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Posterior fossa syndrome

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https://www.readbyqxmd.com/read/27873347/infantile-hemangioma-with-minimal-or-arrested-growth-further-observations-on-clinical-and-histopathologic-findings-of-this-unique-but-underrecognized-entity
#1
Ellen Hui Ma, Susan J Robertson, Chung W Chow, Philip S Bekhor
BACKGROUND: Infantile hemangioma (IH) with minimal or arrested growth (IH-MAG) is becoming increasingly recognized in the literature. It is important to be aware of their existence, because the correct diagnosis is essential for prognostication and treatment and, in the case of facial segmental lesions, the direction of further investigations if PHACE (posterior fossa abnormalities and other structural brain abnormalities; hemangioma(s) of the cervical facial region; arterial cerebrovascular anomalies; cardiac defects, aortic coarctation, and other aortic abnormalities; eye anomalies) syndrome or Sturge-Weber syndrome is suspected...
November 22, 2016: Pediatric Dermatology
https://www.readbyqxmd.com/read/27872228/child-neurology-diencephalic-syndrome-like-presentation-of-a-cervicomedullary-brainstem-tumor
#2
Melanie Conway, Resham Ejaz, Elizabeth Kouzmitcheva, Deena Savlov, James T Rutka, Mahendranath Moharir
Diencephalic syndrome is a rare clinical entity, traditionally encompassing severe failure to thrive, nystagmus, and hyperkinesis, secondary to an intracranial neoplasm that is classically located in the hypothalamic region and its vicinity. However, the presenting features can be variable, often resulting in delayed diagnosis, which may worsen prognosis. This case report describes the atypical presentation of a posterior fossa tumor with features reminiscent of diencephalic syndrome that have not previously been reported in the literature...
November 22, 2016: Neurology
https://www.readbyqxmd.com/read/27865668/a-small-pons-as-a-characteristic-finding-in-down-syndrome-a-quantitative-mri-study
#3
Yuta Fujii, Noriko Aida, Tetsu Niwa, Mikako Enokizono, Kumiko Nozawa, Tomio Inoue
BACKGROUND: Down syndrome (DS) is the most common chromosomal aberration, but the characteristics of the brainstem component in this condition during childhood (from newborn to preteen stages) have not been clarified. OBJECTIVE: To evaluate the morphological features of the brainstem in DS on magnetic resonance imaging (MRI). MATERIALS AND METHODS: MRIs for 32 children with DS (16 boys and girls each; age range, 0-11years) without major brain insults, and 32 age-matched controls (16 boys and girls each) were retrospectively analyzed...
November 16, 2016: Brain & Development
https://www.readbyqxmd.com/read/27830331/posterior-fossa-syndrome-with-delayed-mr-evidence-of-unilateral-superior-cerebellar-peduncle-scp-damage
#4
Kevin Carr, Pegah Ghamasaee, Achint Singh, Izabela Tarasiewicz
Posterior fossa syndrome (PFS) is a well-known sequela of midline posterior fossa tumor resection. Patients typically exhibit transient behavioral, motor, and oculomotor disturbances that resolve within a few weeks to several months after surgery. The underlying pathophysiology of PFS is not completely understood, but contemporary literature has implicated injury to the dentate nucleus and/or exiting dentatothalamocortical fiber bundles as a causative factor. The authors present a case of a young male who developed a delayed variant of PFS typified by motor deficits and demonstrated diffusion restriction in the ipsilateral superior cerebellar peduncle...
November 9, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27810194/-ectopic-craniopharyngioma-and-gardner-s-syndrome-case-report-and-literature-review
#5
Juan Antonio Álvarez Salgado, Francisco González-Llanos Fernández de Mesa, Jorge Javier Villaseñor Ledezma, Maria de Los Angeles Cañizares Méndez, Igor Paredes Sansinenea, Angel Rodríguez de Lope-Llorca, Manuela Mollejo Villanueva
INTRODUCTION: Craniopharyngioma accounts for around 3% of all primary tumours of the central nervous system. It is usually located in the suprasellar region, although it may also have an ectopic location. CASE REPORT: The case is presented on 29 year-old male who underwent surgery for a jaw osteoma when he was 19 years old and was subsequently diagnosed with Gardner's syndrome. He was admitted in our Hospital with right facial paresis and diplopia of one day onset...
October 31, 2016: Neurocirugía
https://www.readbyqxmd.com/read/27808032/-acute-surgical-treatment-of-malignant-stroke
#6
Alexander Lilja-Cyron, Vagn Eskesen, Klaus Hansen, Daniel Kondziella, Jesper Kelsen
Malignant stroke is an intracranial herniation syndrome caused by cerebral oedema after a large hemispheric or cerebellar stroke. Malignant middle cerebral artery infarction is a devastating disease with a mortality around 80% despite intensive medical treatment. Decompressive craniectomy reduces mortality and improves functional outcome - especially in younger patients (age ≤ 60 years). Decompression of the posterior fossa is a life-saving procedure in patients with malignant cerebellar infarctions and often leads to good neurological outcome...
October 24, 2016: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/27787875/posterior-fossa-syndrome-review-of-the-behavioral-and-emotional-aspects-in-pediatric-cancer-patients
#7
REVIEW
Jane C Lanier, Annah N Abrams
Medulloblastoma, the most common malignant brain tumor of childhood, occurs in the posterior fossa, the part of the intracranial cavity that contains the brainstem and the cerebellum. The cerebellum is involved in many complex aspects of human behavior and function, and when it is disrupted or insulted, this can lead to significant sequelae in children with posterior fossa tumors. A constellation of impairing and distressing symptoms, including mutism, ataxia/hypotonia, and emotional lability, develops in approximately 25% of children after the surgical resection of posterior fossa tumors...
October 27, 2016: Cancer
https://www.readbyqxmd.com/read/27785688/dti-fiber-tractography-of-cerebro-cerebellar-pathways-and-clinical-evaluation-of-ataxia-in-childhood-posterior-fossa-tumor-survivors
#8
Myung Eun Oh, Pablo Hernáiz Driever, Rajiv K Khajuria, Stefan Mark Rueckriegel, Elisabeth Koustenis, Harald Bruhn, Ulrich-Wilhelm Thomale
Pediatric posterior fossa (PF) tumor survivors experience long-term motor deficits. Specific cerebrocerebellar connections may be involved in incidence and severity of motor dysfunction. We examined the relationship between long-term ataxia as well as fine motor function and alteration of differential cerebellar efferent and afferent pathways using diffusion tensor imaging (DTI) and tractography. DTI-based tractography was performed in 19 patients (10 pilocytic astrocytoma (PA) and 9 medulloblastoma patients (MB)) and 20 healthy peers...
October 26, 2016: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/27749392/combined-ultrasound-and-exome-sequencing-approach-recognizes-opitz-g-bbb-syndrome-in-two-malformed-fetuses
#9
Anna Maria Pinto, Valentina Imperatore, Laura Bianciardi, Margherita Baldassarri, Paolo Galluzzi, Simone Furini, Giovanni Centini, Alessandra Renieri, Francesca Mari
Orofacial clefts are the most common congenital craniofacial anomalies and can occur as an isolated defect or be associated with other anomalies such as posterior fossa anomalies as a part of several genetic syndromes. We report two consecutive voluntary pregnancy interruptions in a nonconsanguineous couple following the fetal ultrasound finding of cleft lip and palate and posterior fossa anomalies confirmed by means of post-termination examination on the second fetus. The quantitative fluorescent PCR, the karyotype, and the comparative genomic hybridization-array analysis after amniocentesis were normal...
October 4, 2016: Clinical Dysmorphology
https://www.readbyqxmd.com/read/27741106/a-method-to-preserve-limbus-during-penetrating-keratoplasty-for-a-case-of-presumed-phaces-syndrome-with-sclerocornea-a-case-report
#10
Yi-Ju Ho, Hung-Chi Chen, Shirley H L Chang, Lung-Kung Yeh, David Hui-Kang Ma
BACKGROUND: Sclerocornea, a congenital corneal pathology characterized by bilateral scleralization of the cornea, which can be found in few cases with posterior fossa malformationshemangiomas-arterial anomalies-cardiac defects-eye abnormalities-sternal cleft and supraumbilical raphe (PHACES) syndrome. Presence of vascularization in peripheral cornea and smaller diameter of recipient cornea correlate to poor outcome of penetrating keratoplasty (PKP) in sclerocornea. Here we report a method to preserve limbus during PKP for small, irregular, and scleralized cornea...
October 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27738723/-anatomy-and-malformations-of-the-posterior-cranial-fossa
#11
T Struffert
Many important structures are located in the confined space within the posterior cranial fossa. This article describes the main aspects of the anatomy. As a uniform classification of malformations of the posterior cranial fossa does not exist the main syndromes, such as Chiari malformations, zerebellar hypoplasia and dysplasia are discussed separately.
October 13, 2016: Der Radiologe
https://www.readbyqxmd.com/read/27689022/longitudinal-cerebellar-diffusion-tensor-imaging-changes-in-posterior-fossa-syndrome
#12
Sean D McEvoy, Amy Lee, Andrew Poliakov, Seth Friedman, Dennis Shaw, Samuel R Browd, Richard G Ellenbogen, Jeffrey G Ojemann, Christine L Mac Donald
Posterior fossa syndrome is a severe transient loss of language that frequently complicates resection of tumors of the cerebellum. The associated pathophysiology and relevant anatomy to this language deficit remains controversial. We performed a retrospective analysis of all cerebellar tumor resections at Seattle Children's Hospital from 2010 to 2015. Diffusion tensor imaging was performed on each of the patients as part of their clinical scan. Patients included in the study were divided into groups based on language functioning following resection: intact (N = 19), mild deficit (N = 19), and posterior fossa syndrome (N = 9)...
2016: NeuroImage: Clinical
https://www.readbyqxmd.com/read/27644020/progressive-lesions-of-central-nervous-system-in-microcephalic-fetuses-with-suspected-congenital-zika-virus-syndrome
#13
Manoel Sarno, Marcelo Aquino, Kleber Pimentel, Renata Cabral, Gisela Costa, Fernanda Bastos, Carlos Brites
OBJECTIVE: Our aim was to describe the pattern and progression of Central Nervous System (CNS) lesions of microcephalic fetuses with suspected Zika Virus (ZIKV) infection. METHODS: We analyzed fetuses diagnosed with microcephaly after a routine primary care ultrasound scan suspect a fetal microcephaly in Salvador, Brazil, from July/2015 to February/2016 and these pregnancies were followed until delivery. Microcephaly was diagnosed when the head circumference was below two or more standard deviation for gestational age and its relationship with ZIKV infection was defined according to World Health Organization criteria...
September 19, 2016: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/27530801/-research-advances-of-posterior-reversible-encephalopathy-syndrome-in-children
#14
Jing Liu, Jiong Qin
Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiological entity affecting the posterior brain, i.e. occipital and parietal lobes. The syndrome are characterized by headaches, altered mental status, seizures, and visual disturbances. Although the pathogenesis remains unclear, endothelial dysfunction may be a key factor. The basic disease may play a crucial role in the incidence of PRES. In most cases, PRES resolves spontaneously and patients show both clinical and radiological improvements...
August 2016: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/27432679/cerebellar-disorders-clinical-radiologic-findings-and-modern-imaging-tools
#15
Mario Manto, Christophe Habas
Cerebellar disorders, also called cerebellar ataxias, comprise a large group of sporadic and genetic diseases. Their core clinical features include impaired control of coordination and gait, as well as cognitive/behavioral deficits usually not detectable by a standard neurologic examination and therefore often overlooked. Two forms of cognitive/behavioral syndromes are now well identified: (1) the cerebellar cognitive affective syndrome, which combines an impairment of executive functions, including planning and working memory, deficits in visuospatial skills, linguistic deficiencies such as agrammatism, and inappropriate behavior; and (2) the posterior fossa syndrome, a very acute form of cerebellar cognitive affective syndrome occurring essentially in children...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27344090/basilar-artery-dissection-a-rare-complication-of-posterior-fossa-epidermoid-cyst-resection-and-evaluation-of-the-possible-effects-of-cerebrospinal-fluid-drainage-on-disease-progression
#16
Stylianos Pikis, José E Cohen, Emil Margolin
We report a rare case of a 45-year-old female with an unruptured basilar artery dissecting aneurysm presenting with locked-in syndrome due to brainstem ischemia eleven months following resection of a giant cerebellopontine angle epidermoid cyst and three months after insertion of ventriculo peritoneal shunt due to hydrocephalus. The etiology of basilar artery dissection and the effect of hydrocephalus and ventricular cerebrospinal fluid drainage on disease progression in this patient are unclear. Our report suggests a possible effect of hydrocephalus and ventricular cerebrospinal fluid drainage on intracranial arterial dissection progression...
October 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/27291925/neurodevelopmental-outcomes-in-children-with-phace-syndrome
#17
Cheryl L Brosig, Dawn H Siegel, Anita N Haggstrom, Ilona J Frieden, Beth A Drolet
BACKGROUND: Practitioners who work with children with posterior fossa, facial hemangiomas, arterial anomalies, cardiovascular anomalies, and abnormalities of the eye (PHACE) syndrome need information about neurodevelopmental outcomes to provide appropriate anticipatory guidance and education for parents. This study aimed to determine the neurodevelopmental outcomes in children with PHACE syndrome and identify which children may be at greatest risk for delays. METHODS: Children with a diagnosis of PHACE syndrome (ages 4-18 yrs) were recruited from the PHACE Syndrome International Clinical Registry and Genetic Repository...
July 2016: Pediatric Dermatology
https://www.readbyqxmd.com/read/27260839/randomized-controlled-trial-of-talactoferrin-oral-solution-in-preterm-infants
#18
Michael P Sherman, David H Adamkin, Victoria Niklas, Paula Radmacher, Jan Sherman, Fiona Wertheimer, Karel Petrak
OBJECTIVE: To evaluate the safety and explore the efficacy of recombinant human lactoferrin (talactoferrin [TLf]) to reduce infection. STUDY DESIGN: We conducted a randomized, double blind, placebo-controlled trial in infants with birth weight of 750-1500 g. Infants received enteral TLf (n = 60) or placebo (n = 60) on days 1 through 28 of life; the TLf dose was 150 mg/kg every 12 hours. Primary outcomes were bacteremia, pneumonia, urinary tract infection, meningitis, and necrotizing enterocolitis (NEC)...
August 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27215002/ophthalmic-profile-and-systemic-features-of-pediatric-facial-nerve-palsy
#19
Preeti Patil-Chhablani, Sowmya Murthy, Meenakshi Swaminathan
BACKGROUND: Facial nerve palsy (FNP) occurs less frequently in children as compared to adults but most cases are secondary to an identifiable cause. These children may have a variety of ocular and systemic features associated with the palsy and need detailed ophthalmic and systemic evaluation. METHODS: This was a retrospective chart review of all the cases of FNP below the age of 16 years, presenting to a tertiary ophthalmic hospital over the period of 9 years, from January 2000 to December 2008...
December 2015: Eye Science
https://www.readbyqxmd.com/read/27157279/acute-foramen-magnum-syndrome-following-single-diagnostic-lumbar-puncture-consequence-of-a-small-posterior-fossa
#20
Amandeep Kumar, Mohit Agrawal, Surya Prakash, Shambanduram Somorendra, Pankaj Kumar Singh, Ajay Garg, Manmohan Singh, Bhawani Shanker Sharma
BACKGROUND: Type I Chiari malformation (CMI) is a rare complication of lumbar cerebrospinal fluid (CSF) drainage that is usually reported after lumbar drain or lumboperitoneal shunt placement. It usually remains asymptomatic; however, even if it becomes symptomatic, symptoms are usually mild. There are only a few reports of acute foramen magnum syndrome following continuous lumbar CSF drainage, and acute foramen magnum syndrome after a single diagnostic lumbar puncture (LP) has not been previously reported...
July 2016: World Neurosurgery
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