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https://www.readbyqxmd.com/read/27897305/-cervical-ischaemic-neuronopathy-and-cardioembolism-another-cause-of-man-in-the-barrel-syndrome
#1
H Gonzalez-Usigli, A Gandarilla, J J Garcia, J Serrato, N Estrada
INTRODUCTION: Spinal infarction accounts for 1% of all strokes. Cardioembolism is a rare cause. Common areas of ischemic spinal damage are watershed in the dorsal or lumbar regions; however cervical spinal cord infarction has been reported previously. CASE REPORT: We present a new case of a man-in-the-barrel syndrome produced by cardiac embolization associated with atrial fibrillation during an acute myocardial infarction, which caused cervical ischemic neuronopathy (infarction of the anterior horn)...
December 16, 2016: Revista de Neurologia
https://www.readbyqxmd.com/read/27800001/clinical-experiences-of-uncommon-motor-neuron-disease-hirayama-disease
#2
Kyoung Hee Lee, Dae Seob Choi, Young Suk Lee, Dong Ho Kang
Hirayama disease, juvenile muscular atrophy of the distal upper limb, is a rare disease predominantly affecting the anterior horn cells of the cervical spinal cord in young men. This cervical myelopathy is associated with neck flexion. It should be suspected in young male patients with a chronic history of weakness and atrophy involving the upper extremities followed by clinical stability in few years. Herein, we report 2 cases of Hirayama disease on emphasis of diagnostic approach and describe the pathognomonic findings at flexion magnetic resonance imaging...
September 2016: Korean Journal of Spine
https://www.readbyqxmd.com/read/27776698/ethyl-pyruvate-modulates-delayed-paralysis-following-thoracic-aortic-ischemia-reperfusion-in-mice
#3
Bao-Ngoc Nguyen, Hassan Albadawi, Rahmi Oklu, Robert S Crawford, Mitchell P Fink, Richard P Cambria, Michael T Watkins
OBJECTIVE: Delayed paralysis is an unpredictable problem for patients undergoing complex repair of the thoracic/thoracoabdominal aorta. These experiments were designed to determine whether ethyl pyruvate (EP), a potent anti-inflammatory and antioxidant agent, might ameliorate delayed paralysis following thoracic aortic ischemia reperfusion (TAR). METHODS: C57BL6 mice were subjected to 5 minutes of thoracic aortic ischemia followed by reperfusion for up to 48 hours...
November 2016: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/27776522/inositol-1-4-5-trisphosphate-receptor-type-1-autoantibodies-in-paraneoplastic-and-non-paraneoplastic-peripheral-neuropathy
#4
Sven Jarius, Marius Ringelstein, Jürgen Haas, Irina I Serysheva, Lars Komorowski, Kai Fechner, Klaus-Peter Wandinger, Philipp Albrecht, Harald Hefter, Andreas Moser, Eva Neuen-Jacob, Hans-Peter Hartung, Brigitte Wildemann, Orhan Aktas
BACKGROUND: Recently, we described a novel autoantibody, anti-Sj/ITPR1-IgG, that targets the inositol 1,4,5-trisphosphate receptor type 1 (ITPR1) in patients with cerebellar ataxia. However, ITPR1 is expressed not only by Purkinje cells but also in the anterior horn of the spinal cord, in the substantia gelatinosa and in the motor, sensory (including the dorsal root ganglia) and autonomic peripheral nervous system, suggesting that the clinical spectrum associated with autoimmunity to ITPR1 may be broader than initially thought...
October 24, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27766033/changes-in-the-expression-of-fus-tls-in-spinal-cords-of-sod1-g93a-transgenic-mice-and-correlation-with-motor-neuron-degeneration
#5
Jiao Li, Yi Lu, Huiting Liang, Chunyan Tang, Lei Zhu, Jie Zhang, Renshi Xu
In order to searching the possible pathogenesis of amyotrophic lateral sclerosis (ALS), we examined the expression and distribution of FUS/TLS protein in the different anatomic regions, segments and neural cells of adult spinal cord at the different stages of the SOD1 wild-type and G93A transgenic mice using the fluorescent immunohistochemistry. Result revealed that, in the SOD1 wild-type mice, the FUS/TLS expression almost wasn't detected. However, in the SOD1 G93A mice, the FUS/TLS expression in the white matter was significantly more than that in the gray matter...
2016: International Journal of Biological Sciences
https://www.readbyqxmd.com/read/27670757/scaffold-free-tissue-engineered-allogenic-adipose-derived-stem-cells-promote-meniscus-healing
#6
Tatsuhiro Toratani, Junsuke Nakase, Hitoaki Numata, Takeshi Oshima, Yasushi Takata, Koichi Nakayama, Hiroyuki Tsuchiya
PURPOSE: To determine whether meniscal tissue could be healed histologically by the implantation of allogenic three-dimensional formed adipose-derived stem cells (ADSCs) in a rabbit model of partial meniscectomy. METHODS: Forty Japanese white rabbits (aged 15-17 weeks) were assigned to 2 groups. Defects 1.5 mm in diameter were created in the anterior horn of the medial menisci. The defects were left empty in the control group and were filled with cylindrical plugs of allogenic ADSCs extracted from adipose tissue in the experimental group...
September 23, 2016: Arthroscopy: the Journal of Arthroscopic & related Surgery
https://www.readbyqxmd.com/read/27664939/formation-and-maturation-of-the-murine-meniscus
#7
Laura W Gamer, Lin Xiang, Vicki Rosen
Meniscal injuries are commonplace, but current surgical repair procedures do not prevent degenerative joint changes that occur after meniscal injury and often lead to osteoarthritis. Successful tissue regeneration in adults often recapitulates events that occur during embryogenesis, suggesting that understanding the regulatory pathways controlling these early processes may provide clues for developing strategies for tissue repair. While the mouse is now widely used to study joint diseases, detailed knowledge of the basic biology of murine meniscus is not readily available...
September 24, 2016: Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society
https://www.readbyqxmd.com/read/27611334/age-related-uptake-of-heavy-metals-in-human-spinal-interneurons
#8
Roger Pamphlett, Stephen Kum Jew
Toxic heavy metals have been implicated in the loss of spinal motoneurons in amyotrophic lateral sclerosis/motor neuron disease (ALS/MND). Motoneuron loss in the spinal anterior horn is severe in ALS/MND at the time of death, making this tissue unsuitable for examination. We therefore examined spinal cords of people without muscle weakness to look for any presence of heavy metals that could make these neurons susceptible to damage. Spinal cord samples from 50 individuals aged 1-95 y who had no clinical or histopathological evidence of spinal motoneuron loss were studied...
2016: PloS One
https://www.readbyqxmd.com/read/27600517/assessment-of-motor-units-in-neuromuscular-disease
#9
Robert D Henderson, Pamela A McCombe
The motor unit comprises the anterior horn cell, its axon, and the muscle fibers that it innervates. Although the true number of motor units is unknown, the number of motor units appears to vary greatly between different muscles and between different individuals. Assessment of the number and function of motor units is needed in diseases of the anterior horn cell and other motor nerve disorders. Amyotrophic lateral sclerosis is the most important disease of anterior horn cells. The need for an effective biomarker for assessing disease progression and for use in clinical trials in amyotrophic lateral sclerosis has stimulated the study of methods to measure the number of motor units...
September 6, 2016: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/27575868/reverse-split-hand-syndrome-dissociated-intrinsic-hand-muscle-atrophy-pattern-in-hirayama-disease-brachial-monomelic-amyotrophy
#10
Ravinder-Jeet Singh, Veeramani Preethish-Kumar, Kiran Polavarapu, Seena Vengalil, Chandrajit Prasad, Atchayaram Nalini
Preferential involvement of C7, C8, T1 level anterior horn cells is a typical feature in Hirayama disease/brachial monomelic amyotrophy (BMMA). There are no clinico-electrophysiological studies to substantiate the peculiar pattern of muscle involvement. Thirty subjects, 10 in each group of BMMA, amyotrophic lateral sclerosis (ALS) and age-matched normal healthy subjects underwent detailed clinical and electrophysiological testing. Results showed that the mean age at evaluation for BMMA and ALS patients was 25...
August 30, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27562394/nasal-polyp-an-incidental-paraganglioma
#11
Ruchi Srivastava, Neelam Wadhwa, Shikha Gupta, Urvashi Razdan
The nose is an uncommon site for head and neck paraganglioma. The diagnosis is seldom established pre-operatively; its rarity, infrequent functionality and often benign biologic outcome underlie this fact. We present one such case in a 60-year-old man who presented with right nasal obstruction and episodic epistaxis. Rhinoscopy revealed a fleshy polypoid mass arising from the anterior cartilaginous nasal septum. Imaging studies excluded extra-nasal extension. The tumor was highly vascular showing numerous variable sized, mostly thin walled branching blood vessels akin to stag-horn shape simulating a vascular neoplasm...
2016: Türk Patoloji Dergisi
https://www.readbyqxmd.com/read/27514792/hypoparathyroidism-a-rare-mimicker-of-amyotrophic-lateral-sclerosis
#12
Haris Hakeem, Masood Uz Zaman, Sara Khan
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) carries a grim prognosis. Various ALS mimics have been reported and should be excluded before confirming this diagnosis. METHODS: We report the case of a 61-year-old man who presented with progressively worsening limb weakness and dysphagia. His examination showed mixed upper and lower motor neuron signs without sensory impairment. ALS was suspected, however atypical diffuse pain prompted diagnostic work-up to exclude other causes...
August 12, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27503823/-an-autopsy-case-of-globular-glial-tauopathy-presenting-with-amyotrophic-lateral-sclerosis-with-dementia
#13
Ryogen Sasaki, Maya Mimuro, Yasumasa Kokubo, Hiroshi Imai, Mari Yoshida, Hidekazu Tomimoto
We report an autopsy case of globular glial tauopathy (GGT) presenting clinically with amyotrophic lateral sclerosis (ALS) with dementia. A 79-year-old female developed weakness in the right upper limb, which progressed gradually. She developed apathy and speech disorder at 80 years of age. On neurological examination, she showed signs of upper and lower motor neuron disorder and dementia, but no extrapyramidal signs. The clinical diagnosis was ALS with dementia. The autopsy revealed left predominant marked atrophy of the frontal lobe due to severe neuronal loss and Gliosis...
August 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27452920/acute-flaccid-weakness-with-myelopathy-and-peripheral-nerve-involvement-in-2-children-recent-characterization-of-a-previously-observed-phenomenon
#14
Gemma Olive, Antonia Clarke, Katja Doerholt, Vasantha Gowda, Atta Siddiqui, Ming J Lim
BACKGROUND: Acute flaccid weakness may be the first presentation of acute transverse myelitis (ATM), an immune-mediated central nervous system disorder or may be the first presentation of anterior horn cell syndrome or peripheral nervous system disease. CASE REPORTS: We describe two previously healthy female infants who presented with acute flaccid paralysis and encephalopathy. Neuroimaging revealed central cord signal changes in both cases and surprisingly electrophysiological studies performed revealed a generalized axonal motor neuropathy as well...
December 0: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/27428822/alleviating-bone-cancer-induced-mechanical-hypersensitivity-by-inhibiting-neuronal-activity-in-the-anterior-cingulate-cortex
#15
Chiuan-Shiou Chiou, Chien-Chung Chen, Tsung-Chih Tsai, Chiung-Chun Huang, Dylan Chou, Kuei-Sen Hsu
BACKGROUND: The anterior cingulate cortex (ACC) is a brain region that has been critically implicated in the processing of pain perception and modulation. While much evidence has pointed to an increased activity of the ACC under chronic pain states, less is known about whether pain can be alleviated by inhibiting ACC neuronal activity. METHODS: The authors used pharmacologic, chemogenetic, and optogenetic approaches in concert with viral tracing technique to address this issue in a mouse model of bone cancer-induced mechanical hypersensitivity by intratibia implantation of osteolytic fibrosarcoma cells...
October 2016: Anesthesiology
https://www.readbyqxmd.com/read/27418580/cerebral-glucose-hypometabolism-in-tick-borne-encephalitis-a-pilot-study-in-10-patients
#16
Dietmann Anelia, Putzer Daniel, Beer Ronny, Helbok Raimund, Pfausler Bettina, Nordin Abdul Jalil, Virgolini Irene, Grams Astrid E, Schmutzhard Erich
BACKGROUND: Tick borne encephalitis (TBE) is an acute meningoencephalitis with or without myelitis caused by an RNA virus from the flavivirus family transmitted by Ixodes spp ticks. The neurotropic TBE virus infects preferentially large neurons in basal ganglia, anterior horns, medulla oblongata, purkinje cells and thalamus. Brain metabolic changes related to radiologic and clinical findings have not been described so far. METHODS: Here we describe the clinical course of 10 consecutive TBE patients with outcome assessment at discharge and after 12 month using a modified Rankin Scale...
July 11, 2016: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/27357294/pathologic-studies-of-fatal-encephalomyelitis-in-children-caused-by-enterovirus-71
#17
Jingjun Xing, Dan Liu, Shu Shen, Zhengyuan Su, Lin Zhang, Yijie Duan, Fang Tong, Yue Liang, Hualin Wang, Fei Deng, Zhihong Hu, Yiwu Zhou
OBJECTIVES: Enterovirus 71 (EV71) is the major pathogen of hand, foot, and mouth disease and can cause death; however, its pathogenesis remains elusive. METHODS: We performed a detailed systematic histopathologic examination and molecular studies on six autopsy cases of EV71 infection using H&E, immunohistochemistry, double immunofluorescence staining, and nested reverse transcription polymerase chain reaction. RESULTS: Characteristic features of acute encephalomyelitis were observed...
July 2016: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/27301520/brachial-diparesis-due-to-motor-neuronopathy-as-one-of-the-predominant-presenting-signs-of-occult-small-cell-lung-carcinoma
#18
Sefik Evren Erdener, Atay Vural, Cagri Mesut Temucin, Sevim Erdem Ozdamar, Gulay Nurlu, Kubilay Varli, Nese Dericioglu
Sensory neuronopathy is a well-established presentation in paraneoplastic neurological syndromes that is mostly associated with small cell lung cancer and anti-Hu antibodies. Motor neuronopathy, on the other hand, is an extremely rare observation in this syndrome. A 56-year-old man presented with asymmetric brachial diparesis and sensory ataxia. Electrophysiological studies revealed sensory ganglionopathy and progressive anterior horn degeneration in cervical segments. Small cell lung carcinoma with associated anti-Hu antibodies was later diagnosed...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27260405/neuron-specific-knock-down-of-smn1-causes-neuron-degeneration-and-death-through-an-apoptotic-mechanism
#19
Ivan Gallotta, Nadia Mazzarella, Alessandra Donato, Alessandro Esposito, Justin C Chaplin, Silvana Castro, Giuseppina Zampi, Giorgio S Battaglia, Massimo A Hilliard, Paolo Bazzicalupo, Elia Di Schiavi
Spinal muscular atrophy is a devastating disease that is characterized by degeneration and death of a specific subclass of motor neurons in the anterior horn of the spinal cord. Although the gene responsible, survival motor neuron 1 (SMN1), was identified 20 years ago, it has proven difficult to investigate its effects in vivo. Consequently, a number of key questions regarding the molecular and cellular functions of this molecule have remained unanswered. We developed a Caenorhabditis elegans model of smn-1 loss-of-function using a neuron-specific RNA interference strategy to knock-down smn-1 selectively in a subclass of motor neurons...
June 3, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/27256400/14-3-3-eta-isoform-colocalizes-tdp-43-on-the-coarse-granules-in-the-anterior-horn-cells-of-patients-with-sporadic-amyotrophic-lateral-sclerosis
#20
Takahiko Umahara, Toshiki Uchihara, Noriyuki Shibata, Ayako Nakamura, Haruo Hanyu
The immunolocalization of the 14-3-3 eta isoform in the anterior horn cells (AHCs) of patients with sporadic amyotrophic lateral sclerosis (ALS) and controls was examined. Compared with the immunolocalization of other 14-3-3 isoforms, the immunolocalization of the 14-3-3 eta isoform was either synaptic at the periphery of AHCs, spindle-shaped in neurites, or granular in the cytoplasm. By double labeling with phosphorylated (p-)TDP-43, the transactivation response DNA binding protein of 43kDa (TDP-43) demonstrated frequent colocalization of the 14-3-3 eta isoform in granular structures (90%) and spindle-shaped structures (85...
September 1, 2016: Brain Research
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