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https://www.readbyqxmd.com/read/29783013/multiple-hereditary-exostoses-with-tetraparesis-due-to-cervical-spine-osteochondroma
#1
Ali Akhaddar, Mohamed Zyani, Issam Rharrassi
This is a case report about a 34-year-old man with hereditary multiple exostoses presented with gradual tetraparesis. Neuroimaging evaluation revealed an important posterior spinal cord compression by a C3 bony formation. Following posterior microsurgical decompression, the patient recovered nearly completely. Histopathological examination of the intraspinal lesion confirmed the diagnosis of benign osteochondroma. The patient returned to a normal life without evidence of recurrence at 2-year follow-up.
May 18, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29779902/molecular-genetics-and-metabolism-special-edition-diagnosis-diagnosis-and-prognosis-of-mucopolysaccharidosis-iva
#2
REVIEW
Hira Peracha, Kazuki Sawamoto, Lauren Averill, Heidi Kecskemethy, Mary Theroux, Mihir Thacker, Kyoko Nagao, Christian Pizarro, William Mackenzie, Hironori Kobayashi, Seiji Yamaguchi, Yasuyuki Suzuki, Kenji Orii, Tadao Orii, Toshiyuki Fukao, Shunji Tomatsu
Mucopolysaccharidosis IVA (MPS IVA, Morquio A syndrome) is an autosomal recessive disorder caused by the deficiency of N-acetylgalactosamine-6-sulfate sulfatase. Deficiency of this enzyme leads to the accumulation of specific glycosaminoglycans (GAGs), chondroitin-6-sulfate (C6S) and keratan sulfate (KS), which are mainly synthesized in the cartilage. Therefore, the substrates are stored primarily in the cartilage and its extracellular matrix (ECM), leading to a direct impact on bone development and successive systemic skeletal spondylepiphyseal dysplasia...
May 15, 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29775143/testing-the-effectiveness-and-the-contribution-of-experimental-supercharge-reversed-end-to-side-nerve-transfer
#3
Mustafa Nadi, Sudheesh Ramachandran, Abir Islam, Joanne Forden, Gui Fang Guo, Rajiv Midha
OBJECTIVE Supercharge end-to-side (SETS) transfer, also referred to as reverse end-to-side transfer, distal to severe nerve compression neuropathy or in-continuity nerve injury is gaining clinical popularity despite questions about its effectiveness. Here, the authors examined SETS distal to experimental neuroma in-continuity (NIC) injuries for efficacy in enhancing neuronal regeneration and functional outcome, and, for the first time, they definitively evaluated the degree of contribution of the native and donor motor neuron pools...
May 18, 2018: Journal of Neurosurgery
https://www.readbyqxmd.com/read/29774200/thickening-ligamentum-flavum-mimicking-tumor-in-the-epidural-space-of-the-cervical-spine
#4
Sung Hyun Bae, Dong Wuk Son, O Ik Kwon, Su Hun Lee, Jun Seok Lee, Geun Sung Song
In patients with tumors and spinal cord lesions, inflammation and tissue infection can result in mass effect detection on imaging. As a result, surgical biopsy procedures are often performed on the lesions. We report a rare case in which the thickening ligamentum flavum (LF) appeared to be a tumor in the epidural space of the cervical spine based on imaging findings. A 52-year-old man visited our outpatient clinic with severe shoulder pain and radicular pain in his right arm that had developed gradually after a traffic accident two months earlier...
April 2018: Korean Journal of Neurotrauma
https://www.readbyqxmd.com/read/29774198/a-rare-cause-of-thoracic-spinal-cord-compression-by-multiple-large-tarlov-cysts
#5
Ahmed-Salem Kleib, Sidi-Mohamed Salihy, Hussein Hamdi, Romain Carron, Outouma Soumaré
Spinal extradural arachnoid cyst (SEAC) is a rare cause of spinal cord compression. Bifocal location of thoracic and sacral SEACs is rarely reported in the literature. We report a case of thoracic spinal cord compression by SEAC associated with asymptomatic multiple sacral Tarlov cysts (TC). The surgical management and postoperative outcome of the patient are discussed. A 34-year-old woman was referred to the hospital for acute thoracic pain with a history of chronic long-standing back pain. She complained of walking difficulties...
April 2018: Korean Journal of Neurotrauma
https://www.readbyqxmd.com/read/29772841/the-effect-of-human-mesenchymal-stem-cells-derived-from-wharton-s-jelly-in-spinal-cord-injury-treatment-is-dose-dependent-and-can-be-facilitated-by-repeated-application
#6
Petr Krupa, Irena Vackova, Jiri Ruzicka, Kristyna Zaviskova, Jana Dubisova, Zuzana Koci, Karolina Turnovcova, Lucia Machova Urdzikova, Sarka Kubinova, Svatopluk Rehak, Pavla Jendelova
Human mesenchymal stem cells derived from Wharton's jelly (WJ-MSCs) were used for the treatment of the ischemic-compression model of spinal cord injury in rats. To assess the effectivity of the treatment, different dosages (0.5 or 1.5 million cells) and repeated applications were compared. Cells or saline were applied intrathecally by lumbar puncture for one week only, or in three consecutive weeks after injury. Rats were assessed for locomotor skills (BBB, rotarod, flat beam) for 9 weeks. Spinal cord tissue was morphometrically analyzed for axonal sprouting, sparing of gray and white matter and astrogliosis...
May 17, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29770224/hybrid-surgery-radiosurgery-therapy-for-metastatic-epidural-spinal-cord-compression-a-prospective-evaluation-using-patient-reported-outcomes
#7
Ori Barzilai, Mary-Kate Amato, Lily McLaughlin, Anne S Reiner, Shahiba Q Ogilvie, Eric Lis, Yoshiya Yamada, Mark H Bilsky, Ilya Laufer
Background: Patient-reported outcomes (PRO) represent an important measure of cancer therapy effect. For patients with metastatic epidural spinal cord compression (MESCC), hybrid therapy using separation surgery and stereotactic radiosurgery preserves neurologic function and provides tumor control. There is currently a paucity of data reporting PRO after such combined modality therapy for MESCC. Delineation of hybrid surgery-radiosurgery therapy effect on PRO validates the hybrid approach as an effective therapy resulting in meaningful symptom relief...
May 2018: Neuro-oncology Practice
https://www.readbyqxmd.com/read/29764185/management-of-metastatic-spinal-cord-compression-among-veterans-health-administration-radiation-oncologists
#8
Ruchika Gutt, Sheeta Malhotra, Shruti Jolly, Drew Moghanaki, Alice V Cheuk, Helen Fosmire, Maria Kelly, Lori Hoffman-Hogg, Stephen Lutz, Mitchell Anscher, George Dawson
BACKGROUND: Optimal management of metastatic spinal cord compression (MSCC) improves functional outcomes in patients with metastatic disease. This survey study evaluated management of MSCC by Veterans Health Administration (VHA) radiation oncologists (ROs), to determine whether management of MSCC correlates with American College of Radiology (ACR) guidelines, and to compare times to initiation of treatment between surgery and radiotherapy (RT). METHODS: Surveys emailed to 79 VHA ROs included questions on steroid use, surgical care, palliative care, fractionation of irradiation, re-irradiation, and management of common MSCC case scenarios...
April 2018: Annals of Palliative Medicine
https://www.readbyqxmd.com/read/29755804/sapho-syndrome-masquerading-as-metastatic-breast-cancer
#9
Devin Malik, Richa Handa, Andrew Petraszko, Sheela Tejwani
SAPHO syndrome is a rare clinical entity composed of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO). We describe a case of SAPHO syndrome masquerading as metastatic breast cancer in a patient with localized breast cancer who presented with cord compression. There was no pathologic evidence of metastatic cancer; however, a bone scan indicated osseous involvement. After multidisciplinary review of images and with additional findings of pustulosis and acne, a clinical diagnosis of SAPHO was made...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29753381/denosumab-associated-severe-hypocalcemia-in-a-patient-with-chronic-kidney-disease
#10
REVIEW
Sohail Abdul Salim, Lakshmi Ramachandran Nair, Litty Thomas, Vishnu Garla, Venkataraman Palabindala, Mohit Agarwal, Tibor Fülöp
Denosumab is a monoclonal antibody directed against the receptor activator of nuclear factor kappa B ligand (RANKL). Denosumab has been shown to reduce the risk of skeletal-related events, including spinal cord compression, pathologic fracture and hypercalcemia of malignancy in patients with bone metastases. Hypocalcemia is a known side effect of denosumab, occurring in an estimated 8-14% of the patients. Here, we present an asymptomatic patient with stage-5 chronic kidney disease and severe hypocalcemia who had received denosumab 1 month earlier...
May 2018: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29753078/isolated-pediatric-intramedullary-schwannoma-case-report-and-review-of-the-literature
#11
Alessandro Landi, Giovanni Grasso, Fabrizio Gregori, Giorgia Iacopino, Andrea Ruggeri, Roberto Delfini
BACKGROUND: Intramedullary (IM) schwannomas are rare entities representing the 0.3-1% of intramedullary tumors and 1.1% of spinal schwannomas. Beside many theories proposed, their rare occurrence might be related to the absence of Schwann cells into the spinal cord. Pediatric IM schwannomas are uncommon and in absence of neurofibromatosis (NF) they are extremely rare. To date, very few cases have been reported in the literature. CASE DESCRIPTION: We describe the case of a 8 year-old female affected by a progressive paraparesis...
May 9, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29752520/mucopolysaccharidoses-overview-of-neuroimaging-manifestations
#12
Manal Nicolas-Jilwan, Moeenaldeen AlSayed
The mucopolysaccharidoses are a heterogeneous group of inherited lysosomal storage disorders, characterized by the accumulation of undegraded glycosaminoglycans in various organs, leading to tissue damage. Mucopolysaccharidoses include eight individual disorders (IS [Scheie syndrome], IH [Hurler syndrome], II, III, IV, VI, VII and IX). They have autosomal-recessive transmission with the exception of mucopolysaccharidosis II, which is X-linked. Each individual disorder has a wide spectrum of phenotypic variation, depending on the specific mutation, from very mild to very severe...
May 11, 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29751769/repeated-injections-of-human-umbilical-cord-blood-derived-mesenchymal-stem-cells-significantly-promotes-functional-recovery-in-rabbits-with-spinal-cord-injury-of-two-noncontinuous-segments
#13
Chaohua Yang, Gaoju Wang, Fenfen Ma, Baoqing Yu, Fancheng Chen, Jin Yang, Jianjun Feng, Qing Wang
BACKGROUND: Spinal cord injuries (SCIs) are sustained by an increasing number of patients each year worldwide. The treatment of SCIs has long been a hard nut to crack for doctors around the world. Mesenchymal stem cells (MSCs) have shown benefits for the repair of SCI and recovery of function. Our present study aims to investigate the effects of intravenously infused human umbilical cord blood-derived MSCs (hUCB-MSCs) on functional recovery after subacute spinal cord compression injury of two noncontinuous segments...
May 11, 2018: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/29749479/effect-of-combined-chondroitinase-abc-and-hyperbaric-oxygen-therapy-in-a-rat-model-of-spinal-cord-injury
#14
Xiaoyang Liu, Jiefeng Wang, Guangkuo Li, Honglin Lv
The present study aimed to investigate the effect of combined hyperbaric oxygen (HBO) and chondroitinase ABC (ChABC) enzyme therapy in a rat model of spinal cord injury (SCI) and to explore the underlying mechanisms. A total of 48 healthy male Wistar rats were randomly divided into six groups: Sham, SCI, vehicle, HBO, ChABC enzyme and HBO + ChABC. Excluding the sham group, SCI was established in rats by a clip compression injury and rats subsequently received HBO treatment for 2 weeks with or without an intraspinal injection of 0...
April 26, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29747967/endless-story-of-a-spinal-column-hydatid-cyst-disease-a-case-report
#15
Gokhan Cavus, Vedat Acik, Emre Bilgin, Yurdal Gezercan, Ali Ihsan Okten
We describe a case of multifocal relapsing hydatid cyst following multilevel thoracic corpectomy and 360° instrumentation surgery. A 41-year-old male patient presented with cord compression and paraplegia due to a multiseptated cystic lesion at T10-11 level. The cyst was excised with a combined anterior and posterior approach and 360° stabilization was performed. The patient received albendazole for 1 year after the surgery. The patient presented with paraparesis 5 years after the surgery. Cystic lesions between C2-T1 and T10-11 were detected on the spinal MRI and the patient was operated with removal of the lesions on both levels and adjuvant local 20% hypertonic saline application...
May 7, 2018: Acta Orthopaedica et Traumatologica Turcica
https://www.readbyqxmd.com/read/29743771/cervical-myelopathy-in-a-child-a-rare-cause-of-hypoventilation-syndrome-presenting-with-type-2-respiratory-failure
#16
Alolika Mondal, Prabhas Prasun Giri
Hypoventilation syndrome leading to Type 2 respiratory failure is not a rare cause of Pediatric Intensive Care Unit admission and mechanical ventilation. Common causes in pediatric population are Guillain-Barre syndrome and various central nervous system disorders such as encephalitis, traumatic brain injury, and drugs. Any injury or disease in the cervical cord can also produce respiratory paralysis causing respiratory failure. Here, we present two cases of mixed cerebral palsy with cervical myelopathy due to compression effect of fractured segments of first and second cervical vertebrae...
April 2018: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/29742659/anhydramnios-in-the-setting-of-renal-malformations-the-national-institutes-of-health-workshop-summary
#17
Marva Moxey-Mims, Tonse N K Raju
Anhydramnios in the setting of severe malformations of the fetal kidney and urinary tract is associated with a high incidence of stillbirths and life-threatening complications, including severe pulmonary hypoplasia, umbilical cord compression, and perinatal asphyxia. To prevent such adverse outcomes, some centers in the United States and elsewhere are offering amniotic fluid restoration for women diagnosed with anhydramnios in the setting of fetal renal malformations. The procedures include infusions of amniotic fluid substitutes (normal saline), percutaneously or through an amnioport-an implanted system for serial or continuous infusion of normal saline to maintain the desired amniotic fluid volume...
May 7, 2018: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/29737479/locomotor-treadmill-training-promotes-soleus-trophism-by-mammalian-target-of-rapamycin-pathway-in-paraplegic-rats
#18
Caroline Cunha do Espírito Santo, Daniela Dal Secco, Anamaria Meireles, Gabriel Ribeiro de Freitas, Franciane Bobinski, Mauricio Peña Cunha, Ana Lúcia Severo Rodrigues, Alessandra Swarowsky, Adair Roberto Soares Santos, Jocemar Ilha
Assisted-treadmill training, may be helpful in promoting muscle mass preservation after incomplete spinal cord injury (SCI). However, biological mechanism involved in this process is still not fully understood. This study investigated the effects of locomotor treadmill training on muscle trophism mediated by protein kinase B (Akt)/mammalian target of rapamycin (mTOR)/p70 ribosomal protein S6 kinase (p70S6K) in paraplegic rats. Adult female Wistar rats underwent an incomplete thoracic SCI induced by compression using an aneurysm clip...
May 8, 2018: Neurochemical Research
https://www.readbyqxmd.com/read/29733989/prognostic-factors-of-ambulatory-status-for-patients-with-metastatic-spinal-cord-compression-a-systematic-review-and-meta-analysis
#19
Yongheng Liu, Yongcheng Hu, Xionggang Yang, Dengxing Lun, Feng Wang, Li Yang, Hao Zhang, Jiangtao Feng, Kunchi Hua
BACKGROUND: This study aimed to identify prognostic factors for functional outcome of metastatic spinal cord compression (MSCC). METHODS: All full texts in English regarding the prognostic factors for functional outcome of MSCC, published between January 2007 and October 2017, were identified using the electronic databases PubMed, Embase, and the Cochrane Library. An exploratory meta-analysis was also conducted when appropriate data were available. RESULT: A total of 25 studies, involving 4897 patients, met the inclusion criteria...
May 4, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29733987/use-of-bone-morphogenetic-protein-2-in-vertebral-column-tumor-surgery-a-national-investigation
#20
Rafael De la Garza Ramos, Jonathan Nakhla, Murray Echt, Yaroslav Gelfand, David J Altschul, Woojin Cho, Merrit D Kinon, Reza Yassari
OBJECTIVE: To report the rate of bone morphogenetic protein-2 (BMP-2) use in vertebral column tumor surgery. METHODS: The Nationwide Inpatient Sample database (2012-2014) was queried to identify patients who underwent spinal fusion for vertebral column tumors (primary benign, primary malignant, or metastatic). The rate of BMP-2 use was calculated and patient/operative factors associated with its use were also investigated. RESULTS: A total of 9,375 patients who underwent fusion surgery for spinal tumors between 2012 and 2014 were identified, with 540 cases using BMP-2 (5...
May 4, 2018: World Neurosurgery
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