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Posterior fossa syndrome

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https://www.readbyqxmd.com/read/28713626/recurrent-hemorrhage-in-hemangioblastoma-involving-the-posterior-fossa-case-report
#1
Eric Marvin, Asad S Akhter, Jeroen R Coppens
BACKGROUND: Hemangioblastomas (HGBs) are the most common primary intra-axial posterior fossa tumor in adults. Although spontaneous hemorrhage of these tumors is exceedingly rare, despite their vascular nature, we describe a case of recurrent hemorrhage with associated tonsillar herniation, and demonstrate that a surgical approach can provide a suitable outcome. CASE DESCRIPTION: A 54-year-old female with von Hippel-Lindau (VHL) syndrome presented with acute loss of consciousness and Glasgow Coma Scale (GCS) was 4...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28686325/rnf213-variants-in-a-child-with-phace-syndrome-and-moyamoya-vasculopathy
#2
Kala F Schilter, Jack E Steiner, Wendy Demos, Mohit Maheshwari, Jeremy W Prokop, Elizabeth Worthey, Beth A Drolet, Dawn H Siegel
Segmental infantile hemangiomas (IH) can be associated with congenital anomalies in a regional distribution. PHACE refers to large cervicofacial segmental IH in association with congenital anomalies of the aortic arch and medium-sized arteries of the head and neck, as well as structural anomalies of the posterior fossa and eye. A subset of PHACE patients have arterial anomalies that progress to moyamoya vasculopathy (MMV). MMV is defined as stenosis of the supraclinoid segment of the internal carotid arteries and/or their major branches, with subsequent development of a compensatory collateral vessel network...
July 7, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28685259/extremely-large-sinus-pericranii-with-involvement-of-the-torcular-and-associated-with-crouzon-s-syndrome
#3
Felipe H Sanders, Bryan A Edwards, Matthew Fusco, Rod J Oskouian, R Shane Tubbs, James M Johnston
INTRODUCTION: Sinus pericranii is a rare vascular malformation that connects the intracranial dural sinuses to the extracranial venous drainage system and is caused by either trauma or congenital defects. Although the majority of these vascular structures are due to trauma, some are congenital. CASE REPORT: Herein, we report a 5-month-old patient with a very large and fluctuating subcutaneous mass over the occiput and the diagnosis of Crouzon's syndrome. The child presented with a large midline mass that on imaging, connected to the underlying torcular and was diagnosed as a sinus pericranii...
July 6, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28665854/morphometric-analysis-of-the-posterior-cranial-fossa-in-syndromic-and-nonsyndromic-craniosynostosis
#4
Thomas Beez, David Koppel, Meharpal Sangra
Posterior cranial fossa (PCF) anatomy can be abnormal in craniosynostosis, and hindbrain herniation may occur. This study analyzed PCF anatomy in single suture and complex craniosynostosis.Children with craniosynostosis and age-matched controls were identified. Cephalic index (CI) for cranial vault and PCF as well as tentorial (TA) and occipital angles (OA) were measured on preoperative imaging.Children with syndromic (N = 6), bicoronal (N = 4), sagittal (N = 12), and metopic synostosis (N = 4) as well as controls (N = 10) were enrolled...
June 29, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28637445/cerebellar-mutism-syndrome-in-children-with-brain-tumours-of-the-posterior-fossa
#5
Morten Wibroe, Johan Cappelen, Charlotte Castor, Niels Clausen, Pernilla Grillner, Thora Gudrunardottir, Ramneek Gupta, Bengt Gustavsson, Mats Heyman, Stefan Holm, Atte Karppinen, Camilla Klausen, Tuula Lönnqvist, René Mathiasen, Pelle Nilsson, Karsten Nysom, Karin Persson, Olof Rask, Kjeld Schmiegelow, Astrid Sehested, Harald Thomassen, Ingrid Tonning-Olsson, Barbara Zetterqvist, Marianne Juhler
BACKGROUND: Central nervous system tumours constitute 25% of all childhood cancers; more than half are located in the posterior fossa and surgery is usually part of therapy. One of the most disabling late effects of posterior fossa tumour surgery is the cerebellar mutism syndrome (CMS) which has been reported in up to 39% of the patients but the exact incidence is uncertain since milder cases may be unrecognized. Recovery is usually incomplete. Reported risk factors are tumour type, midline location and brainstem involvement, but the exact aetiology, surgical and other risk factors, the clinical course and strategies for prevention and treatment are yet to be determined...
June 21, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28626825/phace-s-syndrome-report-of-a-case-with-new-ocular-and-systemic-manifestations
#6
Raheleh Assari, Vahid Ziaee, Sasan Moghimi, Mohammad Reza Akbari, Arash Mirmohammadsadeghi
PURPOSE: To describe an infant with PHACE(S) syndrome [posterior fossa anomalies (P), hemangiomas (H), arterial anomalies (A), cardiac abnormalities and coarctation of aorta (C), eye abnormalities (E), and the sternal defects (S)] with unusual strabismus, congenital glaucoma, and new systemic manifestations. METHODS: A 6-month-old girl was referred with large hemangiomas on the left side of the face. RESULTS: In the ocular examination, right esotropia and hypotropia, and limitation of elevation in adduction in the right eye were seen...
June 2017: Journal of Current Ophthalmology
https://www.readbyqxmd.com/read/28607814/chiari-i-malformation-and-syringomyelia-in-mucopolysaccharidosis-type-i-hurler-syndrome-treated-with-posterior-fossa-decompression-case-report-and-review-of-the-literature
#7
Vyacheslav Makler, Christina L Goldstein, Daniel Hoernschemeyer, Tomoko Tanaka
BACKGROUND: Hurler Syndrome is the most severe phenotype of mucopolysaccharidosis type I. With bone marrow transplant and enzyme replacement therapy, the life expectancy of a child with Hurler syndrome has been extended, predisposing them to multiple musculoskeletal issues most commonly involving the spine. CASE DESCRIPTION: This is the case report of a 6-year-old male with Hurler syndrome who was diagnosed with Chiari I malformation and cervicothoracic syringomyelia on a preoperative magnetic resonance imaging (MRI) for his thoracolumbar kyphosis...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28555453/-a-phenotypic-description-of-26-patients-with-ritscher-schinzel-syndrome-cranio-cerebello-cardiac-dysplasia-or-3c-syndrome
#8
S M Pira-Paredes, J H Montoya-Villada, J L Franco-Restrepo, M Moncada-Velez, J W Cornejo
INTRODUCTION: Ritscher-Schinzel syndrome (also known as cranio-cerebello-cardiac dysplasia or 3C syndrome) is a rare genetic syndrome that is mainly characterised by the association of cardiac and craniofacial anomalies together with others affecting the posterior fossa. PATIENTS AND METHODS: We report on 26 patients with Ritscher-Schinzel syndrome at a hospital in Medellin, in the Department of Antioquia, Colombia. RESULTS: Males account for 69% of this cohort...
June 1, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28547518/life-threatening-posterior-reversible-encephalopathy-syndrome-in-the-cerebellum-treated-by-posterior-fossa-decompression
#9
Yousef Abusabha, Athanasios K Petridis, Bastian Kraus, Marcel A Kamp, Hans-Jakob Steiger, Kerim Beseoglu
Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiological syndrome associated with various clinical conditions, such as headache, encephalopathy, and seizures. It is reversible if a prompt diagnosis is made and treatment undertaken. We report a 52-year-old male with hypertensive crisis. Progressing somnolence and an unresponsive left pupil occurred. MRI revealed an intra-axial hyperintensity of the cerebellum and brainstem and occlusive hydrocephalus suggestive of encephalitis or a tumor...
July 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28537352/is-pseudotumor-cerebri-an-unusual-expression-of-chiari-syndrome-a-case-report-and-review-of-the-literature
#10
Paolo Pacca, Roberto Altieri, Francesco Zenga, Diego Garbossa, Alessandro Ducati, Michele Lanotte
The Chiari I malformation (CM-I) is a developmental alteration of the posterior cranial fossa (PCF), radiographically defined as the descent of the cerebellar tonsils = 5 mm below the foramen magnum (FM) inside the cervical canal. Headache is the most frequent symptom associated with CM-I. The association of CM-I and neurological symptoms configures with Chiari syndrome. A rare symptom associated with Chiari syndrome is intracranial hypertension syndrome with cephalea and papilloedema-the typical findings of pseudotumor cerebri (PTC)...
May 24, 2017: Surgical Technology International
https://www.readbyqxmd.com/read/28522228/accessory-subscapularis-muscle%C3%A2-%C3%A2-a-forgotten-variation
#11
L A S Pires, C F C Souza, A R Teixeira, T F O Leite, M A Babinski, C A A Chagas
The quadrangular space is a space in the axilla bounded by the inferior margin of the teres minor muscle, the superior margin of the teres major muscle, the lateral margin of the long head of the triceps brachii muscle and the surgical neck of the humerus, medially. The axillary nerve (C5-C6) and the posterior circumflex humeral artery and veins pass through this space in order to supply their territories. The subscapularis muscle is situated into the scapular fossa and inserts itself into the lesser tubercle of the humerus, thus helping stabilize the shoulder joint...
May 15, 2017: Morphologie: Bulletin de L'Association des Anatomistes
https://www.readbyqxmd.com/read/28515557/dandy-walker-variant-with-schizophrenia-comorbidity-or-cerebellar-cognitive-affective-syndrome
#12
Pallavi Sinha, Jatin Tarwani, Pankaj Kumar, Amit Garg
Dandy-Walker complex (DWC) is a series of neurodevelopmental anomalies involving the posterior cranial fossa. The cerebellum has long been considered to be involved in motor coordination and balance. However, it has now been noted to play an important role in higher order cognitive, emotional, and behavioral functions. The concept of cerebellar cognitive affective syndrome, describing a coherent spectrum of cognitive and behavioral disturbances in adults following cerebellar damage has long been proposed. There have been reported cases of co-occurring psychiatric symptoms and DWC in literature, but the conclusive evidence for an association between the same remains lacking...
March 2017: Indian Journal of Psychological Medicine
https://www.readbyqxmd.com/read/28512124/use-of-propranolol-in-a-remote-region-of-rural-guatemala-to-treat-a-large-facial-infantile-haemangioma
#13
Vera Goldberg, Boris Martinez, Katia Cnop, Peter Rohloff
We present a female infant with a right-sided facial and neck haemangioma, from a remote, resource-poor community in rural Guatemala. She received first-line treatment, propranolol, with marked reduction in tumour size and erythema. Treatment was stopped after 35 weeks due to recurrent diarrhoea and sustained weight loss. Propranolol can be used to safely treat infants with haemangiomas in remote, rural communities if there is adequate follow-up, education and communication. Periocular haemangiomas should be treated promptly to avoid visual impairment...
May 16, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28488069/bobble-head-doll-syndrome-in-an-80-year-old-man-associated-with-a-giant-arachnoid-cyst-of-the-lamina-quadrigemina-treated-with-endoscopic-ventriculocystocisternotomy-and-cystoperitoneal-shunt
#14
Jorge Octavio Olvera-Castro, Hugo Morales-Briceño, Bayron Sandoval-Bonilla, David Gallardo-Ceja, Miguel Angel Venegas-Cruz, Eric Misael Estrada-Estrada, Marisol Contreras-Mota, Gerardo Guinto-Balanzar, Rabindranath Garcia-Lopez
Bobble-head doll syndrome (BHDS) is a rare entity, characterized by antero-posterior head bobbing, which is of the type "yes-yes." Less frequently, having a head movement of the type "no-no" is described. We report an unusual case of an 80-year-old man with a cystic mass of the lamina quadrigemina, extending to the posterior fossa. We conclude that ventriculocystocisternotomy associated with a cystoperitoneal shunt is an effective treatment for a symptomatic giant arachnoid cyst in the lamina quadrigemina.
May 9, 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28468145/experiences-in-performing-posterior-calvarial-distraction
#15
Kevin McMillan, Mark Lloyd, Martin Evans, Nicholas White, Hiroshi Nishikawa, Desiderio Rodrigues, Melanie Sharp, Pete Noons, Guirish Solanki, Stephen Dover
The use of posterior calvarial distraction (PCD) for the management of craniosynostosis is well recognized. The advantages of using this technique include increased cranial volume, decreased intracranial pressure, relief of posterior fossa crowding, improved cerebrospinal fluid (CSF) circulation at the cranio-cervical junction with cessation, and possible resolution of syrinx.The authors retrospectively review their first 50 patients who have undergone PCD under the senior author's care in our unit.The demographics, diagnoses, intraoperative approach with techniques in distractor placement and outcomes of each patient were obtained through an electronic craniofacial database and written patient records...
May 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28408631/asymmetric-meckel-cave-enlargement-a-potential-marker-of-phaces-syndrome
#16
J N Wright, V Wycoco
BACKGROUND AND PURPOSE: PHACES syndrome is a complex of morphologic abnormalities of unknown cause and includes posterior fossa abnormalities; head and neck infantile hemangiomas; arterial, cardiac, and eye anomalies; and sternal or abdominal wall defects. Accurate identification of the syndrome is important for optimal treatment. The purpose of this study was to investigate the incidence of asymmetric Meckel cave enlargement, a potential novel imaging marker, in a population of patients referred for evaluation of possible PHACES syndrome...
April 13, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28405869/long-term-neuropsychological-follow-up-of-young-children-with-medulloblastoma-treated-with-sequential-high-dose-chemotherapy-and-irradiation-sparing-approach
#17
Taryn B Fay-McClymont, Danielle M Ploetz, Don Mabbott, Karin Walsh, Amy Smith, Susan N Chi, Elizabeth Wells, Jennifer Madden, Ashley Margol, Jonathan Finlay, Mark W Kieran, Douglas Strother, Girish Dhall, Roger J Packer, Nicholas K Foreman, E Bouffet, Lucie Lafay-Cousin
High-dose chemotherapy (HDC) strategies were developed in brain tumor protocols for young children to prevent neuropsychological (NP) impairments associated with radiotherapy. However, comprehensive NP evaluations of these children treated with such strategies remain limited. We examined the long-term neurocognitive outcomes of young children (<6 years) with medulloblastoma, treated similarly, with a HDC strategy "according to" the chemotherapy regimen of the protocol CCG 99703. This retrospective study included young children less than 6 years of age at diagnosis of medulloblastoma treated from 1998 to 2011 at 7 North American institutions...
April 12, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28397227/-prenatal-diagnosis-and-follow-up-of-a-case-with-lowe-syndrome-caused-by-interstitial-deletion-of-xq25-26
#18
Xiangyu Zhu, Jie Li, Tong Ru, Ruifang Zhu, Chenyan Dai, Wanjun Wang, Yali Hu
OBJECTIVE: To report on a sporadic case of Lowe syndrome diagnosed prenatally with ultrasound examination and genetic testing. METHODS: Detailed sonographic fetal screening was performed by an experienced sonographer at 32 weeks of gestation. Fetal cranial magnetic resonance imaging (MRI) was applied to detect potential brain abnormality. Chromosomal microarray analysis (CMA) was conducted on amniotic fluid sample from the fetus and peripheral blood sample from the mother...
April 10, 2017: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/28337694/the-posterior-fossa-and-foreign-accent-syndrome-report-of-two-new-cases-and-review-of-the-literature
#19
Stefanie Keulen, Peter Mariën, Kim van Dun, Roelien Bastiaanse, Mario Manto, Jo Verhoeven
Foreign accent syndrome is a rare motor speech disorder that causes patients to speak their language with a non-native accent. In the neurogenic condition, the disorder develops after lesions in the language dominant hemisphere, often affecting Broca's area, the insula, the supplementary motor area and the primary motor cortex. Here, we present two new cases of FAS after posterior fossa lesions. The first case is a 44-year-old, right-handed, Dutch-speaking man who suffered motor speech disturbances and a left hemiplegia after a pontine infarction...
March 23, 2017: Cerebellum
https://www.readbyqxmd.com/read/28318729/acute-cerebellitis-in-paediatric-patients-our-experience
#20
J P García-Iñiguez, F J López-Pisón, P Madurga Revilla, I Montejo Gañán, M Domínguez Cajal, L Monge Galindo, S B Sánchez Marco, M C García Jiménez
INTRODUCTION: Acute cerebellitis is a rare inflammatory disease with a highly variable clinical course that ranges from benign self-limiting symptoms to a fulminant presentation associated with a high risk of death due to compression of the posterior fossa, acute hydrocephalus, and intracranial hypertension. METHODS: We reviewed clinical, laboratory, and radiological findings from children diagnosed with acute cerebellitis between May 2007 and November 2016. We analysed treatments and clinical and radiological progression...
March 15, 2017: Neurología: Publicación Oficial de la Sociedad Española de Neurología
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