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https://www.readbyqxmd.com/read/28983837/treatment-advances-in-spinal-muscular-atrophy
#1
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
#2
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
https://www.readbyqxmd.com/read/28926131/region-specific-impairment-of-the-cervical-spinal-cord-sc-in-amyotrophic-lateral-sclerosis-a-preliminary-study-using-sc-templates-and-quantitative-mri-diffusion-tensor-imaging-inhomogeneous-magnetization-transfer
#3
Henitsoa Rasoanandrianina, Aude-Marie Grapperon, Manuel Taso, Olivier M Girard, Guillaume Duhamel, Maxime Guye, Jean-Philippe Ranjeva, Shahram Attarian, Annie Verschueren, Virginie Callot
In this preliminary study, our objective was to investigate the potential of high-resolution anatomical imaging, diffusion tensor imaging (DTI) and conventional/inhomogeneous magnetization transfer imaging [magnetization transfer (MT)/inhomogeneous magnetization transfer (ihMT)] at 3 T, analyzed with template-extracted regions of interest, to measure the atrophy and structural changes of white (WM) and gray (GM) matter spinal cord (SC) occurring in patients with amyotrophic lateral sclerosis (ALS). Ten patients with ALS and 20 age-matched healthy controls were recruited...
September 19, 2017: NMR in Biomedicine
https://www.readbyqxmd.com/read/28895473/neuroinflammation-in-amyotrophic-lateral-sclerosis-role-of-redox-dys-regulation
#4
Nadia D'Ambrosi, Mauro Cozzolino, Maria Teresa Carrì
SIGNIFICANCE: Amyotrophic lateral sclerosis (ALS) is due to degeneration of upper and lower motor neurons in the anterior horn of the spinal cord and in the motor cortex. Mechanisms leading to motor neuron death are complex and currently the disease is untreatable. Recent Advances: Work in genetic models of ALS indicates that an imbalance in the cross talk that physiologically exists between motor neurons and the surrounding cells is eventually detrimental to motor neurons. In particular, the cascade of events collectively known as neuroinflammation and mainly characterized by a reactive phenotype of astrocytes and microglia, moderate infiltration of peripheral immune cells, and elevated levels of inflammatory mediators has been consistently observed in motor regions of the central nervous system (CNS) in sporadic and familial ALS, constituting a hallmark of the disease...
October 16, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/28867325/neuroprotective-effects-of-valproic-acid-in-a-rat-model-of-cauda-equina-injury
#5
Qing-Jie Kong, Yuan Wang, Yang Liu, Jing-Chuan Sun, Xi-Ming Xu, Xiao-Fei Sun, Jian-Gang Shi
BACKGROUND: Histone deacetylase inhibitors (HDACi), including valproic acid (VPA), are promising therapeutic interventions in neurological disorders and play an important role in synaptic activity and neuronal function. MATERIAL AND METHODS: A total of 30 rats were randomly allocated to three groups: sham, control, and VPA. The rats in the VPA and control groups received laminectomy at the L4 level of the vertebrae and silicone gel implantation into the epdidural spaces L5 and L6...
August 31, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28855045/acute-flaccid-myelitis-in-german-children-in-2016-the-return-of-polio
#6
Johannes Hübner, Bernd Kruse, Hans-Jürgen Christen, Jürgen Weidenmann, Viktoria Weiner, Jan-Christoph Schöne-Bake, Anna Eichinger, Sabine Diedrich, Wolfgang Müller-Felber
BACKGROUND: Although poliomyelitis has almost been eradicated worldwide, cases of a polio-like disease with asymmetrical flaccid paralysis of variable severity have been seen repeatedly in recent years. METHODS: Data were collected on children treated in hospitals in the German federal states of Bavaria and Lower Saxony in 2016. The frequency of disease across Germany was estimated on the basis of voluntary reporting to the Robert Koch Institute. 16 cases were registered there for the entire year 2016...
August 21, 2017: Deutsches Ärzteblatt International
https://www.readbyqxmd.com/read/28853070/activation-of-microglyocytes-in-the-anterior-horns-of-rat-spinal-cord-after-administration-of-bacterial-lipopolysaccharide
#7
E A Kolos, D E Korzhevskii
We studied the reaction of the microglia of the anterior horns of the rat spinal cord to intraperitoneal administration of bacterial LPS. Immunohistochemical analysis showed that acute systemic inflammation leads to activation of more than half of microglial cells as soon as in 24 h after LPS injection, while the total number of microglial cells does not change significantly. It was hypothesized that activated microglial cells are involved in the reorganization of synaptic connections, but do not have a neurotoxic effect...
August 29, 2017: Bulletin of Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28832294/-acute-flaccid-myelitis-after-a-respiratory-tract-infection-first-dutch-case-related-to-enterovirus-type-d68-infection
#8
J Helfferich, E M Kingma, L C Meiners, E H Schölvinck, H D Mulder, O F Brouwer
BACKGROUND: Acute flaccid myelitis (AFM) is a relatively rare disorder affecting the anterior horn of the spinal cord and brain stem. It is characterised by rapid progressive weakness of the limbs and respiratory muscles, often combined with cranial nerve dysfunction. This used to be seen in infections with the polio virus, but in recent years, AFM has been mainly associated with enterovirus D68 infection. CASE DESCRIPTION: A boy of nearly 4 years-old developed rapidly progressive weakness and respiratory failure after an upper airway infection...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28829151/validation-of-an-immunohistochemistry-assay-for-detection-of-cd155-the-poliovirus-receptor-in-malignant-gliomas
#9
Vidyalakshmi Chandramohan, Jeffrey D Bryant, Hailan Piao, Stephen T Keir, Eric S Lipp, Michaela Lefaivre, Kathryn Perkinson, Darell D Bigner, Matthias Gromeier, Roger E McLendon
CONTEXT: - The oncolytic polio-rhinovirus recombinant (PVSRIPO) has demonstrated promise in currently ongoing phase I/II clinical trials against recurrent glioblastoma and was granted breakthrough therapy designation by the Food and Drug Administration/Center for Biologics Evaluation and Research. A reliable clinical assay to document expression of the poliovirus receptor, CD155, in routinely available patient tumor samples is needed for continued clinical development of PVSRIPO oncolytic immunotherapy in primary brain tumors and beyond...
August 22, 2017: Archives of Pathology & Laboratory Medicine
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
#10
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
#11
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
#12
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
#13
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...
August 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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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