disease characterized by muscular atrophy of the forearms and hands. Generally, the pathology is limited to the gray matter of the anterior horns in the lower cervical spinal cord. However, in rare cases the damage can spread to the white matter and present as long tract signs. CASE PRESENTATION: We report on a 30-year-old female whose onset presented as unilateral muscle atrophy of the right hand in her teens...
2017: Spinal Cord Series and Cases
https://www.readbyqxmd.com/read/28479534/-cortical-motor-neuron-hyperexcitability-and-motor-neuron-death-in-als-dying-forward-hypothesis
#17
Kazumoto Shibuya
In the late 19th century, Charcot examined patients with amyotrophic lateral sclerosis (ALS) and concluded that the sclerosis in the lateral columns of the spinal cord induced the loss of neurons in the anterior horns. The reason for this remains unknown. In contrast, hyperexcitability of motor neurons is believed to be one of the pathogenetic factors for motor neuron death in ALS. In this article, I have reviewed the relationship between motor neuron hyperexcitability and motor neuron death and considered the mechanism of ALS pathogenesis...
May 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/28413866/inositol-hexakisphosphate-kinase-2-is-a-presymptomatic-biomarker-for-amyotrophic-lateral-sclerosis
#18
Yusuke Moriya, Eiichiro Nagata, Natsuko Fujii, Tadayuki Satoh, Haruko Ogawa, Shinji Hadano, Shunya Takizawa
OBJECTIVE: Inositol hexakisphosphate kinase 2 (InsP6K2), an enzyme that converts inositol hexakisphosphate (InsP6) to diphosphoinositol pentakisphosphate (InsP7), induces cell death. InsP6K2 is abundant in the central nervous system, especially anterior horn cells of spinal cord. To identify the role of InsP6K2 in amyotrophic lateral sclerosis (ALS), we investigated the expression levels of InsP6K2 in transgenic mice expressing mutant superoxide dismutase-1 (SOD1) (mSOD1 Tg mice). METHODS: The specimens of spinal cords were obtained from mSOD1 Tg mice and age-matched wild-type mice...
April 20, 2017: Tokai Journal of Experimental and Clinical Medicine
https://www.readbyqxmd.com/read/28352498/traumatic-cervical-nerve-root-avulsion-with-pseudomeningocele-formation
#19
Ali S Haider, Ian T Watson, Suraj Sulhan, Dean Leonard, Eliel N Arrey, Umair Khan, Phu Nguyen, Kennith F Layton
Cervical nerve root avulsion is a well-documented result of motor vehicle collision (MVC), especially when occurring at high velocities. These avulsions are commonly traction injuries of nerve roots that may be accompanied by a tear in the meninges through the vertebral foramina with associated collections of cerebrospinal fluid (CSF), thereby resulting in a pseudomeningocele. We present a case of a 19-year-old male who experienced an MVC and was brought to the emergency department (ED) with right arm paralysis and other injuries...
February 14, 2017: Curēus
https://www.readbyqxmd.com/read/28283675/amyotrophic-lateral-sclerosis-gene-deregulation-in-the-anterior-horn-of-the-spinal-cord-and-frontal-cortex-area-8-implications-in-frontotemporal-lobar-degeneration
#20
Pol Andrés-Benito, Jesús Moreno, Ester Aso, Mónica Povedano, Isidro Ferrer
Transcriptome arrays identifies 747 genes differentially expressed in the anterior horn of the spinal cord and 2,300 genes differentially expressed in frontal cortex area 8 in a single group of typical sALS cases without frontotemporal dementia compared with age-matched controls. Main up-regulated clusters in the anterior horn are related to inflammation and apoptosis; down-regulated clusters are linked to axoneme structures and protein synthesis. In contrast, up-regulated gene clusters in frontal cortex area 8 involve neurotransmission, synaptic proteins and vesicle trafficking, whereas main down-regulated genes cluster into oligodendrocyte function and myelin-related proteins...
March 9, 2017: Aging
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