41. TheFetus.net - Joubert Syndrome -Philippe Jeanty, MD, PhD, Nashville, TN And Gia aplasia or agenesis of the cerebellar vermis. fragmentation several brainstemnuclei (dentate nuclei, inferior olives, and basis pontis) http://www.thefetus.net/page.php?id=122

42. Rhomb-update.rtf ABSTRACT agenesis of the cerebellar vermis (paleocerebellar agenesis) with fusionof the cerebellar hemispheres (rhombencephalosynapsis) is a rare http://www.indiana.edu/~pietsch/rhomb-update.html

web contact: pietsch@indiana.edu Rhombencephalosynapsis -1996-1999 (September) to earlier set and introduction This search was conducted at Indiana University , Bloomington, Indiana and is presented with the generous co-operation and kind permission of SilverPlatter Record 1 of 5 in MEDLINE EXPRESS (R) 1996-1998 TITLE: Rhombencephalosynapsis: cerebellar embryogenesis. AUTHOR(S): Utsunomiya-H; Takano-K; Ogasawara-T; Hashimoto-T; Fukushima-T; Okazaki-M ADDRESS OF AUTHOR: Department of Diagnostic Radiology, Fukuoka University Hospital, Japan. SOURCE (BIBLIOGRAPHIC CITATION): AJNR-Am-J-Neuroradiol. 1998 Mar; 19(3): 547-9 INTERNATIONAL STANDARD SERIAL NUMBER: 0195-6108 PUBLICATION YEAR: 1998 LANGUAGE OF ARTICLE: ENGLISH COUNTRY OF PUBLICATION: UNITED-STATES ABSTRACT: We describe two infants in whom rhombencephalosynapsis was diagnosed with MR imaging in vivo. In contrast to Dandy-Walker malformation, the vermian maldevelopment in this anomaly is characterized by an absence of the anterior vermis and a deficiency of the posterior vermis. The cerebellar hemispheres are fused. In an attempt to identify the pathogenesis of these anatomic manifestations, we question the traditional concept of the embryologic development of the cerebellar primordium. MINOR MESH HEADINGS: Brain-pathology; Cerebellum-pathology; Child,-Preschool; Fetal-Development-physiology; Magnetic-Resonance-Imaging

43. EMedicine - Dandy-Walker Malformation : Article By Lutfi Incesu, MD The condition is characterized by agenesis or hypoplasia of the cerebellar vermis, Note abnormal cerebellar vermis in association with a posterior fossa http://www.emedicine.com/radio/topic206.htm

(advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Patient Education Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Radiology Pediatrics Dandy-Walker Malformation Last Updated: October 31, 2003 Rate this Article Email to a Colleague Synonyms and related keywords: Dandy-Walker syndrome, Dandy-Walker cyst, Dandy-Walker deformity, Luschka-Magendie foramina atresia AUTHOR INFORMATION Section 1 of 11 Author Information Introduction Differentials X-ray ... Bibliography Author: Lutfi Incesu, MD , Chief of Neuroradiology and MR Section, Professor, Department of Radiology, Ondokuz Mayis University Hospital, Samsun, Turkey Coauthor(s): Anil Khosla, MD , Assistant Professor, Department of Radiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine Lutfi Incesu, MD, is a member of the following medical societies: American Society of Neuroradiology , and Radiological Society of North America Editor(s): Charles M Glasier, MD

44. EMedicine - Dandy-Walker Malformation : Article Excerpt By: Lutfi Incesu, MD The condition is characterized by agenesis or hypoplasia of the cerebellar vermis,cystic dilatation of the fourth ventricle, and enlargement of the http://www.emedicine.com/radio/byname/dandy-walker-malformation.htm

(advertisement) Excerpt from Dandy-Walker Malformation Synonyms, Key Words, and Related Terms: Dandy-Walker syndrome, Dandy-Walker cyst, Dandy-Walker deformity, Luschka-Magendie foramina atresia Please click here to view the full topic text: Dandy-Walker Malformation Background: Dandy-Walker malformation is a rare congenital malformation and involves the cerebellum and fourth ventricle. The condition is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlargement of the posterior fossa. A large number of concomitant problems may be present, but the syndrome exists whenever these 3 features are found. Approximately 70-90% of patients have hydrocephalus, which often develops postnatally. Dandy-Walker malformation may be associated with atresia of the foramen of Magendie and, possibly, the foramen of Luschka. Dandy-Walker malformation first was described by Dandy and Blackfan in 1914. Since the original description, additional studies have reported on the various morphologic features of the syndrome. Not until 1954 did Benda first emphasize that atresia of the cerebellar outlet foramina is not an essential feature of the condition and suggested the now widely accepted term Dandy-Walker malformation. Studies by D’Agostino in 1963 and Hart et al in 1972 further defined the characteristic triad of Dandy-Walker malformation as consisting of (1) complete or partial agenesis of the vermis, (2) cystic dilatation of the fourth ventricle, and (3) an enlarged posterior fossa with upward displacement of lateral sinuses, tentorium, and torcular herophili. The triad typically is found in association with supratentorial hydrocephalus, which should be considered a complication rather than part of the malformation complex.

45. OBGYN.net - OBGYN.net Ultrasound Section - Ultrasound Image Gallery agenesis of the cerebellar vermis by Miguel Octavio Sosa Palaviccini, TRV FetalHead Choroid Plexus Cyst by Mario Ernesto Libardi. . Fetal Profile http://www.obgyn.net/us/us.asp?page=gallery/gallery

46. Glossary In Congenital Malformations NeuroLearn NeuroHelp Type II Displacement of the cerebellar vermis combined with deformities of the Caudal Regression Syndrome (sacral agenesis) The caudal regression http://moon.ouhsc.edu/kfung/JTY1/NeuroHelp/ZNG0BS01-C.htm

Glossary in Congenital Malformations NeuroLearn NeuroHelp Malformations General ... Z General #Camptodactyly: Means permenant flexion of one or more fingers. (Campto- means flex). #Cavum septi pellucidi and cavum Vergae: As the corpus callosum grows, the banks of the median groove ventral to the callosum separate to form a small pocket that develops into the cavum septum pellucidi (also kown as 5 th ventricle), a space that persists until term and that gradually narrows to a potential space in the adult. Persistence of the cavum septi pellucidi, and its posterior extension the cavum vergae are not malformations. The cavum septi pellucidi and cavum vergae should not be communicating with the ventricles. #Cephalocele (encephalocele): a herniation of cranial contents through a skull defect. #Cerebellar dysplasia: I prefer to limit this term to abnormal formation of the cerebellar gyri and/or dentate nuclei in order to distinguish this entity from cerebellar heterotopia. The hemispheres are preferentially affected. #Cerebellar Dysplasia: [according to Rorke et al., 1968]

47. Glossary In Congenital Malformations NeuroLearn NeuroHelp agenesis of cerebellar vermis this term essentially means the absence of acerebellar vermis or that the vermis is not separated from the cerebellar http://moon.ouhsc.edu/kfung/JTY1/NeuroHelp/ZNG0BS01-A.htm

Glossary in Congenital Malformations NeuroLearn NeuroHelp Malformations General ... Z General #Agenesis: the complete absence of an organ and its associated primordium. #Agenesis of corpus callosum: Clinical features: Agenesis of corpus callosum is often associated with marked ventricular dilatation and a large head. Agenesis of the corpus callosum should be reserved to the cases when mechanism(s) by which the axons of the corpus callosum cross the midline in the usual position are absent of deficient (i.e., the agenesis is a primary event but not secondary to other damage or malformation of the neurons or axons up stream to the callosal axons). Probst bundle can be found. Lipomas and hamartomas: The association of hamartomas and lipomas in some cases support the hypothesis that agenesis of corpus callosum in these cases may be resulted from disruption of outgrowth of corpus callosum by mechanical obstruction. Since the corpus callosum develops in an anterior to posterior fashion, partial agenesis usually involves the posterior part while complete agenesis involves both anterior and posterior portions. If the anterior part of the corpus callosum is absent but the posterior part is present, a destructive mechanism should be seriously considerd. Complete or partial agenesis of the corpus callosum rarely exists as an isolated entity. They are associated with many different malformations of the brain with Aicardi syndrome as the prototype. Agenesis of corpus callosum also has increased association with a midline mass such as lipoma, meningioma, cysts, and hamartoma. They also have increased association with visceral abnormalities.

48. Indian Pediatrics - Case Reports Neuropathological studies reveal agenesis of cerebellar vermis, hypoplasia orfragmentation of several brainstem nuclei and dysplasia of structures at the http://www.indianpediatrics.net/sept2001/sept-1045-1049.htm

Home Past Issue About IP About IAP ... Subscription Case Reports Indian Pediatrics 2001; 38: 1045-1049 Joubert Syndrome Rekha Solomon, Atanu Kumar Jana, Surendra Singh*, Agnihotri Biswas From the Departments of Neonatology and Radio-diagnosis*, Christian Medical College Hospital, Vellore 632 004, Tamil Nadu, India. Correspondence to: Dr. A.K. Jana, Professor and Head, Neonatology Department, Christian Medi-cal College Hospital, Vellore 632 004, Tamil Nadu, India. E-mail: neonat@cmcvellore.ac.in Manuscript received: December 5, 2000;Initial review completed: December 29, 2000;Revision accepted: February 27, 2001. Joubert syndrome is a rare autosomal recessive disorder characterized by abnormal respiratory pattern and eye movements, hypotonia, ataxia, developmental retardation with neuropathologic abnormalities of cerebellum and brainstem. We report a case of Joubert syndrome presenting in the neonatal period. Although previously described in Indian literature(1), this is probably the first case report of Joubert syndrome in a neonate from our country. Case Report A 12-hour-old term baby boy born to a second gravida mother was admitted with history of respiratory distress since 4 hours of age. The infant was the product of a second degree consanguineous marriage. The first pregnancy of the mother ended in an

49. Journal Of Computer Assisted Tomography - UserLogin Magnetic resonance imaging showed agenesis of the cerebellar vermis, dorsal fusionof the cerebellar hemispheres, and clear reduction of the cerebellar http://www.jcat.org/pt/re/jcat/fulltext.00004728-200411000-00006.htm

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50. Medical Image Database, Radiology Teaching Files And Cases, MedPix Complete or partial absence of the cerebellar vermis and cystic dilatation ofthe fourth ventricle agenesis of the corpus callos ( continues ) http://rad.usuhs.mil/medpix/medpix.html?mode=tsearch2&srchstr=Dandy Walker Varia

51. Level II Ultrasound:The Fetal Head And Neck agenesis of the cerebellar vermis, abnormal eye movenment, ataxia, mentalretardation, early death. Autosomal recessive. Information and Sonograms SOURCE http://www.perinatology.com/ultrasound/cns.htm

perinatology.com Level II Ultrasound: The Fetal Head and Neck Return to Homepage Translate Site Map Agencies and Organizations Calculators Critical Care Exposures Chemicals Drugs Infection Physical Agent ... About us Ultrasound Menu Agenesis of Corpus Anencephaly Aneurysm of the Vein of Galen Arachnoid Cyst Arnold Chiari Malformation Choroid Plexus Cyst Cleft Palate Craniosynostosis Cystic Hygroma Encephalocele Dandy-Walker Malformation Hydrocephaly Holoprosencephaly Hydranencephaly Intracranial Mass Isolated Mild Ventriculomegaly "Lemon" Sign Microcephaly Nuchal Translucency Porencephaly Ventriculomegaly (If the menus below are not expanded, then reload the page.) Sonographic finding(s) Differential Diagnosis General: Face and neck Central Nervous System Source: TheFetus.net Sonographic Evaluation of the Fetal Head Source: GE Medical Systems US Assessment of the Fetal Head and Neck: A State-of-the-Art Pictorial Review Source: Radiological Society of North America Measurement of the Biparietal Diameter(BPD) and Head Circumference(HC) The BPD is measured at the level of the thalami and cavum septi pellucidi from the outer edge of the skull table to the inner edge of the opposite skull table.

52. CHDD Center - Joubert Syndrome REA of hindbrain disorders consisting of partial agenesis of the cerebellar vermis,cystic dilatation of the 4th ventricle of the brain, and hydrocephalus. http://depts.washington.edu/chdd/MRDDRC/REAs/JoubertREA.html

Site Map MRDDRC UCEDD Organization ... Admin Research Emphasis Area on Joubert Syndrome Coordinator: Phillip F. Chance, MD In addition, there are several related conditions with retinal, renal, and/or hepatic manifestations that share the molar tooth sign on MRI, thereby increasing the prevalence of this distinctive hindbrain malformation. Other developmental disorders of the cerebellum of interest to this Research Emphasis Area (REA) that are distinct from the molar tooth group of disorders include the Dandy-Walker malformation and Dandy-Walker variants, a relatively prevalent group of hindbrain disorders consisting of partial agenesis of the cerebellar vermis, cystic dilatation of the 4th ventricle of the brain, and hydrocephalus. Pontocerebellar hypoplasia and cerebellar hypoplasia are poorly understood disorders characterized by congenital malformations and/or early-onset atrophy of the pons and/or cerebellum, a heterogeneous group of conditions which may be associated with other birth defects in some cases. In contrast to the dogma that the cerebellum has a limited role in cognitive development, learning delays and/or mental retardation are almost universal in individuals with Joubert syndrome and many of these cerebellar malformation conditions. The Joubert syndrome REA represents an interdisciplinary collaboration by investigators with the shared purpose to understand cerebellar malformation conditions and their impact on learning.

53. Rt-image.com: Your Weekly Source For RT Imaging, Radiology Jobs, Editorials And agenesis of the callosum is the congenital absence of the brain s corpus a partial or complete absence of the cerebellar vermis (the area between the http://www.rt-image.com/content=9504J05C4896A08040B69C74446860441

FAQ Advertising Info Career Center Continuing Education ... Subscribe the weekly source for radiology professionals vol. 18, no. 7 - February 14, 2005 Current Issue Events Classifieds Past Issues COVER STORY On the Road to Clinical Advancement FEATURES Agenesis of the Corpus Callosum Education for RTs DEPARTMENTS First Read News Extras Technology Spotlight Mail Slot ... In the News RADIOLOGY DIRECTORY 2005 Radiology Directory E-MAIL NEWSLETTER Enter your e-mail below to receive our free weekly newsletter. READER POLL How safe do you feel your facility is from terrorism? completely safe somewhat safe somewhat susceptible very susceptible don’t know ...and see the results! Survey Results LOOKING FOR… Agenesis of the Corpus Callosum Unraveling the mystery By Susan L. Koubek Article available online at: http://www.rt-image.com/0214agenesis This article, submitted by Susan L. Koubek, won first place in the Student category of the 2004 RT Image Writing Competition. The corpus callosum is the largest connection pathway in the human brain. It is made of more than 200 million nerve fibers that connect the left and right hemispheres of the brain. The corpus callosum allows the sides of the brain to "communicate" with each other and coordinate their efforts. Each hemisphere of the brain is specialized to control movement and feeling of the opposite half of the body, and each hemisphere specializes in processing certain types of information, such as language or spatial patterns. In order for the corpus to coordinate movement or think about complex information, the hemispheres must communicate with each other, and the corpus callosum is the main connector that allows that communication.

54. Blackwell Synergy - Cookie Absent Familial agenesis of the cerebellar vermis. A syndrome of episodic hyperpnea,abnormal eye movements, ataxia, and retardation. Neurology 1969; 19 813 825. http://www.blackwell-synergy.com/doi/abs/10.1111/j.1460-9592.2004.01286.x

Home An Error Occurred Setting Your User Cookie A cookie is a small amount of information that a web site copies onto your hard drive. Synergy uses cookies to improve performance by remembering that you are logged in when you go from page to page. If the cookie cannot be set correctly, then Synergy cannot determine whether you are logged in and a new session will be created for each page you visit. This slows the system down. Therefore, you must accept the Synergy cookie to use the system. What Gets Stored in a Cookie? Synergy only stores a session ID in the cookie, no other information is captured. In general, only the information that you provide, or the choices you make while visiting a web site, can be stored in a cookie. For example, the site cannot determine your email name unless you choose to type it. Allowing a web site to create a cookie does not give that or any other site access to the rest of your computer, and only the site that created the cookie can read it. Please read our for more information about data collected on this site.

55. Blackwell Synergy - Cookie Absent agenesis of the cerebellar vermis is an uncommon defect, and usually is detectedwhile evaluating the posterior fossa. It can be part of the DWM or an http://www.blackwell-synergy.com/doi/abs/10.1046/j.0960-7692.2001.00621.x

Home An Error Occurred Setting Your User Cookie A cookie is a small amount of information that a web site copies onto your hard drive. Synergy uses cookies to improve performance by remembering that you are logged in when you go from page to page. If the cookie cannot be set correctly, then Synergy cannot determine whether you are logged in and a new session will be created for each page you visit. This slows the system down. Therefore, you must accept the Synergy cookie to use the system. What Gets Stored in a Cookie? Synergy only stores a session ID in the cookie, no other information is captured. In general, only the information that you provide, or the choices you make while visiting a web site, can be stored in a cookie. For example, the site cannot determine your email name unless you choose to type it. Allowing a web site to create a cookie does not give that or any other site access to the rest of your computer, and only the site that created the cookie can read it. Please read our for more information about data collected on this site.

56. The American Journal Of Forensic Medicine And Pathology - UserLogin We describe a suicide with unexpected cerebellar agenesis in which there were however, the right cerebellar hemisphere and vermis were absent (Figs. http://www.amjforensicmedicine.com/pt/re/ajfmp/fulltext.00000433-200003000-00016

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57. Development Of Hydrocephalus In A Patient With Joubert Syndrome Genel Ferah, Atl imaging showed dilation of the posterior fossa communicating with the fourthventricle, ventricular dilation and agenesis of the cerebellar vermis. http://www.jpgmonline.com/article.asp?issn=0022-3859;year=2004;volume=50;issue=2

58. Current Opinion In Pediatrics - UserLogin Joubert syndrome is characterized by absence of the cerebellar vermis, in Joubert syndrome a genetic disorder with agenesis of the cerebellar vermis. http://www.co-pediatrics.com/pt/re/copeds/fulltext.00008480-200012000-00002.htm

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59. Portal Toolkit Invalid Site URL by the association of complete or partial agenesis of the cerebellar vermis The cerebellar vermis was absent, the cerebellar hemispheres separated, http://ppv.ovid.com/pt/re/ajrp/fulltext.00001833-199902000-00002.htm

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60. EDUCATION PLANET - 6 Web Sites For Cerebellum agenesis OF THE cerebellar vermis * Pediatric Database (PEDBASE) DisciplineCNS Last Updated 5/21/94 agenesis OF THE cerebellar vermis DEFINITION A http://www.educationplanet.com/search/Science/Biology/Neuroscience/Brain_Anatomy

All Grades Pre-K K-2 Higher Ed Search 100,000+ top educational sites, lessons and more! Home Science Biology Neuroscience ... Cerebellum Found 6 ' Cerebellum ' Web Sites. Also for ' Cerebellum 10 Lesson Plans 24 Videos Web Sites (1 - 6 of 6): StandardDeviants.com - Offering fun, free educational resources for 36 high school, college and continuing education subjects, StandardDeviants.com is the ultimate learning resource on the Web. Grades: Cache Report Link Problem Add Comment ACUTE CEREBELLAR ATAXIA - Pediatric Database (PEDBASE) Discipline: CNS Last Updated: 5/21/94 ACUTE CEREBELLAR ATAXIA DEFINITION: A movement disorder characterized by the sudden onset of acute truncal ataxia 2-3 weeks after a viral infection. EPIDEMIOLOGY: incidence: ? age of onse... Cache Report Link Problem Add Comment CNS Pathology Index ... Sumeet Gupta's Neural Network Page - A huge list of neural network , fuzzy logic , artificial life, genetic algorithms and classical AI sites by Sumeet Gupta Cache Report Link Problem Add Comment Prion Diseases - This document describes infectious agents which (almost certainly) do not have a nucleic acid genome. It seems that a protein alone is the infectious agent. The infectious agent has been called a prion. A prion has been defined as "small proteinaceou... Grades: Cache Report Link Problem Add Comment Brain Facts and Figures - Brain Facts and Figures These data were obtained from several textbooks. Note that all data are estimates and averages. Check literature for appropriate

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