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https://www.readbyqxmd.com/read/28072961/-a-case-report-of-childhood-farber-s-disease-and-literature-review
#1
X H Bao, J M Tian, T Y Ji, X Z Chang
Objective: To explore the clinical features, diagnosis, treatment and the prognosis of Farber disease by case report and literature review. Method: The clinical information of a case with farber's disease diagnosed in October 2015 at Peking University First Hospital was collected and analyzed, including clinical manifestation, electrophysiology, magnetic resonance imaging, pathology, treatments and prognosis.ASAH1 gene mutational analysis was conducted in the patient and her parents.By using "Farber's disease, ASAH1" as keywords, literature was searched from Pubmed, CHKD and HGMD database from January 1951 to January 2016...
January 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28024286/taurine-treatment-preserves-brain-and-liver-mitochondrial-function-in-a-rat-model-of-fulminant-hepatic-failure-and-hyperammonemia
#2
Akram Jamshidzadeh, Reza Heidari, Mozhgan Abasvali, Mehdi Zarei, Mohammad Mehdi Ommati, Narges Abdoli, Forouzan Khodaei, Yasaman Yeganeh, Faezeh Jafari, Azita Zarei, Zahra Latifpour, Elnaz Mardani, Negar Azarpira, Behnam Asadi, Asma Najibi
Ammonia-induced mitochondrial dysfunction and energy crisis is known as a critical consequence of hepatic encephalopathy (HE). Hence, mitochondria are potential targets of therapy in HE. The current investigation was designed to evaluate the role of taurine treatment on the brain and liver mitochondrial function in a rat model of hepatic encephalopathy and hyperammonemia. The animals received thioacetamide (400mg/kg, i.p, for three consecutive days at 24-h intervals) as a model of acute liver failure and hyperammonemia...
December 23, 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28009405/fulminant-idiopathic-intracranial-hypertension-and-venous-stasis-retinopathy-resulting-in-severe-bilateral-visual-impairment
#3
Alexander Voldman, Breanna Durbin, John Nguyen, Brian Ellis, Monique Leys
PURPOSE: To report a complicated case of fulminant idiopathic intracranial hypertension and concomitant venous stasis retinopathy leading to postpapilledema optic atrophy. METHODS: Case report. RESULTS: A 34-year-old morbidly obese woman with a history of idiopathic intracranial hypertension (IIH) presented with a 1-month history of bilateral vision loss, diplopia, and left eye pain after being lost to follow-up for 6 years. Fundus examination revealed florid papilledema with venous tortuosity bilaterally...
December 6, 2016: European Journal of Ophthalmology
https://www.readbyqxmd.com/read/27998460/-treatment-of-idiopathic-intracranial-hypertension
#4
Y H Wang, J Ma, Y Zhong
Idiopathic intracranial hypertension(IIH) is a kind of diffuse brain swelling disease. Several causes could lead to IIH. Intracranial venous circulatory disturbance is more common. The clinical manifestations include intracranial pressure increased, headache and papilledema. Since IIH usually has serious impact on vision even blindness, the diagnosis and corresponding treatment are especially important. This review is to discuss the new insights on the therapeutic options of IIH, which cover medical treatment, weight-losing therapy, surgical treatment, interventional therapy and supportive treatment...
December 11, 2016: [Zhonghua Yan Ke za Zhi] Chinese Journal of Ophthalmology
https://www.readbyqxmd.com/read/27938476/traumatic-brain-injury-current-treatment-strategies-and-future-endeavors
#5
Michael Galgano, Gentian Toshkezi, Xuecheng Qiu, Thomas Russell, Lawrence Chin, Li-Ru Zhao
Traumatic brain injury presents in various forms ranging from mild alterations of consciousness to an unrelenting comatose state and death. In the most severe form of traumatic brain injury, the entirety of the brain is affected by a diffuse type of injury and swelling. Treatment modalities vary extensively based on the severity of the injury and range from daily cognitive therapy sessions to radical surgery such as bilateral decompressive craniectomies. Guidelines have been set forth regarding the optimal management of traumatic brain injury, but they must be taken in context of the situation and cannot be used in every individual circumstance...
22, 2016: Cell Transplantation
https://www.readbyqxmd.com/read/27931151/regulation-of-mitochondrial-function-and-glutamatergic-system-are-the-target-of-guanosine-effect-in-traumatic-brain-injury
#6
Fernando Dobrachinski, Rogério da Rosa Gerbatin, Gláubia Sartori, Naiani Ferreira Marques, Ana Paula Zemolin, Luiz Fernando Almeida Silva, Jeferson Luis Franco, Luiz Fernando Freire Royes, Michele Rechia Fighera, Félix Alexandre Antunes Soares
Traumatic brain injury (TBI) is a highly complex multi-factorial disorder. Experimental trauma involves primary and secondary injury cascades that underlie delayed neuronal dysfunction and death. Mitochondrial dysfunction and glutamatergic excitotoxicity are the hallmark mechanisms of damage. Accordingly, a successful pharmacological intervention requires a multi-faceted approach. Guanosine (GUO) is known for its neuromodulator effects in various models of brain pathology, specifically those that involve the glutamatergic system...
January 13, 2017: Journal of Neurotrauma
https://www.readbyqxmd.com/read/27923754/successful-coil-embolization-of-pediatric-carotid-cavernous-fistula-due-to-ruptured-posttraumatic-giant-internal-carotid-artery-aneurysm
#7
Daisuke Wajima, Ichiro Nakagawa, Hun Soo Park, Shohei Yokoyama, Takeshi Wada, Kimihiko Kichikawa, Hiroyuki Nakase
BACKGROUND: The goal of the treatment of direct carotid cavernous fistula (CCF) is to occlude the arteriovenous shunt and to preserve the patency of the concerned internal carotid artery. However, for the ipsilateral posttraumatic fragile cerebrum, coil embolization plus parent artery occlusion for the high-flow direct CCF is better for the prevention of hyperperfusion syndrome and intracranial hemorrhage. We experienced such a case and managed it successfully. CASE DESCRIPTION: A 6-year-old boy had severe head trauma caused by being hit by a car...
December 5, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27918421/decreased-stat3-phosphorylation-mediates-cell-swelling-in-ammonia-treated-astrocyte-cultures
#8
Arumugam R Jayakumar, Kevin M Curtis, Kiran S Panickar, Nagarajarao Shamaladevi, Michael D Norenberg
Brain edema, due largely to astrocyte swelling, and the subsequent increase in intracranial pressure and brain herniation, are major complications of acute liver failure (ALF). Elevated level of brain ammonia has been strongly implicated in the development of astrocyte swelling associated with ALF. The means by which ammonia brings about astrocyte swelling, however, is incompletely understood. Recently, oxidative/nitrosative stress and associated signaling events, including activation of mitogen-activated protein kinases (MAPKs), as well as activation of the transcription factor, nuclear factor-kappaB (NF-κB), have been implicated in the mechanism of ammonia-induced astrocyte swelling...
December 2, 2016: Biology
https://www.readbyqxmd.com/read/27896810/multiple-pathways-for-elevating-extracellular-adenosine-in-the-rat-hippocampal-ca1-region-characterized-by-adenosine-sensor-cells
#9
Kunihiko Yamashiro, Yuki Fujii, Shohei Maekawa, Mitsuhiro Morita
Extracellular adenosine in the brain, which modulates various physiological and pathological processes, fluctuates in a complicated manner that reflects the circadian cycle, neuronal activity, metabolism, and disease states. The dynamics of extracellular adenosine in the brain are not fully understood, largely because of the lack of simple and reliable methods of measuring time-dependent changes in tissue adenosine distribution. This study describes the development of a biosensor, designated an adenosine sensor cell, expressing adenosine A1 receptor, and a genetically modified G protein...
January 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/27858578/severe-cerebral-edema-following-nivolumab-treatment-for-pediatric-glioblastoma-case-report
#10
Xiao Zhu, Michael M McDowell, William C Newman, Gary E Mason, Stephanie Greene, Mandeep S Tamber
Nivolumab is an immune checkpoint inhibitor (ICI) currently undergoing Phase III clinical trials for the treatment of glioblastoma. The authors present the case of a 10-year-old girl with glioblastoma treated with nivolumab under compassionate-use guidelines. After the first dose of nivolumab the patient developed hemiparesis, cerebral edema, and significant midline shift due to severe tumor necrosis. She was managed using intravenous dexamethasone and discharged on a dexamethasone taper. The patient's condition rapidly deteriorated after the second dose of nivolumab, demonstrating hemiplegia, seizures, and eventually unresponsiveness with a fixed and dilated left pupil...
November 18, 2016: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/27857999/do-age-and-anticoagulants-affect-the-natural-history-of-acute-subdural-hematomas
#11
Brandon P Lucke-Wold, Ryan C Turner, Darnell Josiah, Chelsea Knotts, Sanjay Bhatia
Acute subdural hematoma is a serious complication following traumatic brain injury. Large volume hematomas or those with underlying brain injury can cause mass effect, midline shift, and eventually herniation of the brain. Acute subdural hematomas in the young are associated with high-energy trauma and often have underlying contusions, while acute subdural hematomas in the elderly are associated with minor trauma and an absence of underlying contusions, even though the elderly are more likely to be on anticoagulants or anti-platelet therapy...
2016: Archives of Emergency Medicine and Critical Care
https://www.readbyqxmd.com/read/27857913/addition-of-resection-of-temporal-muscle-and-fascia-in-decompressive-craniectomy-in-the-treatment-of-traumatic-brain-injury
#12
Seung Han Yu, Byung Chul Kim, Jae Young Choi, Jae Il Lee, Won Ho Cho, Hyuk Jin Choi
OBJECTIVE: Decompressive craniectomy (DC) is a widely used surgical procedure for control of severely increased intracranial pressure in various conditions. The goal of this study is to evaluate the effectiveness of the addition of resection of temporalis muscle and fascia in DC particularly in the treatment of traumatic brain injury. METHODS: Twenty patients underwent temporalis muscle and fascia resection in addition to conventional DC and duroplasty due to massive brain swelling in a single tertiary hospital from 2013 to 2015 were enrolled...
October 2016: Korean Journal of Neurotrauma
https://www.readbyqxmd.com/read/27725221/fresh-frozen-plasma-modulates-brain-gene-expression-in-a-swine-model-of-traumatic-brain-injury-and-shock-a-network-analysis
#13
Martin Sillesen, Ted Bambakidis, Simone E Dekker, Yongqing Li, Hasan B Alam
BACKGROUND: Resuscitation with fresh frozen plasma (FFP) decreases brain lesion size and swelling in a swine model of traumatic brain injury and hemorrhagic shock. We hypothesized that brain gene expression profiles after traumatic brain injury and hemorrhagic shock would be modulated by FFP resuscitation. STUDY DESIGN: Fifteen swine underwent a protocol of traumatic brain injury and hemorrhagic shock and 2 hours of shock followed by resuscitation with FFP, normal saline, or hetastarch (5/group)...
January 2017: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/27694105/establishment-of-a-novel-retinoblastoma-rb-nude-mouse-model-by-intravitreal-injection-of-human-rb-y79-cells-comparison-of-in-vivo-analysis-versus-histological-follow-up
#14
Alexander V Tschulakow, Ulrich Schraermeyer, H Peter Rodemann, Sylvie Julien-Schraermeyer
Retinoblastoma (Rb) is the most frequent primary intraocular tumour in children and, if left untreated, can cause death. Preclinical animal models that mimic molecular, genetic, and cellular features of cancers are essential for studying cancer and searching for promising diagnosis and treatment modalities. There are several models described for Rb, but none of them fully meet our requirements. The aim of this study was to create a novel xenograft-nude mouse-model with broad application possibilities, which closely resembles the clinical observations of Rb patients and which could be used to investigate the development and spread of the tumour by using scanning laser ophthalmoscopy/optical coherence tomography (SLO/OCT) as well as histology methods...
November 15, 2016: Biology Open
https://www.readbyqxmd.com/read/27623738/acetazolamide-mitigates-astrocyte-cellular-edema-following-mild-traumatic-brain-injury
#15
Nasya M Sturdivant, Sean G Smith, Syed F Ali, Jeffrey C Wolchok, Kartik Balachandran
Non-penetrating or mild traumatic brain injury (mTBI) is commonly experienced in accidents, the battlefield and in full-contact sports. Astrocyte cellular edema is one of the major factors that leads to high morbidity post-mTBI. Various studies have reported an upregulation of aquaporin-4 (AQP4), a water channel protein, following brain injury. AZA is an antiepileptic drug that has been shown to inhibit AQP4 expression and in this study we investigate the drug as a therapeutic to mitigate the extent of mTBI induced cellular edema...
September 14, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27621948/mechanical-solitaire-thrombectomy-with-low-dose-booster-tirofiban-injection
#16
Duck-Ho Goh, Sung-Chul Jin, Hae Woong Jeong, Sam Yeol Ha
PURPOSE: Mechanical thrombectomy using a Solitaire stent has been associated with a high recanalization rate and favorable clinical outcome in intra-arterial thrombolysis. To achieve a higher recanalization rate for mechanical Solitaire thrombectomy, we used an intra-arterial low-dose booster tirofiban injection into the occluded segment after stent deployment. We report the safety and recanalization rates for mechanical Solitaire thrombectomy with a low-dose booster tirofiban injection...
September 2016: Neurointervention
https://www.readbyqxmd.com/read/27591100/volume-of-brain-herniation-in-ischemic-stroke-patients-following-decompressive-craniectomy
#17
Kirsten E Stoner, Kingsley O Abode-Iyamah, Nicole M Grosland, Matthew A Howard
BACKGROUND: Decompressive craniectomy procedures are performed in patients with malignant intracranial hypertension. A bone flap is removed to relieve pressure. Later a second operation is performed to reconstruct the skull after brain swelling has resolved. This surgical treatment would be improved if it were possible to perform a single operation that decompressed the brain acutely while eliminating the need for a second operation. In order to design a device and procedure that achieves this objective it is essential to understand how the brain swells following a craniectomy procedure...
August 30, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27588068/3d-dir-for-early-differential-diagnostic-and-prognostic-evaluation-of-nmo
#18
Yanbing Wang, Hong Yan, Qixing Ding, Cunhua Mao, Yelong Shen, Guangbin Wang
Neuromyelitis optica (NMO) is an acute or subacute lesion of demyelinating disease involving the optic nerve and spinal cord, and imaging techniques and their effects have been the focus of investigations. The aim of the present study was to examine the value of three-dimensional double inversion recovery (3D-DIR) in the early differential diagnostic and prognostic evaluation of NMO. Forty-eight patients with suspicious NMO were included into the study and underwent a combination of serum NMO-IgG quantitative detection and 3D-DIR examination...
September 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/27583179/clinical-outcome-prediction-in-aneurysmal-subarachnoid-hemorrhage-alterations-in-brain-body-interface
#19
Benjamin W Y Lo, Hitoshi Fukuda, Mark Angle, Jeanne Teitelbaum, R Loch Macdonald, Forough Farrokhyar, Lehana Thabane, Mitchell A H Levine
BACKGROUND: Brain-body associations are essential in influencing outcome in patients with ruptured brain aneurysms. Thus far, there is scarce literature on such important relationships. METHODS: The multicenter Tirilazad database (3551 patients) was used to create this clinical outcome prediction model in order to elucidate significant brain-body associations. Traditional binary logistic regression models were used. RESULTS: Binary logistic regression main effects model included four statistically significant single prognostic variables, namely, neurological grade, age, stroke, and time to surgery...
2016: Surgical Neurology International
https://www.readbyqxmd.com/read/27567243/safety-and-efficacy-of-intravenous-glyburide-on-brain-swelling-after-large-hemispheric-infarction-games-rp-a-randomised-double-blind-placebo-controlled-phase-2-trial
#20
Kevin N Sheth, Jordan J Elm, Bradley J Molyneaux, Holly Hinson, Lauren A Beslow, Gordon K Sze, Ann-Christin Ostwaldt, Gregory J Del Zoppo, J Marc Simard, Sven Jacobson, W Taylor Kimberly
BACKGROUND: Preclinical models of stroke have shown that intravenous glyburide reduces brain swelling and improves survival. We assessed whether intravenous glyburide (RP-1127; glibenclamide) would safely reduce brain swelling, decrease the need for decompressive craniectomy, and improve clinical outcomes in patients presenting with a large hemispheric infarction. METHODS: For this double-blind, randomised, placebo-controlled phase 2 trial, we enrolled patients (aged 18-80 years) with a clinical diagnosis of large anterior circulation hemispheric infarction for less than 10 h and baseline diffusion-weighted MRI image lesion volume of 82-300 cm(3) on MRI at 18 hospitals in the USA...
October 2016: Lancet Neurology
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