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Type 1 chiari

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https://www.readbyqxmd.com/read/28738751/a-single-center-retrospective-study-clinical-features-of-different-types-of-budd-chiari-syndrome-in-chinese-patients-in-the-hubei-area
#1
Gangping Li, Ying Huang, Shunyu Tang, Yuhu Song, Huimin Liang, Dehan Liu, Ling Yang, Xiaohua Hou
Background The characteristics and prevalence of Budd-Chiari syndrome in China remain unclear. This study aimed to analyze the clinical features of Budd-Chiari syndrome in Chinese patients in the Hubei area. Methods One-hundred and thirty patients with Budd-Chiari syndrome, admitted to Union Hospital from January 2002 to January 2011, were included in this retrospective study. Clinical features, laboratory data, imaging characteristics, and cumulative patency rates were analyzed. Results Of the 130 patients with Budd-Chiari syndrome, 77 were men (59...
January 1, 2017: Vascular
https://www.readbyqxmd.com/read/28734813/incidence-and-types-of-pediatric-nystagmus
#2
David L Nash, Nancy N Diehl, Brian G Mohney
PURPOSE: To report the incidence, prevalent subtypes and clinical characteristics of pediatric nystagmus diagnosed over a 30-year period. DESIGN: Retrospective, population-based study METHODS: Using the Rochester Epidemiology Project, the medical records of all children (<19 years) diagnosed as residents in Olmsted County, Minnesota with any form of nystagmus from January 1, 1976, through December 31, 2005, were reviewed. RESULTS: Seventy-one children were diagnosed during the 30-year period, yielding an annual incidence of 6...
July 19, 2017: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/28732419/evolution-of-different-surgical-treatment-techniques-for-management-and-improving-outcome-of-chiari-malformation-type-1
#3
Guru Dutta Satyarthee
No abstract text is available yet for this article.
August 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28693072/-comparison-of-posterior-fossa-decompression-with-and-without-duraplasty-for-surgical-management-for-adult-chiari-malformation-type-%C3%A2
#4
H Y Li, Y M Li, H Chen, Y Li, X W Shi
Objective: To evaluate and compare the efficacy between posterior fossa decompression without duraplasty (PFD) and posterior fossa decompression with duraplasty (PFDD) in the surgical management for adult Chiari Ⅰ malformation. Methods: Fifty-seven patients suffered from Chiari malformation type Ⅰ were treated in Department of Neurosurgery, Henan Provincial People's Hospital from August 2008 to October 2013. Twenty-three patients received posterior fossa decompression without duraplasty and the other 34 patients received posterior fossa decompression with duraplasty...
July 4, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28690584/decompression-in-chiari-malformation-clinical-ocular-motor-cerebellar-and-vestibular-outcome
#5
Nicolina Goldschagg, Katharina Feil, Franziska Ihl, Siegbert Krafczyk, Mathias Kunz, Jörg Christian Tonn, Michael Strupp, Aurelia Peraud
BACKGROUND: Treatment of Chiari malformation can include suboccipital decompression with resection of one cerebellar tonsil. Its effects on ocular motor and cerebellar function have not yet been systematically examined. OBJECTIVE: To investigate whether decompression, including resection of one cerebellar tonsil, leads to ocular motor, vestibular, or cerebellar deficits. PATIENTS AND METHODS: Ten patients with Chiari malformation type 1 were systematically examined before and after (1 week and 3 months) suboccipital decompression with unilateral tonsillectomy...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28686331/neural-tube-defects-in-waardenburg-syndrome-a-case-report-and-review-of-the-literature
#6
Joseph Hart, Kalpana Miriyala
Waardenburg syndrome type 1 (WS1) is an autosomal dominant genetic condition characterized by sensorineural deafness and pigment abnormalities, and is caused by variants in the PAX3 homeodomain. PAX3 variants have been associated with severe neural tube defects in mice and humans, but the frequency and clinical manifestations of this symptom remain largely unexplored in humans. Consequently, the role of PAX3 in human neural tube formation remains a study of interest, for clinical as well as research purposes...
July 7, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28665389/-syringosubarachnoid-shunting-in-treatment-of-syringomyelia-a-literature-review-and-a-clinical-case-report
#7
V S Klimov, Yu S Gulay, A V Evsyukov, G I Moysak
In the article, we describe a clinical case of syringomyelia associated with an Arnold-Chiari type 1 malformation, evaluate the efficacy of syringosubarachnoid shunting, and analyze the literature data of domestic and international researchers involved in investigation and treatment of the pathology. Application of syringosubarachnoid shunting in the described case resulted in a clinical improvement in the form of regression of paresis and hypoesthesia, which demonstrated the efficacy of the shunting technique for correction of the syringomyelia symptoms...
2017: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
https://www.readbyqxmd.com/read/28647656/delayed-retroclival-and-cervical-spinal-subdural-hematoma-complicated-by-pre-existing-chiari-malformation-in-an-adult-trauma-patient
#8
Ha Nguyen, Hoon Choi, Shekar Kurpad, Hesham Soliman
INTRODUCTION: Traumatic spinal subdural hematoma involving the retroclival region and upper cervical spine is a rare pathology. To our knowledge, there have only been two prior cases in an adult trauma patient. We describe a patient with pre-existing Chiari 1 malformation, who recently sustained a unilateral Type 1 OCF (occipital condyle fracture) with associated disruption of the tectorial membrane and transverse ligament, that returned with a retroclival subdural hematoma extending down to C7, causing spinal cord compression and symptomatic obstructive hydrocephalus...
June 21, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28592078/-treatment-of-single-one-stage-posterior-atlantoaxial-fixation-in-chiari-malformation
#9
L N Lu, R Zong, X B Xu, X G Yu, G Y Qiao
Objective: To explore the effects of surgical technique of single one-stage posterior C(1-2) screw rod fixation of Chiari malformation (CM) associated with occipitalization and without atlantoaxial dislocation. Methods: A total of 23 patients with CM treated between January 2014 and October 2015 in Department of Neurosurgery of Chinese People's Liberation Army General Hospital were retrospective reviewed. All of them were diagnosis with CM associated with occipitalization and without atlantoaxial dislocation, including 8 males and 15 females, aging from 11 to 57 years (mean (35...
June 1, 2017: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/28586935/endoscopic-management-of-arnold-chiari-malformation-type-i-with-or-without-syringomyelia
#10
Shailendra Ratre, Nishtha Yadav, Yad Ram Yadav, Vijay Singh Parihar, Jitin Bajaj, Yatin Kher
Introduction Several different surgical techniques have been used in the treatment of patients with symptomatic Arnold-Chiari malformation type 1 (ACM-1) with or without syrinx. Endoscope-assisted decompression of the posterior fossa has been found to be safe and effective. We report our initial experience of endoscopic management of ACM-I. Material and Methods This was a prospective study of 15 symptomatic patients. Pre- and postoperative clinical status and computed tomography and magnetic resonance imaging findings were recorded...
June 6, 2017: Journal of Neurological Surgery. Part A, Central European Neurosurgery
https://www.readbyqxmd.com/read/28582306/delayed-presentation-of-isolated-sagittal-synostosis-with-raised-intracranial-pressure-and-secondary-chiari-malformation-with-cervical-syringomyelia
#11
Stratos S Sofos, Ben Robertson, Christian Duncan, Ajay Sinha
Sagittal craniosynostosis (SC) is the most common type of premature suture fusion presenting in approximately 1 in every 5000 births with a 3:1 male:female ratio. The most common indication for surgery is the improvement of the cosmetic appearance of the skull, since a cranial deformation may have a significant psychosocial impact on affected patients. Relief from raised intracranial pressure is a further indication for surgery, although an increased intracranial pressure (ICP) can be demonstrated only in a minority of affected children at diagnosis...
June 2, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28566405/intraspinal-anomalies-in-early-onset-idiopathic-scoliosis
#12
E A C Pereira, M Oxenham, K S Lam
AIMS: In the United Kingdom, lower incidences of intraspinal abnormalities in patients with early onset idiopathic scoliosis have been observed than in studies in other countries. We aimed to determine the rates of these abnormalities in United Kingdom patients diagnosed with idiopathic scoliosis before the age of 11 years. PATIENTS AND METHODS: This retrospective study of patients attending an urban scoliosis clinic identified 71 patients satisfying a criteria of: clinical diagnosis of idiopathic scoliosis; age of onset ten years and 11 months or less; MRI screening for intraspinal abnormalities...
June 2017: Bone & Joint Journal
https://www.readbyqxmd.com/read/28564670/current-concepts-in-the-pathogenesis-diagnosis-and-management-of-type-i-chiari-malformations
#13
Cody A Doberstein, Radmehr Torabi, Petra M Klinge
Type 1 Chiari malformations (CMs) are a group of congenital or acquired disorders which include the abnormal presence of the cerebellar tonsils in the upper spinal canal, rather than the posterior fossa. The resulting anatomic abnormality causes crowding of the structures at the craniocervical junction and can impair the normal flow of cerebral spinal fluid (CSF) in this region. This impairment in CSF flow dynamics can led to the development of syringomyelia or hydrocephalus. Type 1 CMs have been associated with a wide array of symptoms resulting from either cerebellar and brainstem compression and distortion or disturbances in CSF dynamics, and can affect both children and adults...
June 1, 2017: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/28561456/investigation-of-plasminogen-activator-inhibitor-1-pai-1-4g-5g-promoter-polymorphism-in-indian-venous-thrombosis-patients-a-case-control-study
#14
Aniket Prabhudesai, Shrimati Shetty, Kanjaksha Ghosh, Bipin Kulkarni
BACKGROUND: The role of PAI-1 4G/5G polymorphism in venous thrombosis has been contradictory. PAI-1 4G/4G genotype is associated with elevated levels of PAI-1 resulting in a hypofibrinolytic state and a higher thrombotic risk. OBJECTIVE: In this study, the distribution of genotypes and frequency of alleles of the 4G/5G polymorphism of PAI-1 gene in Indian patients with different types of venous thrombosis was investigated for its role in development of thrombosis...
May 31, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28527081/chiari-malformation-type-1-related-headache-the-importance-of-a-multidisciplinary-study
#15
Marcella Curone, L G Valentini, I Vetrano, E Beretta, M- Furlanetto, L Chiapparini, A Erbetta, G Bussone
Chiari type 1 Malformation (CM 1) is a structural defect consisting of a displacement of the cerebellar tonsils through the foramen magnum causing obstruction of cerebrospinal fluid (CSF) outflow. CM 1 has a variety of presentation with headache being the most common symptom. The evaluation and treatment of headache related to CM 1 are often difficult, because the pain in the occipital-suboccipital region or headache that is of cough-type suggests symptomatic CM 1, but patients suffering from CM 1 can also report migraine or tension-type headache...
May 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28524792/analysis-and-interrater-reliability-of-pb-c2-using-mri-and-ct-data-from-the-park-reeves-syringomyelia-research-consortium-on-behalf-of-the-pediatric-craniocervical-society
#16
Todd C Hankinson, Gerald F Tuite, Dagmara I Moscoso, Leslie C Robinson, James C Torner, David D Limbrick, Tae Sung Park, Richard C E Anderson
OBJECTIVE The distance to the ventral dura, perpendicular to the basion to C2 line (pB-C2), is commonly employed as a measure describing the anatomy of the craniovertebral junction. However, both the reliability among observers and the clinical utility of this measurement in the context of Chiari malformation Type I (CM-I) have been incompletely determined. METHODS Data were reviewed from the first 600 patients enrolled in the Park-Reeves Syringomyelia Research Consortium with CM-I and syringomyelia. Thirty-one cases were identified in which both CT and MRI studies were available for review...
August 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28523220/cardiovascular-risk-factors-in-chiari-malformation-and-idiopathic-intracranial-hypertension
#17
Radek Frič, Are Hugo Pripp, Per Kristian Eide
OBJECTIVES: Both Chiari malformation type 1 (CMI, i.e., the idiopathic caudal ectopy of cerebellar tonsils into foramen magnum) and idiopathic intracranial hypertension (IIH) are characterized by reduced intracranial compliance (ICC) due to disturbed circulation of cerebrospinal fluid (CSF). An increasing body of evidence links cardiovascular disease to CSF circulation disturbances. The aim of this study was to explore whether the prevalence of cardiovascular risk factors in patients with CMI or IIH is higher than in the general population...
May 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28513615/exome-sequencing-of-two-italian-pedigrees-with-non-isolated-chiari-malformation-type-i-reveals-candidate-genes-for-cranio-facial-development
#18
Elisa Merello, Lorenzo Tattini, Alberto Magi, Andrea Accogli, Gianluca Piatelli, Marco Pavanello, Domenico Tortora, Armando Cama, Zoha Kibar, Valeria Capra, Patrizia De Marco
Chiari malformation type I (CMI) is a congenital abnormality of the cranio-cerebral junction with an estimated incidence of 1 in 1280. CMI is characterized by underdevelopment of the occipital bone and posterior fossa (PF) and consequent cerebellar tonsil herniation. The presence for a genetic basis to CMI is supported by many lines of evidence. The cellular and molecular mechanisms leading to CM1 are poorly understood. The occipital bone formation is dependent on complex interactions between genes and molecules with pathologies resulting from disruption of this delicate process...
August 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28453783/sleep-related-breathing-disorders-in-chiari-malformation-type-1-a-prospective-study-of-90-patients
#19
Álex Ferré, Maria A Poca, Maria D de la Calzada, Dulce Moncho, Odile Romero, Gabriel Sampol, Juan Sahuquillo
Study objective: The aim of the present study is to describe the prevalence of sleep disorders in a large group of patients with Chiari malformation type 1 (CM-1) and determine the presence of risk factors associated with these abnormalities. Methods: Prospective study with consecutive patient selection. We included 90 adult patients with CM-1, defined by the presence of a cerebellar tonsillar descent (TD) ≥3 mm. Clinical, neuroradiological studies, and nocturnal polysomnography (PSG) was carried out...
June 1, 2017: Sleep
https://www.readbyqxmd.com/read/28437811/symptomatic-outcome-after-bone-only-suboccipital-decompression-in-adult-patients-with-chiari-type-i-malformations-in-the-absence-of-hydromyelia-or-hydrocephalus
#20
Nicolas Olmo Koechlin, Hazem J Abuhusain, Manuri Gunawardena, Tyler S Auschwitz, Charles Teo
Background Type I Chiari malformation presents without an associated hydromyelia in 30 to 70% of cases, yet there is no agreement regarding the optimal surgical treatment for these patients. We review our experience for treating symptomatic adult type I Chiari malformation without hydromyelia using a suboccipital bone decompression of the hindbrain and no duraplasty in 12 adult patients. Outcome was measured according to the Chicago Chiari Outcome Scale (CCOS). Results Nine of 12 patients were female; average age at surgery was 34...
July 2017: Journal of Neurological Surgery. Part A, Central European Neurosurgery
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