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https://www.readbyqxmd.com/read/28449459/endovascular-repair-of-residual-intimal-tear-or-distal-new-entry-after-frozen-elephant-trunk-for-type-a-aortic-dissection
#1
Xu-Dong Pan, Bin Li, Wei-Guo Ma, Jun Zheng, Yong-Min Liu, Jun-Ming Zhu, Lian-Jun Huang, Li-Zhong Sun
BACKGROUND: In patients with type A dissection, residual dissection and new distal entry tears following the frozen elephant trunk (FET) procedure adversely affect long-term prognosis. Management include open and endovascular repair, while clinical experience is limited. We evaluate the efficacy of thoracic endovascular aortic repair (TEVAR) in management of residual intimal tear or distal new entry tear following FET in patients with type A aortic dissection (TAAD). METHODS: Between May 2003 and April 2013, we performed FET and total arch replacement for 1,003 patients with TAAD...
March 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28444460/hydrocephalus-a-rare-initial-manifestation-of-sporadic-intramedullary-hemangioblastoma-intramedullary-hemangioblastoma-presenting-as-hydrocephalus
#2
Barbara Albuquerque Morais, Daniel Dante Cardeal, Renan Ribeiro E Ribeiro, Fernando Pereira Frassetto, Fernanda Goncalves Andrade, Hamilton Matushita, Manoel Jacobsen Teixeira
BACKGROUND: Intramedullary hemangioblastomas are rare benign vascular tumors, infrequent in pediatric patients. Clinical symptoms vary according to the age of presentation, tumor size, location, and concomitant syringomyelia. This is the second reported case of hemangioblastoma presenting with acute hydrocephalus. CASE PRESENTATION: A 3-month-old infant with acute hydrocephalus was asymptomatic after a ventriculoperitoneal shunt was placed. She returned 3 months later with irritability, acute paraplegia, and respiratory distress...
April 25, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28435564/acute-paraplegia-in-a-toddler-a-diagnostic-journey-compounding-the-challenge-in-management-a-case-report
#3
J H Goh, M Fazir, N A Zainal-Abidin, D Amir, M Singh
Spinal tuberculosis is not common in the paediatric age group. Initial clinical features are often vague and non specific until the disease progresses to later stages. We highlight the diagnostic difficulties and management challenges of a complicated extradural tuberculoma with neurological deficits in a very young girl.
July 2016: Malaysian Orthopaedic Journal
https://www.readbyqxmd.com/read/28433727/superficial-swab-versus-deep-tissue-biopsy-for-the-microbiological-diagnosis-of-local-infection-in-advanced-stage-pressure-ulcers-of-spinal-cord-injured-patients-a-prospective-study
#4
Sara Tedeschi, Luca Negosanti, Rossella Sgarzani, Filippo Trapani, Salvatore Pignanelli, Micaela Battilana, Rita Capirossi, Donatella Brillanti Ventura, Maddalena Giannella, Michele Bartoletti, Fabio Tumietto, Francesco Cristini, Pierluigi Viale
OBJECTIVE: To assess the predictive value of superficial ulcer swab culture to make a microbiological diagnosis of deep wound infections in spinal cord injury (SCI) patients with advanced-stage pressure ulcers (PU). METHODS: From July 2011 to February 2014, we performed a prospective, single-centre study on adult SCI patients undergoing scheduled surgical debridement and reconstruction for advanced-stage PU, at Montecatone Rehabilitation Institute, a 150-bed hospital dedicated to SCI care...
April 19, 2017: Clinical Microbiology and Infection
https://www.readbyqxmd.com/read/28428749/deep-brain-stimulation-improves-the-symptoms-and-sensory-signs-of-persistent-central-neuropathic-pain-from-spinal-cord-injury-a-case-report
#5
Walter J Jermakowicz, Ian D Hentall, Jonathan R Jagid, Corneliu C Luca, James Adcock, Alberto Martinez-Arizala, Eva Widerström-Noga
Central neuropathic pain (CNP) is a significant problem after spinal cord injury (SCI). Pharmacological and non-pharmacological approaches may reduce the severity, but relief is rarely substantial. While deep brain stimulation (DBS) has been used to treat various chronic pain types, the technique has rarely been used to attenuate CNP after SCI. Here we present the case of a 54-year-old female with incomplete paraplegia who had severe CNP in the lower limbs and buttock areas since her injury 30 years prior. She was treated with bilateral DBS of the midbrain periaqueductal gray (PAG)...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28428525/-total-arch-replacement-using-manufactured-frozen-elephant-trunk-after-acute-type-a-dissection-repair
#6
Makoto Hibino, Masato Mutsuga, Hideki Oshima, Yuji Narita, Tomonobu Abe, Kazuro Fujimoto, Yoshiyuki Tokuda, Akihiko Usui
The purpose of this study is to assess the result of total arch replacement(TAR) using manufactured frozen elephant trunk(FET) for chronic aortic dissection after initial repair including the effect of aortic remodeling by the FET. Between 2003 and 2015, we performed 11 TAR using manufactured FET. Initial repairs before were 9 ascending aortic replacements and 2 Bentall operations. The entry of residual dissection was located at arch in 7 and at distal anastomosis site in 4. There was no hospital death. The operative complication included 2 surgical site infection, 1 interstitial pneumonia and 1 paraplegia with almost full recovery...
April 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28422159/spastic-paraplegia-linked-phospholipase-papla1-is-necessary-for-development-reproduction-and-energy-metabolism-in-drosophila
#7
Martina Gáliková, Peter Klepsatel, Judith Münch, Ronald P Kühnlein
The human PAPLA1 phospholipase family is associated with hereditary spastic paraplegia (HSP), a neurodegenerative syndrome characterized by progressive spasticity and weakness of the lower limbs. Taking advantage of a new Drosophila PAPLA1 mutant, we describe here novel functions of this phospholipase family in fly development, reproduction, and energy metabolism. Loss of Drosophila PAPLA1 reduces egg hatchability, pre-adult viability, developmental speed, and impairs reproductive functions of both males and females...
April 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28419689/kinetic-and-structural-changes-in-hsmtphers-induced-by-pathogenic-mutations-in-human-fars2
#8
Ekaterine Kartvelishvili, Dmitry Tworowski, Hilary Vernon, Nina Moor, Jing Wang, Lee-Jun Wong, Zofia Chrzanowska-Lightowlers, Mark Safro
Mutations in the mitochondrial aminoacyl-tRNA synthetases (mtaaRSs) can cause profound clinical presentations, and have manifested as diseases with very selective tissue specificity. To date most of the mtaaRS mutations could be phenotypically recognized, such that clinicians could identify the affected mtaaRS from the symptoms alone. Among the recently reported pathogenic variants are point mutations in FARS2 gene, encoding the human mitochondrial PheRS. Patient symptoms range from spastic paraplegia to fatal infantile Alpers encephalopathy...
April 17, 2017: Protein Science: a Publication of the Protein Society
https://www.readbyqxmd.com/read/28415681/a-reproducible-model-of-intramedullary-spinal-cord-tumor-in-rats-bearing-rg2-cells
#9
Yuandong Zhuang, Wei Zhao, Weiqiang Zhang, Hao Wei, Xinming Huang, Gangfeng Cai, Chaofeng Fu, Chunhua Wang, Rui Wang, Songsheng Shi, Weizhong Yang, Chunmei Chen
Intramedullary spinal cord tumors (IMSCTs) are lethal diseases to many patients. The lack of adequate animal model has hampered the development of novel treatments. In the current study, a rodent intramedullary glioma model is established to study IMSCT progression. Fischer 344 rats received a intramedullary implantation of RG2 glioma cells. The neurological state of each rat was evaluated on daily basis using the Basso, Beattie and Bresnahan (BBB) scale. Rats implanted with RG2 cells developed significant hind limb paraplegia 20 days after implantation...
March 9, 2017: Oncotarget
https://www.readbyqxmd.com/read/28413272/an-analysis-of-perioperative-adverse-neurological-events-associated-with-anesthetic-management-at-a-tertiary-care-center-of-a-developing-country
#10
Fauzia Anis Khan, Sobia Khan, Gauhar Afshan
BACKGROUND AND AIMS: Existing literature on neurological complications related to anesthesia is reported from affluent countries but the trends may vary in less affluent countries. MATERIAL AND METHODS: The objective was to find the associated factors contributing to neurological adverse events occurring within 48 h of anesthesia and surgery. The existing departmental morbidity and mortality database was reviewed from 1992 to 2012 for major adverse neurological events...
January 2017: Journal of Anaesthesiology, Clinical Pharmacology
https://www.readbyqxmd.com/read/28409289/expert-s-comment-concerning-grand-rounds-case-entitled-acute-complete-paraplegia-of-a-8-year-old-girl-caused-by-spinal-cord-infarction-following-minor-trauma-complicated-with-longitudinal-signal-change-of-spinal-cord-by-k-nagata-et-al-eur-spine-j-2017-doi-10
#11
https://www.readbyqxmd.com/read/28407703/determination-of-the-effect-of-diameter-of-the-sac-on-prognosis-in-64-cases-operated-for-meningomyelocele
#12
Metehan Eseoğlu, Ahmet Eroğlu, Serkan Kemer, Mehmet Arslan
OBJECTIVE: To examine the effect of meningomyelocele sac size on prognosis by retrospective review of 64 cases operated for meningomyelocele between January 2009 and December 2012. METHODS: We evaluated newborn babies operated for meningomyelocele by retrospectively reviewing their files for head circumference, location and with of the defect, accompanying anomalies, treatments administered, drugs that mother used during pregnancy. Based on the defect size, 3 patient groups were created as 0-24 cm(2) (group I), 25-39 cm(2) (group II), and 40 cm(2) and above (group III)...
March 2017: Korean Journal of Spine
https://www.readbyqxmd.com/read/28407430/chronic-urinary-tract-infections-in-patients-with-spinal-cord-lesions-biofilm-infection-with-need-for-long-term-antibiotic-treatment
#13
REVIEW
Nete Tofte, Alex C Y Nielsen, Hannah Trøstrup, Christine B Andersen, Michael Von Linstow, Birgitte Hansen, Fin Biering-Sørensen, Niels Høiby, Claus Moser
Patients suffering from spinal cord injuries resulting in complete or incomplete paraplegia or tetraplegia are highly disposed to frequent, recurrent or even chronic urinary tract infections (UTIs). The reason for the increased risk of acquiring UTIs is multifactorial, including reduced sensation of classical UTI symptoms, incomplete bladder emptying, frequent catheterizations or chronic urinary tract catheters. Biofilms in relation to UTIs have been shown both on catheters, on concrements or as intracellular bacterial communities (IBCs)...
April 2017: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
https://www.readbyqxmd.com/read/28398512/mutant-spastin-proteins-promote-deficits-in-axonal-transport-through-an-isoform-specific-mechanism-involving-casein-kinase-2-activation
#14
Lanfranco Leo, Carina Weissmann, Matthew Burns, Minsu Kang, Yuyu Song, Liang Qiang, Scott T Brady, Peter W Baas, Gerardo Morfini
Mutations of various genes cause hereditary spastic paraplegia (HSP), a neurological disease involving dying-back degeneration of upper motor neurons. From these, mutations in the SPAST gene encoding the microtubule-severing protein spastin account for most HSP cases. Cumulative genetic and experimental evidence suggests that alterations in various intracellular trafficking events, including fast axonal transport (FAT), may contribute to HSP pathogenesis. However, the mechanisms linking SPAST mutations to such deficits remain largely unknown...
April 7, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28396731/clinical-features-and-genotype-phenotype-correlation-analysis-in-patients-with-atl1-mutations-a-literature-reanalysis
#15
Guo-Hua Zhao, Xiao-Min Liu
BACKGROUND: The hereditary spastic paraplegias (HSPs) are a group of clinically and genetically heterogeneous disorders. Approximately 10% of the autosomal dominant (AD) HSPs (ADHSPs) have the spastic paraplegia 3A (SPG3A) genotype which is caused by ATL1 gene mutations. Currently there are more than 60 reported ATL1 gene mutations and the genotype-phenotype correlation remains unclear. The study aims to investigate the genotype-phenotype correlation in SPG3A patients. METHODS: We performed a reanalysis of the clinical features and genotype-phenotype correlations in 51 reported studies exhibiting an ATL1 gene mutation...
2017: Translational Neurodegeneration
https://www.readbyqxmd.com/read/28395872/outcomes-of-antegrade-stent-graft-deployment-during-hybrid-aortic-arch-repair
#16
Seyed Hossein Aalaei-Andabili, Salvatore Scali, Charles Klodell, Teng Lee, Philip Hess, Tomas Martin, Adam Beck, Robert Feezor, Mahmoud Alhussaini, George Arnaoutakis, Thomas Beaver
BACKGROUND: Complex aortic arch disease can be a formidable challenge and is often treated with a two-stage elephant trunk technique. We examined our experience with hybrid arch repair with combined zone 0 stent graft deployment. METHODS: A retrospective review was conducted of all patients who underwent type 2 hybrid arch replacement and zone 0 antegrade endovascular stent graft deployments at a single university center from June 2010 to August 2015. RESULTS: The review included 48 patients, 25 (52%) elective and 23 (48%) nonelective, with a mean ± SD age of 64 ± 11 years...
April 7, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28392907/protective-effect-of-crocus-sativus-l-saffron-extract-on-spinal-cord-ischemia-reperfusion-injury-in-rats
#17
Gholam Hossein Farjah, Shadi Salehi, Mohammad Hasan Ansari, Bagher Pourheidar
OBJECTIVES: Ischemia/reperfusion (I/R) injury of spinal cord is leading to the paraplegia observed. In this study, we investigated the protective effect of the saffron extract on spinal cord I/R injury. MATERIALS AND METHODS: Thirty five male Sprague-Dawley rats were divided into 5 groups: intact, sham surgery, normal saline (NS), low dose saffron aqua extract, high dose saffron aqua extract. RESULTS: The mean motor deficit index (MDI) scores were significantly lower in the saffron extract groups than in the NS group at 48 hr after spinal cord ischemia (P<0...
March 2017: Iranian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/28392418/spastin-regulates-vamp7-containing-vesicles-trafficking-in-cortical-neurons
#18
C Plaud, V Joshi, M Marinello, D Pastré, T Galli, P A Curmi, A Burgo
Alteration of axonal transport has emerged as a common precipitating factor in several neurodegenerative disorders including Human Spastic Paraplegia (HSP). Mutations of the SPAST (SPG4) gene coding for the spastin protein account for 40% of all autosomal dominant uncomplicated HSP. By cleaving microtubules, spastin regulates several cellular processes depending on microtubule dynamics including intracellular membrane trafficking. Axonal transport is fundamental for the viability of motor neurons which often have very long axons and thus require efficient communication between the cell body and its periphery...
April 6, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28389505/onabotulinumtoxina-injections-in-detrusor-facilitate-self-catheterisation-in-a-patient-with-paraplegia-and-bladder-outlet-dyssynergia
#19
François Hervé, Annick Viaene, Karel Everaert
This case report describes the case of improvement of clean intermittent catheterisation procedures after each intradetrusor administration of onabotulinumtoxinA in a 45-year-old man with L1 paraplegia with neurogenic detrusor overactivity and bladder outlet dyssynergia.On three occasions, improvement on clean intermittent catheterisation procedures appeared 10 to 14 days after intradetrusor injections of onabotulinumtoxinA and lasted for 9 months. We hypothesise a possible influence of intravesical injections of onabotulinumtoxinA on the storage reflex...
April 6, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28389476/defects-in-er-endosome-contacts-impact-lysosome-function-in-hereditary-spastic-paraplegia
#20
Rachel Allison, James R Edgar, Guy Pearson, Tania Rizo, Timothy Newton, Sven Günther, Fiamma Berner, Jennifer Hague, James W Connell, Jürgen Winkler, Jennifer Lippincott-Schwartz, Christian Beetz, Beate Winner, Evan Reid
Contacts between endosomes and the endoplasmic reticulum (ER) promote endosomal tubule fission, but the mechanisms involved and consequences of tubule fission failure are incompletely understood. We found that interaction between the microtubule-severing enzyme spastin and the ESCRT protein IST1 at ER-endosome contacts drives endosomal tubule fission. Failure of fission caused defective sorting of mannose 6-phosphate receptor, with consequently disrupted lysosomal enzyme trafficking and abnormal lysosomal morphology, including in mouse primary neurons and human stem cell-derived neurons...
April 7, 2017: Journal of Cell Biology
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