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Posterior fossa syndrome

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https://www.readbyqxmd.com/read/29445946/paravertebral-tumours-of-the-cervicothoracic-junction-extending-into-the-mediastinum-surgical-strategies-in-a-no-man-s-land
#1
Georgios K Prezerakos, Parag Sayal, Antonios Kourliouros, Periclis Pericleous, George Ladas, Adrian Casey
PURPOSE: Cervicothoracic paravertebral neoplasms extending into the mediastinum pose a surgical challenge due the complex regional anatomy, their biological nature, rarity and surgeon's unfamiliarity with the region. We aim to define a surgical access framework addressing the aforementioned complexities whilst achieving oncological clearance. METHODS: We carried out a retrospective review of 28 consecutive patients operated in two tertiary referral centres between 1998 and 2015...
February 14, 2018: European Spine Journal
https://www.readbyqxmd.com/read/29436723/variable-prenatal-presentation-of-pfeiffer-syndrome-suggested-aids-to-prenatal-sonographic-diagnosis
#2
Souha Saliba, Baptiste Morel, Marie Gonzales, Marie-Victoire Sénat, Lucie Guilbaud, Jean-Marie Jouannic, Marie Cassart, Catherine Garel, Eléonore Blondiaux
BACKGROUND: Our purpose was to describe and compare the cranial and extracranial abnormalities of Pfeiffer syndrome on prenatal imaging with postnatal or postmortem findings, which may help in prenatal diagnosis of Pfeiffer syndrome (PS). METHODS: Cases of fetuses with a confirmed diagnosis of PS over a 4-year period (2012-2016) were retrospectively reviewed. Prenatal imaging findings, postnatal or postmortem investigations and genetic test results were analyzed...
February 13, 2018: Prenatal Diagnosis
https://www.readbyqxmd.com/read/29433337/development-of-a-pre-operative-scoring-system-for-predicting-risk-of-post-operative-paediatric-cerebellar-mutism-syndrome
#3
Jo-Fen Liu, Robert A Dineen, Shivaram Avula, Tom Chambers, Manali Dutta, Tim Jaspan, Donald C MacArthur, Simon Howarth, Daniele Soria, Philip Quinlan, Srikrishna Harave, Chan Chang Ong, Conor L Mallucci, Ram Kumar, Barry Pizer, David A Walker
BACKGROUND: Despite previous identification of pre-operative clinical and radiological predictors of post-operative paediatric cerebellar mutism syndrome (CMS), a unifying pre-operative risk stratification model for use during surgical consent is currently lacking. The aim of the project is to develop a simple imaging-based pre-operative risk scoring scheme to stratify patients in terms of post-operative CMS risk. METHODS: Pre-operative radiological features were recorded for a retrospectively assembled cohort of 89 posterior fossa tumour patients from two major UK treatment centers (age 2-23yrs; gender 28 M, 61 F; diagnosis: 38 pilocytic astrocytoma, 32 medulloblastoma, 12 ependymoma, 1 high grade glioma, 1 pilomyxoid astrocytoma, 1 atypical teratoid rhabdoid tumour, 1 hemangioma, 1 neurilemmoma, 2 oligodendroglioma)...
February 12, 2018: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/29407505/role-of-cone-beam-computed-tomography-with-a-large-field-of-view-in-goldenhar-syndrome
#4
Cosimo Nardi, Luisa De Falco, Valeria Selvi, Chiara Lorini, Linda Calistri, Stefano Colagrande
INTRODUCTION: Goldenhar syndrome is a rare disease with hemifacial microsomia and craniofacial disorders originating from the first and second branchial arches, such as ocular, auricular, and vertebral anomalies. The complexity and variety of the ways in which the disease presents itself usually need several examinations. In this study, we aimed to evaluate both craniofacial and vertebral skeletal anomalies and asymmetries between the nonaffected and affected sides in patients with Goldenhar syndrome by using cone-beam computed tomography...
February 2018: American Journal of Orthodontics and Dentofacial Orthopedics
https://www.readbyqxmd.com/read/29405773/brain-infarction-due-to-vertebral-artery-dissection-caused-by-a-bone-protrusion-from-the-condylar-fossa-in-a-juvenile-case
#5
Mutsumi Fujii, Miki Ohgushi, Takaaki Chin
A 16-year-old boy presented with multiple posterior circulation ischemic strokes resulting from vertebral artery (VA) dissection. Three-dimensional computed tomography showed aberrant sub-occipital bone protuberance, proximal to the VA dissection. Since the patient was a habitual neck cracker, VA dissection was thought to result from the impact shock of the rotational head movement. This could be due to either the osseous prominence or the compression between the prominence and the C1. Although it is a rare etiology of Bow Hunter's syndrome, VA dissection due to sub-occipital bone spur because of neck cracking should be considered in the diagnosis of Bow Hunter's syndrome in juvenile patients...
February 6, 2018: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/29403363/a-retrospective-2d-morphometric-analysis-of-adult-female-chiari-type-i-patients-with-commonly-reported-and-related-conditions
#6
Maggie S Eppelheimer, James R Houston, Jayapalli R Bapuraj, Richard Labuda, Dorothy M Loth, Audrey M Braun, Natalie J Allen, Soroush Heidari Pahlavian, Dipankar Biswas, Aintzane Urbizu, Bryn A Martin, Cormac O Maher, Philip A Allen, Francis Loth
Purpose: Researchers have sought to better understand Chiari type I malformation (CMI) through morphometric measurements beyond tonsillar position (TP). Soft tissue and bone structures within the brain and craniocervical junction have been shown to be different for CMI patients compared to healthy controls. Yet, several morphological characteristics have not been consistently associated with CMI. CMI is also associated with different prevalent conditions (PCs) such as syringomyelia, pseudotumor, Ehlers-Danlos syndrome (EDS), scoliosis, and craniocervical instability...
2018: Frontiers in Neuroanatomy
https://www.readbyqxmd.com/read/29395695/a-new-diagnostic-approach-to-the-adult-patient-with-acute-dizziness
#7
Jonathan A Edlow, Kiersten L Gurley, David E Newman-Toker
BACKGROUND: Dizziness, a common chief complaint, has an extensive differential diagnosis that includes both benign and serious conditions. Emergency physicians must distinguish the majority of patients with self-limiting conditions from those with serious illnesses that require acute treatment. OBJECTIVE OF THE REVIEW: This article presents a new approach to diagnosis of the acutely dizzy patient that emphasizes different aspects of the history to guide a focused physical examination with the goal of differentiating benign peripheral vestibular conditions from dangerous posterior circulation strokes in the emergency department...
January 31, 2018: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29386918/bilateral-total-retinal-detachment-at-birth-a-case-report-of-walker-warburg-syndrome
#8
Navid Hakim, Cristina Soare, Jamil Hakim
Walker-Warburg syndrome (WWS) is a disorder characterized by ocular and brain malformations, and congenital muscular dystrophy. Retinal malformations are common in WWS; however, bilateral retinal detachment is a rare occurrence. We present a case of a newborn baby delivered at 36+3 weeks, who was the first living child of consanguineous parents of Turkish origin. On antenatal anomaly scans, the fetus had hydrocephalus that had increased throughout pregnancy, and a diagnosis of hydrancephaly was made at 36 weeks of gestation...
2018: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/29354754/spinal-subdural-hematoma-post-foramen-magnum-decompression-rare-complication-in-a-patient-with-rhomboencephalosynapsis
#9
Kapil Mohan Rajwani, Anastasios Giamouriadis, Pawanjit Singh Minhas
Spinal subdural hematoma (SSDH) as a complication of posterior fossa surgery is extremely rare and can have potentially serious consequences. We report a case of delayed extensive SSHD presenting as cauda equina syndrome (CES) following foramen magnum decompression and occipito-cervical (OC) fusion.
December 2017: Journal of Spine Surgery (Hong Kong)
https://www.readbyqxmd.com/read/29318978/cognitive-rehabilitation-of-cerebellar-deficits
#10
Peter Marien, Kim van Dun, Frank Van Overwalle
During the past 3 decades numerous neurophysiological, neuroimaging, experimental and clinical studies have evidenced a crucial role for the cerebellum in cognitive, affective and behavioral functions. As a result of the acknowledged modulatory role of the cerebellum upon remote structures such as the cerebral cortex, cerebellar injury may give rise to a constellation of behavioral, affective and cognitive symptoms (Schmahmann's Syndrome). In sharp contrast to the wide range of therapeutical interventions to treat cognitive and affective disorders following cerebral cortical lesions and despite the consequences of Schmahmann's syndrome upon daily life activities, the literature is surprisingly only scantly documented with studies investigating the impact of cognitive therapies on cerebellar induced cognitive and affective disorders...
January 10, 2018: CNS & Neurological Disorders Drug Targets
https://www.readbyqxmd.com/read/29298741/-posterior-fossa-infarct-misdiagnosed-as-acute-peripheral-vestibulopathy
#11
Karen Stepanidis, Mads Klokker
Patients with acute central vestibular syndrome (AVS) can mimic acute peripheral vestibulopathy, which can mislead to a diagnosis of posterior fossa infarcts. Delayed diagnosis will prevent relevant treatment and may lead to severe disability and in worst case death. Understanding of AVS is extremely relevant for physicians in hospital and prehospital care to insure the right treatment. I this case report of a 35-year-old male patient with AVS the correct diagnosis was made relatively late.
January 1, 2018: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/29279964/hypertrophic-olivary-degeneration-in-children-after-posterior-fossa-surgery-an-underdiagnosed-condition
#12
Matheus Fernando Manzolli Ballestero, Dinark Conceição Viana, Thiago Lyrio Teixeira, Marcelo Volpon Santos, Ricardo Santos de Oliveira
BACKGROUND: Hypertrophic olivary degeneration (HOD) is a rare transsynaptic form of degeneration occurring after injury to the dentato-rubro-olivary pathway ("Guillain-Mollaret triangle"). The majority of studies have described HOD resulting from posterior fossa (PF) hemorrhage or infarction. HOD in patients undergoing PF surgery has not been well characterized. These lesions are rare and symptomatic children with HOD are even more uncommon. The purpose of this study was to evaluate HOD that develops after PF operations in children...
December 26, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29250697/two-girl-patients-with-medulloblastoma-case-reports
#13
Laura Daniela Marinău, Cristina Elena Singer, Cristian Meşină, Elena Carmen Niculescu, Ileana Puiu, Ileana Octavia Petrescu, Cristiana Geormăneanu, Augustina Cornelia Enculescu, Daniela Elise Tache, Ştefana Oana Purcaru, Simona Răciulă, Cosmina Lucia Damian
In childhood, the most common type of brain tumors is medulloblastoma, a highly malignant primary brain tumor that is found in the cerebellum or posterior fossa. The tumor mass increases and generates obstructive hydrocephalus. Risk factors (that might be involved in some cases) include the genetic syndrome such as type 1 neurofibromatosis, exposure to ionizing radiation and Epstein-Barr virus. Medulloblastoma is associated with recessively inherited Turcot disease and with conditions as ataxia-telangiectasia syndrome in several cases...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/29250689/anatomic-variants-in-dandy-walker-complex
#14
Maria Claudia Jurcă, Kinga Kozma, CodruŢa Diana Petcheşi, Marius Bembea, Ovidiu Laurean Pop, Gabriela MuŢiu, Mihaela Cristiana Coroi, Alexandru Daniel Jurcă, Luciana Dobjanschi
Dandy-Walker complex (DWC) is a malformative association of the central nervous system. DWC includes four different types: Dandy-Walker malformation (vermis agenesis or hypoplasia, cystic dilatation of the fourth ventricle and a large posterior fossa); Dandy-Walker variant (vermis hypoplasia, cystic dilatation of the fourth ventricle, normal posterior fossa); mega cysterna magna (large posterior fossa, normal vermis and fourth ventricle) and posterior fossa arachnoid cyst. We present and discuss four cases with different morphological and clinical forms of the Dandy-Walker complex...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/29224766/reversible-profound-sensorineural-hearing-loss-due-to-propranolol-sensitive-hemangioma-in-an-infant-with-phace-syndrome
#15
John N Bangiyev, Richard Gurgel, Sheryll L Vanderhooft, J Fredrik Grimmer
PHACE syndrome is the association of large or segmental infantile hemangiomas of the face or scalp with abnormalities within the posterior fossa, arteries, cardiovascular system, and eyes. We present a case of reversible profound sensorineural hearing loss due to a cerebellopontine angle infantile hemangioma that was successfully treated with propranolol.
December 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29191538/collet-sicard-syndrome-attributable-to-extramedullary-plasmacytoma-of-the-jugular-foramen
#16
Soliman Oushy, Christopher S Graffeo, Avital Perry, Jonathan M Morris, Matthew L Carlson, Jamie J Van Gompel
BACKGROUND: Collet-Sicard syndrome is a rare manifestation of skull base disease involving the jugular and hypoglossal foramina. We report the first case of Collet-Sicard attributable to extramedullary plasmacytoma-multiple myeloma (EP-MM), as well as the second case of EP-MM precipitating a jugular foramen syndrome (JFS)-spectrum disorder. CASE DESCRIPTION: A fifty-nine-year-old woman presented with 4-months of left aural fullness and pulsatile tenderness, positional vertigo, hoarseness, and dysphagia...
November 27, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29180084/cerebellar-mutism-syndrome-and-other-complications-after-surgery-in-the-posterior-fossa-in-adults-a-prospective-study
#17
Morten Wibroe, Per Rochat, Marianne Juhler
BACKGROUND: Cerebellar mutism syndrome (CMS) is rarely described in adults; however, data on self-assessed linguistic complications after posterior fossa surgery do not exist. METHODS: Through a prospective single-center study, data on 59 tumor operations in the posterior fossa were collected preoperatively as well as 1 week and 1 month postoperatively. Data on self-assessed problems in 5 CMS-related domains, CMS scores, and neurology as well as surgical procedure and complications were obtained...
November 24, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29173061/targeted-sequencing-of-malignant-supratentorial-pediatric-brain-tumors-demonstrates-a-high-frequency-of-clinically-relevant-mutations
#18
Bonnie L Cole, Colin C Pritchard, Maia Anderson, Sarah Es Leary
Pediatric brain tumors cause more deaths than any other childhood malignancy, and the identification of potentially actionable genomic alterations in this rare heterogeneous group of tumors may improve treatment and outcome. The genetic landscape of common posterior fossa tumors has been described in the past several years, yet the classification of malignant pediatric supratentorial tumors remains controversial. Next-generation sequencing is a promising tool to evaluate multiple genes concurrently. The clinical utility of next-generation sequencing has not been proven in pediatric brain tumors...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29165672/trans-sylvian-approach-to-microvascular-decompression-for-trigeminal-neuralgia-in-syndromic-cranial-base-settling
#19
Mrityunjoy Sarkar, Ashok Pillai
BACKGROUND AND IMPORTANCE: The lateral suboccipital approach for microvascular decompression (MVD) of the trigeminal nerve has become a standard-of-care over the past several decades. Syndromic cranial base settling, a rare but known cause for trigeminal neuralgia (TN), poses significant dilemmas in clinical management. In such cases, distorted anatomy may render surgery via the suboccipital approach difficult or even impossible. CLINICAL PRESENTATION: A 34-yr-old male with osteogenesis imperfecta and severe basilar invagination suffered from TN that was refractory to medication and stereotactic radiosurgery...
November 18, 2017: Operative Neurosurgery (Hagerstown, Md.)
https://www.readbyqxmd.com/read/29165437/endolymphatic-sac-tumour-in-von-hippel-lindau-disease-management-strategies
#20
E Zanoletti, L Girasoli, D Borsetto, G Opocher, A Mazzoni, A Martini
Endolymphatic sac tumour (ELST) is infrequent, as emerges from small series reported in the literature. It is a slow-growing malignancy with local aggressiveness and a low risk of distant metastases. It is often misdiagnosed because of the late onset of symptoms and difficulty in obtaining a biopsy. Its frequency is higher in von Hippel-Lindau (VHL) disease (a genetic systemic syndrome involving multiple tumours), with a prevalence of around 25%. The diagnosis is based on radiology, with specific patterns on contrast-enhanced MRI and typical petrous bone erosion on bone CT scan...
October 2017: Acta Otorhinolaryngologica Italica
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