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Chiari I

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https://www.readbyqxmd.com/read/28527081/chiari-malformation-type-1-related-headache-the-importance-of-a-multidisciplinary-study
#1
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
#2
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...
May 19, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28523220/cardiovascular-risk-factors-in-chiari-malformation-and-idiopathic-intracranial-hypertension
#3
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/28516323/diffusion-tensor-imaging-findings-of-the-brainstem-in-subjects-with-tonsillar-ectopia
#4
Serpil Kurtcan, Alpay Alkan, Huseyin Yetis, Umit Tuzun, Ayse Aralasmak, Huseyin Toprak, Huseyin Ozdemir
We aimed to evaluate the differences between apparent diffusion coefficient (ADC), fractional anisotropy (FA), axial diffusivity (AD), and radial diffusivity (RD) values obtained from different cranial sites in subjects with Chiari I Malformation (CM-I) and borderline tonsillar ectopia (BTE), and to determine correlations between diffusion tensor imaging (DTI) metrics and the severity of tonsillar ectopia. A total of 73 subjects with CM-I and BTE and 35 control underwent MRI and DTI. In our study, ADC values measured from the level of medulla oblongata and the RD values measured in middle cerebellar peduncles, thalamus, and globus pallidus were higher in CM-I patients than in controls...
May 17, 2017: Acta Neurologica Belgica
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
#5
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...
May 17, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28508159/constant-dripping-wears-away-the-bone-intraosseous-c2-and-suboccipital-intradiploic-cerebrospinal-fluid-collection-after-chiari-i-decompressive-surgery
#6
Arthur R Kurzbuch, Shailendra Magdum
The objective of decompressive surgery for Chiari I malformation is to create additional space to accommodate the ectopic cerebellar tonsils and to improve the flow of cerebrospinal fluid. To the best of our knowledge, we report the first case of a patient with previous Chiari I decompression presenting 5 years after surgery at the same time as an intraosseous C2 and a suboccipital intradiploic cerebrospinal fluid accumulation. Because of the progressive nature of the C2 cerebrospinal fluid collection causing bone destruction, surgical management was indicated...
May 15, 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28498073/does-the-mesodermal-derangement-in-chiari-type-i-malformation-extend-to-the-cervical-spine-evidence-from-an-analytical-morphometric-study-on-cervical-paraspinal-muscles
#7
Sumit Thakar, Avinash Kurudi Siddappa, Saritha Aryan, Dilip Mohan, Narayanam Anantha Sai Kiran, Alangar S Hegde
OBJECTIVE The mesodermal derangement in Chiari Type I malformation (CMI) has been postulated to encompass the cervical spine. The objectives of this study were to assess the cross-sectional areas (CSAs) of cervical paraspinal muscles (PSMs) in patients with CMI without syringomyelia, compare them with those in non-CMI subjects, and evaluate their correlations with various factors. METHODS In this retrospective study, the CSAs of cervical PSMs in 25 patients were calculated on T2-weighted axial MR images and computed as ratios with respect to the corresponding vertebral body areas...
May 12, 2017: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/28490224/prevalence-of-abnormal-magnetic-resonance-imaging-findings-in-children-with-persistent-symptoms-after-pediatric-sports-related-concussion
#8
Robert H Bonow, Seth D Friedman, Francisco A Perez, Richard G Ellenbogen, Samuel R Browd, Christine L MacDonald, Monica S Vavilala, Frederick P Rivara
A subset of patients experience persistent symptoms after pediatric concussion, and magnetic resonance imaging (MRI) is commonly used to evaluate for pathology. The utility of this practice is unclear. We conducted a retrospective cohort study to describe the MRI findings in children with concussion. A registry of all patients seen at our institution from January 2010 through March 2016 with pediatric sports-related concussion was cross-referenced with a database of radiographic studies. Radiology reports were reviewed for abnormal findings...
May 10, 2017: Journal of Neurotrauma
https://www.readbyqxmd.com/read/28484540/a-common-case-with-an-unusual-association-chiari-i-malformation-with-holocord-syrinx
#9
Sumit Bansal, Sachin A Borkar, Ashok K Mahapatra
Authors are presenting a common case of Chiari malformation but with a very unusual associated finding, holocord syringomyelia, which responded to posterior fossa decompression. An 11-year-old male patient presented with progressive left hemiparesis and numbness on left half of the body for 4 years. Magnetic resonance imaging of the spine revealed peg-shaped herniation of tonsils 8 mm below the foramen magnum and holocord syringomyelia. No focal intraspinal mass was seen. Chiari I malformation with holocord syrinx was diagnosed...
April 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28483229/use-of-three-dimensional-cad-cam-assisted-virtual-surgical-simulation-and-planning-in-the-pediatric-craniofacial-population
#10
Rachel Gray, Alexander Gougoutas, Vinh Nguyen, Jesse Taylor, Nicholas Bastidas
OBJECTIVE: Virtual Surgical Planning (VSP) and computer-aided design/computer-aided manufacturing (CAD/CAM) have recently helped improve efficiency and accuracy in many different craniofacial surgeries. Research has mainly focused on the use in the adult population with the exception of the use for mandibular distractions and cranial vault remodeling in the pediatric population. This study aims to elucidate the role of VSP and CAD/CAM in complex pediatric craniofacial cases by exploring its use in the correction of midface hypoplasia, orbital dystopia, mandibular reconstruction, and posterior cranial vault expansion...
June 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28479808/chiari-i-malformation-associated-with-turner-syndrome
#11
Kamble Jayaprakash Harsha, Jeevan S Nair
Turner syndrome (TS) is a rare genetic disease due to the absence of one X chromosome. Patients with TS have more subtle neurological/neuropsychiatric problems, while headache is an uncommon clinical presentation which needs attention. We report a 12-year-old child presenting with typical cough headache. Her magnetic resonance imaging revealed Chiari I malformation associated with TS. To the best of our knowledge, Chiari I malformation associated with TS is not described in literature. We report the first case of TS associated with Chiari I malformation...
April 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28444624/targeting-ntrk-fusion-in-non-small-cell-lung-cancer-rationale-and-clinical-evidence
#12
REVIEW
Biagio Ricciuti, Marta Brambilla, Giulio Metro, Sara Baglivo, Roberta Matocci, Matteo Pirro, Rita Chiari
In the era of personalized medicine, the identification of targetable genetic alterations represented a major step forward in anticancer therapy. NTRK rearrangements represent the molecular driver of a subset of solid tumors, including 3% of non-small-cell lung cancers (NSCLCs). Preliminary data indicate that molecularly selected NSCLC patients harboring NTRK fusions derive an unprecedented clinical benefit from Trk-directed targeted therapies. The aim of this review is to describe the molecular biology of NTRK signaling pathway and to summarize the preclinical data on novel Trk inhibitors, touching upon the clinical development of these inhibitors for the treatment of advanced NSCLC, which have already shown encouraging anticancer activity and acceptable safety profile in early phase I clinical trials...
June 2017: Medical Oncology
https://www.readbyqxmd.com/read/28441280/the-clinical-utility-of-flexion-extension-cervical-spine-mri-in-22q11-2-deletion-syndrome
#13
Samuel E Kolman, Stephanie Y Ohara, Aashim Bhatia, Tamara Feygin, Dino Colo, Keith D Baldwin, Donna Mcdonald-Mcginn, David A Spiegel
BACKGROUND: Our goal is to correlate the findings on flexion and extension radiographs with dynamic magnetic resonance imaging (MRI), and the clinical history, in a nonrandomly selected cohort of patients with 22q11.2 deletion syndrome (22q). METHODS: All patients with the 22q who had a dynamic MRI from January 2004 to March 2015 were included. We analyzed multiple radiographic measurements on both the dynamic plain films and the MRIs, and correlated these findings with a review of each patient's medical record...
April 24, 2017: Journal of Pediatric Orthopedics
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
#14
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...
April 24, 2017: Journal of Neurological Surgery. Part A, Central European Neurosurgery
https://www.readbyqxmd.com/read/28416844/clinical-characteristics-of-neurogenic-dysphagia-in-adult-patients-with-chiari-malformation-type-i
#15
T Yu, J Li, K Wang, Y Ge, A C Jiang, L P Duan, Z Y Wang
OBJECTIVE: To investigate changes of swallowing function and associated symptoms in Chiari malformation typeI (CMI) patients with and without dysphagia by the analysis of their clinical and high-resolution manometry (HRM) parameters. METHODS: A total of 42 patients diagnosed with symptomatic CMI without atlantoaxial dislocations which were confirmed by clinical manifestations and magnetic resonance imaging (MRI) findings between January 2010 and July 2015 at Peking University Third Hospital were included in this study...
April 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/28409726/the-suboccipital-ligament
#16
Kelsey Alabaster, M Fred Bugg, Bruno Splavski, Frederick A Boop, Kenan I Arnautovic
OBJECTIVE A fibrous structure located dorsal to the dura at the posterior craniocervical junction stretches horizontally between the bilateral occipital condyles and the upper borders of the C-1 laminae. Partially covered by the occipital bone, this structure is always encountered when the bone is removed from the foramen magnum rim during approaches to the posterior cranial fossa. Although known to surgeons, this structure has not been defined, studied, or named. The most appropriate name for this structure is "the suboccipital ligament," and a detailed rationale for this name is provided...
April 14, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28403928/-prenatal-diagnosis-of-neural-tube-defects-correlation-between-prenatal-and-postnatal-data
#17
L Matuszewski, E Perdriolle-Galet, I Clerc-Urmès, P Bach-Segura, O Klein, J P Masutti, O Morel
OBJECTIVES: Neural tube defects (NTD) are congenital anomalies that can cause significant neurological long-term disabilities. Theses malformations are accessible to prenatal diagnosis and quite recently, to in utero repair for some myelomeningoceles. The aim of this study was to analyse the correlation between prenatal and postnatal examinations. MATERIALS AND METHODS: A descriptive retrospective monocentric study has been conducted between January 2004 and December 2014 in a tertiary care maternity...
March 2017: J Gynecol Obstet Hum Reprod
https://www.readbyqxmd.com/read/28384788/neurologic-evaluation-in-children-with-laryngeal-cleft
#18
Ryan D Walker, Alexandria L Irace, Margaret A Kenna, David K Urion, Reza Rahbar
Importance: Referral to a neurologist and imaging play important roles in the management of laryngeal cleft. Swallowing involves a complex series of neuromuscular interactions, and aspiration can result from anatomical causes (eg, laryngeal cleft), neuromuscular disorders, or some combination thereof. To date, no protocols or guidelines exist to identify which patients with laryngeal cleft should undergo neuroimaging studies and/or consultation with a neurologist. Objective: To establish guidelines for neurologic evaluation and imaging techniques to identify or rule out neuromuscular dysfunction in children with laryngeal cleft...
April 6, 2017: JAMA Otolaryngology—Head & Neck Surgery
https://www.readbyqxmd.com/read/28384597/no-significant-difference-between-chiari-malformation-type-1-5-and-type-i
#19
Wei Liu, Hongxing Wu, Yalikun Aikebaier, Maoliti Wulabieke, Rexiti Paerhati, Xiaopeng Yang
OBJECTIVE: Chiari malformation Type 1.5 (CM 1.5) was defined as the association of Chiari malformation Type I (CM I) and brainstem herniation. The objective was to demonstrate the difference of clinical features and surgical outcomes between CM 1.5 and CM I. PATIENTS AND METHODS: All CM 1.5 and CM I adult patients who underwent posterior fossa decompression with duraplasty at our institution between 2006 and 2010 were retrospectively reviewed. Clinical characteristics, imaging features, and long-term outcomes were compared between CM 1...
March 30, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28382434/evaluation-of-the-central-sleep-apnea-in-asymptomatic-children-with-chiari-1-malformation-an-open-question
#20
Marco Zaffanello, Francesco Sala, Luca Sacchetto, Emma Gasperi, Giorgio Piacentini
INTRODUCTION: Type I is the most common Chiari malformation in children. In this condition, the lower part of the cerebellum, but not the brain stem, extends into the foramen magnum at the base of the skull leading to intermittent brain hypertension. In symptomatic children, central sleep apneas are shown in polysomnography evaluation. A central apnea index of 1/h or more is considered abnormal, but >5/h is clearly considered pathological. Therefore, central sleep apnea evaluation in pediatric age may show great age-related variability...
April 5, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
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