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https://www.readbyqxmd.com/read/29656576/repurposing-carbamazepine-for-the-treatment-of-amyotrophic-lateral-sclerosis-in-sod1-g93a-mouse-model
#1
Jing-Jing Zhang, Qin-Ming Zhou, Sheng Chen, Wei-Dong Le
AIMS: To investigate the effect and mechanisms of carbamazepine (CBZ) on the onset and progression of amyotrophic lateral sclerosis (ALS) in SOD1-G93A mouse model. METHODS: Starting from 64 days of age, SOD1-G93A mice were orally administered with CBZ at 200 mg/kg once daily until death. The disease onset and life span of SOD1-G93A mice were recorded. Motor neurons (MNs) in anterior horn of spinal cord were quantified by Nissl staining and SMI-32 immunostaining...
April 14, 2018: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/29627031/pregnancy-outcomes-in-women-with-spinal-muscular-atrophy-a-review
#2
REVIEW
Elena Abati, Stefania Corti
Spinal muscular atrophy (SMA) is an autosomal recessive disease characterized by muscle weakness and atrophy resulting from progressive degeneration and loss of the anterior horn cells in the spinal cord and brain stem nuclei. The onset of weakness ranges from prenatal age to young adulthood. Thus, many female patients reach fertile age and may consider getting pregnant. However, only little information is available about outcomes and complications of pregnancy in women with SMA. In this review, we compared different studies on the subject, then we analyzed outcomes in the different stages of the pregnancy (preconceptional period, embryonal period, fetal period, delivery and post partum), with a special focus on maternal and fetal complications, prematurity, mode of delivery, anesthesiological risk, respiratory function and influence of pregnancy on the disease course...
May 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29587636/the-attenuation-of-neurological-injury-from-the-use-of-simvastatin-after-spinal-cord-ischemia-reperfusion-injury-in-rats
#3
Jung-Hee Ryu, Jin-Woo Park, Jin-Young Hwang, Seong-Joo Park, Jin-Hee Kim, Hye-Min Sohn, Sung Hee Han
BACKGROUND: Spinal cord ischemic injury remains a serious complication of open surgical and endovascular aortic procedures. Simvastatin has been reported to be associated with neuroprotective effect after spinal cord ischemia-reperfusion (IR) injury. The aim of this study was to determine the therapeutic efficacy of starting simvastatin after spinal cord IR injury in a rat model. METHODS: In adult Sprague-Dawley rats, spinal cord ischemia was induced using a balloon-tipped catheter placed in the descending thoracic aorta...
March 27, 2018: BMC Anesthesiology
https://www.readbyqxmd.com/read/29560943/-spinal-mechanisms-of-neuroplasticity-induced-by-neuromidin-in-treatment-of-traumatic-neuropathies
#4
S A Zhivolupov, L S Onischenko, N A Rashidov, I N Samartsev, E V Jakovlev
AIM: To analyze the spinal mechanisms of neuroplastic changes induced by neuromidin (ipidacrine) in a model of traumatic axonotomy of the sciatic nerve in rats. MATERIAL AND METHODS: The histological study of the material from the anterior horn (lumbar portion of spinal cord) was performed in 6 rats using electron microscopy. RESULTS AND CONCLUSION: The comparative analysis of neuromorphological changes in rats of control and main groups revealed the neuroprotective and modifying effects of neuromidin on the spinal neuroplasticity...
2018: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/29541850/hirayama-disease
#5
David C Kieser, P J Cox, S C J Kieser
PURPOSE: Hirayama disease is an initially progressive disease caused by cervical neck flexion compressing the anterior horns of the lower cervical spinal cord. It is primarily seen in young males of Indian or Asian descent. With increasing dispersion of these populations this condition is increasingly being encountered internationally. This grand round reviews this rare but increasingly recognized condition. MATERIALS AND METHODS: We present a classic case of a young Indian male with progressive hand and forearm weakness...
March 14, 2018: European Spine Journal
https://www.readbyqxmd.com/read/29482893/enterovirus-d68-and-acute-flaccid-myelitis-evaluating-the-evidence-for-causality
#6
REVIEW
Kevin Messacar, Edwin J Asturias, Alison M Hixon, Coretta Van Leer-Buter, Hubert G M Niesters, Kenneth L Tyler, Mark J Abzug, Samuel R Dominguez
Increased circulation of enterovirus D68 in 2014 and 2016 temporally and geographically coincided with increases in cases of acute flaccid myelitis, an uncommon condition of paralysis due to lesions in the anterior horn of the spinal cord. The identification of enterovirus D68 in respiratory specimens from cases of acute flaccid myelitis worldwide further supports an association, yet the absence of direct virus isolation from affected tissues, infrequent detection in cerebrospinal fluid, and the absence, until recently, of an animal model has left the causal nature of the relationship unproven...
February 23, 2018: Lancet Infectious Diseases
https://www.readbyqxmd.com/read/29427106/the-neuropathology-of-spinocerebellar-ataxia-type-3-machado-joseph-disease
#7
Arnulf H Koeppen
Spinocerebellar ataxia type 3 (SCA-3)/Machado-Joseph disease (MJD), the most common autosomal dominant ataxia, affects many regions of the brain and spinal cord. Similar to SCA-1, SCA-2, SCA-6, SCA-7, and SCA-17, the mutation consists of a pathogenic translated cytosine-adenine-guanine (CAG) trinucleotide repeat expansion. Almost invariably, the substantia nigra and the dentate nucleus of the cerebellum bear the brunt of the disease, and these lesions account for the Parkinsonian and ataxic phenotypes. Lesions of motor nuclei in the brain stem cause the complex disturbance of ocular motility and weakness of the tongue...
2018: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29381509/aspirin-suppresses-neuronal-apoptosis-reduces-tissue-inflammation-and-restrains-astrocyte-activation-by-activating-the-nrf2-ho-1-signaling-pathway
#8
Wang Wei, Chen Shurui, Zhou Zipeng, Dai Hongliang, Wang Hongyu, Li Yuanlong, Zhou Kang, Shen Zhaoliang, Guo Yue, Liu Chang, Xifan Mei
The nuclear factor E2-related factor 2 (Nrf2)/antioxidant response element signaling pathway plays a substantial role in preventing oxidative stress-related diseases. Aspirin has been shown to exert several pharmacological effects by inducing the expression of the heme oxygenase-1 (HO-1) protein. However, the effects of aspirin on spinal cord injury (SCI) have rarely been studied. Therefore, we sought to investigate the neuroprotective effects of aspirin after SCI. We employed a spinal cord contusion model in Sprague-Dawley rats, and aspirin was administered intraperitoneally for 7 days...
May 2, 2018: Neuroreport
https://www.readbyqxmd.com/read/29374221/fingerprint-analysis-of-huolingshengji-formula-and-its-neuroprotective-effects-in-sod1-g93a-mouse-model-of-amyotrophic-lateral-sclerosis
#9
Qinming Zhou, Youjie Wang, Jingjing Zhang, Yaping Shao, Song Li, Yuan Wang, Huaibin Cai, Yi Feng, Weidong Le
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disease characterized by progressive loss of motor neurons. There are no definitive pathogenic mechanisms and effective treatments for ALS now. Traditional Chinese medicine (TCM) plays an important role in Chinese health care system. Huolingshengji Formula (HLSJ) is a TCM formula which is applied for treating flaccid syndrome. Our previous clinical study has indicated that HLSJ may have therapeutic effects in ALS patients. In the present study, we analyzed the chemical profile of HLSJ by the high-performance liquid chromatographic (HPLC) fingerprint analysis...
January 26, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29350170/atypical-lower-motor-neuron-disease-with-enlargement-of-nissl-substance-report-of-an-autopsy-case
#10
Masayuki Shintaku, Daita Kaneda, Kiyomitsu Oyanagi
The patient was an 81-year-old woman diagnosed with atypical motor neuron disease who died after a long clinical course (7.5 years without mechanical assistance of ventilation) characterized by lower motor neuron signs and symptoms. Upper motor neuron signs and cognitive impairment were not apparent. Autopsy demonstrated severe neuronal loss in the anterior horn of the spinal cord, and some of the remaining neurons showed enlargement of Nissl substance and apparent thickening of the nuclear envelopes. No Bunina bodies, skein-like inclusions, or structures immunoreactive for phosphorylated transactivation response DNA-binding protein 43 were found...
March 2018: Clinical Neuropathology
https://www.readbyqxmd.com/read/29286067/changes-in-microrna-expression-in-the-brachial-plexus-avulsion-model-of-neuropathic-pain
#11
Yuzhou Liu, Le Wang, Jie Lao, Xin Zhao
The present study aimed to perform microRNA (miRNA/miR) expression profiling of the thalamus (T), the anterior cingulate (AC), the dorsal horn of the spinal cord (DHSC) and the blood (B) in post‑complete brachial plexus avulsion (CBPA) pain model, and analyze biological functions. Neuropathic pain was induced in Sprague‑Dawley rats by CBPA. Animal behavioral tests were performed to differentiate the pain and control groups. DHSC, T, AC and B tissues were collected from the two groups for miRNA array analysis...
March 2018: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/29172006/two-cases-of-spinal-muscular-atrophy-type-ii-with-eosinophilic-oesophagitis
#12
Heidi R Fuller, Hannah K Shorrock, Thomas H Gillingwater, Anna Pigott, Victoria Smith, Richa Kulshrestha, Caroline S Sewry, Tracey A Willis
Although primarily characterised by loss of motor neurons from the anterior horn of spinal cord and muscle atrophy, spinal muscular atrophy (SMA) is now recognised as a multi-systemic disorder. Here, we report two SMA Type II patients with eosinophilic oesophagitis (EoE), a rare, chronic immune/antigen-mediated condition. One patient presented with dysphagia and poor weight gain, and the second patient had symptoms of gastro-oesophageal reflux (GOR) and poor weight gain. In both patients, macroscopic observations during gastroscopy indicated typical signs of EoE, which were verified during histological examination of oesophageal biopsies...
2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/29171910/mutant-huntingtin-protein-expression-and-blood-spinal-cord-barrier-dysfunction-in-huntington-disease
#13
Giacomo Sciacca, Francesca Cicchetti
OBJECTIVE: The aim of the study was to assess the distribution, frequency, and specific location of mutant huntingtin protein (mHTT) aggregates-the pathological hallmark of Huntington disease (HD)-within the various compartments of the spinal cord and their potential impact on the local vasculature and blood-spinal cord barrier (BSCB). METHODS: We performed a series of postmortem immunohistochemical and immunofluorescent stainings, as well as Western blot analyses, on cervical and lumbar sections of the spinal cord in patients diagnosed with HD (n = 11 of all grades of disease severity) along with sex- and age-matched healthy controls (n = 9)...
December 2017: Annals of Neurology
https://www.readbyqxmd.com/read/29167994/congenital-zika-virus-infection-a-neuropathological-review
#14
L Chimelli, E Avvad-Portari
BACKGROUND: A relationship between Zika virus (ZikV) infection in pregnancy and the occurrence of microcephaly was established during the Zika outbreak in Brazil (2015-2016). Neuropathological findings in congenital Zika syndrome helped to understand its pathogenetic mechanisms. RESULTS: The most relevant postmortem findings in the central nervous system (CNS) of fetuses and neonates infected with ZikV early in gestation are microcephaly with ex-vacuo ventriculomegaly and large head circumference associated with obstructive hydrocephalus due to severe midbrain and aqueduct distortion...
January 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29150766/spinobot-an-mri-guided-needle-positioning-system-for-spinal-cellular-therapeutics
#15
Alexander Squires, John N Oshinski, Nicholas M Boulis, Zion Tsz Ho Tse
The neurodegenerative disease amyotrophic lateral sclerosis (ALS) results in the death of motor neurons in voluntary muscles. There are no cures for ALS and few available treatments. In studies with small animal models, injection of cellular therapeutics into the anterior horn of the spinal cord has been shown to inhibit the progression of ALS. It was hypothesized that spinal injection could be made faster and less invasive with the aid of a robot. The robotic system presented-SpinoBot-uses MRI guidance to position a needle for percutaneous injection into the spinal cord...
November 17, 2017: Annals of Biomedical Engineering
https://www.readbyqxmd.com/read/29118923/ddx3-binding-with-ck1%C3%AE%C2%B5-was-closely-related-to-motor-neuron-degeneration-of-als-by-affecting-neurite-outgrowth
#16
Yanchun Chen, Qing Wang, Qiaozhen Wang, Huancai Liu, Fenghua Zhou, Yawen Zhang, Meng Yuan, Chunyan Zhao, Yingjun Guan, Xin Wang
Amyotrophic lateral sclerosis (ALS) is a chronic neurodegenerative disease characterized by progressive degeneration of motor neurons. The pathogenesis of ALS remains largely unknown. RNA helicase DDX3 is a multifunctional protein involved in several steps of gene expression. Casein kinase 1ε (CK1ε) is an important signal molecule of Wnt signaling pathway and is closely related to neurite growth. However, the roles of DDX3 and CK1ε in the pathogenesis of ALS remain unclear. In this study, we first investigated the expression of DDX3 and CK1ε in the spinal cord of SOD1-G93A ALS transgenic mice using RT-PCR, Western blot and immunohistochemical technique...
2017: American Journal of Translational Research
https://www.readbyqxmd.com/read/29062563/segmental-spinal-muscular-atrophy-localised-to-the-lower-limbs-first-case-from-oman
#17
Roshan Koul, Amna Al-Futaisi, Khalid Al-Thihli, Zandre Bruwer, Patrick Scott
Spinal muscular atrophy (SMA) is a genetic lower motor neuron disease. It usually involves all of the skeletal muscles innervated by the anterior horn cells of the spinal cord. In rare cases, there is also localised involvement of the spinal cord. We report a 10-year-old boy who presented to the Sultan Qaboos University Hospital, Muscat, Oman, in 2015 with muscle weakness restricted to the lower limbs. The presence of a homozygous deletion within the survival of motor neuron 1 gene confirmed the diagnosis of SMA...
August 2017: Sultan Qaboos University Medical Journal
https://www.readbyqxmd.com/read/29053833/clinical-pathological-and-functional-characterization-of-riboflavin-responsive-neuropathy
#18
Andreea Manole, Zane Jaunmuktane, Iain Hargreaves, Marthe H R Ludtmann, Vincenzo Salpietro, Oscar D Bello, Simon Pope, Amelie Pandraud, Alejandro Horga, Renata S Scalco, Abi Li, Balasubramaniem Ashokkumar, Charles M Lourenço, Simon Heales, Rita Horvath, Patrick F Chinnery, Camilo Toro, Andrew B Singleton, Thomas S Jacques, Andrey Y Abramov, Francesco Muntoni, Michael G Hanna, Mary M Reilly, Tamas Revesz, Dimitri M Kullmann, James E C Jepson, Henry Houlden
Brown-Vialetto-Van Laere syndrome represents a phenotypic spectrum of motor, sensory, and cranial nerve neuropathy, often with ataxia, optic atrophy and respiratory problems leading to ventilator-dependence. Loss-of-function mutations in two riboflavin transporter genes, SLC52A2 and SLC52A3, have recently been linked to Brown-Vialetto-Van Laere syndrome. However, the genetic frequency, neuropathology and downstream consequences of riboflavin transporter mutations are unclear. By screening a large cohort of 132 patients with early-onset severe sensory, motor and cranial nerve neuropathy we confirmed the strong genetic link between riboflavin transporter mutations and Brown-Vialetto-Van Laere syndrome, identifying 22 pathogenic mutations in SLC52A2 and SLC52A3, 14 of which were novel...
November 1, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28983837/treatment-advances-in-spinal-muscular-atrophy
#19
REVIEW
Diana Bharucha-Goebel, Petra Kaufmann
PURPOSE OF REVIEW: Spinal muscular atrophy (SMA) is a genetic disorder of motor neurons in the anterior horns of the spinal cord and brainstem that results in muscle atrophy and weakness. SMA is an autosomal recessive disease linked to deletions of the SMN1 gene on chromosome 5q. Humans have a duplicate gene (SMN2) whose product can mitigate disease severity, leading to the variability in severity and age of onset of disease, and is therefore a target for drug development. RECENT FINDINGS: Advances in preclinical and clinical trials have paved the way for novel therapeutic options for SMA patients, including many currently in clinical trials...
October 6, 2017: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/28966654/a-progressive-compression-model-of-thoracic-spinal-cord-injury-in-mice-function-assessment-and-pathological-changes-in-spinal-cord
#20
Guo-Dong Sun, Yan Chen, Zhi-Gang Zhou, Shu-Xian Yang, Cheng Zhong, Zhi-Zhong Li
Non-traumatic injury accounts for approximately half of clinical spinal cord injury, including chronic spinal cord compression. However, previous rodent spinal cord compression models are mainly designed for rats, few are available for mice. Our aim is to develop a thoracic progressive compression mice model of spinal cord injury. In this study, adult wild-type C57BL/6 mice were divided into two groups: in the surgery group, a screw was inserted at T9 lamina to compress the spinal cord, and the compression was increased by turning it further into the canal (0...
August 2017: Neural Regeneration Research
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