AGENESIS OF THE CEREBELLAR VERMIS agenesis OF THE cerebellar vermis. DEFINITION 1. CT/MRI. partial or completeagenesis of the cerebellar vermis; enlargement of the cisterna magna; http://www.icondata.com/health/pedbase/files/AGENESI2.HTM
Extractions: Pediatric Database (PEDBASE) Discipline: CNS Last Updated: 5/21/94 A congenital anomaly of the cerebellum characterized by partial or complete agenesis of the vermis. risk factors: associated anomalies: partial or complete agenesis of the cerebellar vermis enlargement of the cisterna magna other congenital malformations - Dandy-Walker Malformation, Agenesis of the Corpus Callosum
The 18-23-week Scan - Chapter 2.06 The 1823-week scan Gianluigi Pilu Kypros H. Nicolaides The DandyWalker complex refers to a spectrum of abnormalities of the cerebellar vermis, cystic dilatation of the fourth ventricle and enlargement of the cisterna magna. (1) DandyWalker malformation (complete or partial agenesis of the cerebellar vermis and enlarged posterior fossa); http://www.fetalmedicine.com/18-23scanbook/Chapter2/chap02-06.htm
Ae DandyWalker Malformation, agenesis of Corpus Callosum, and Gray Matter Heterotopia The corpus callosum and cerebellar vermis are absent. http://www.med.uc.edu/neurorad/webpage/dda.html
Extractions: Callosal agenesis and heterotopias are discusssed elsewhere (unknowns #39, #85). Dandy Walker complex is thought to be caused by developmental atresia of the fourth ventricular foramina with resultant variable hypoplasia of the cerebellar vermis and a posterior fossa cyst which communicates with the fourth ventricle. The malformation is not inherited, but has numerous associations as follows: The classic Dandy Walker malformation is associated with torcular-lambdoid inversion, but this is not specific. Dandy Walker variants are more common, are characterized by a more benign course with variable hypoplasia of the vermis, and are not associated with T-L inversion BACK TO UNKNOWNS BACK TO CATEGORIES HOME
PedLynx ADENOIDS ADENOVIRUS ENTERITIS ADRENOLEUKODYSTROPHY AEROMONAS ENTERITIS agenesisOF THE CORPUS CALLOSUM agenesis OF THE cerebellar vermis AICARDI SYNDROME http://www.icondata.com/health/pedbase/pedlynx.htm
Extractions: The Pediatric Database (PEDBASE) contains descriptions of over 550 childhood illnesses and has been on the Internet since November 15, 1995. Beginning in November of 1996, new diseases will be added and revisions made to existing files. Information on each disorder in this Database has been obtained from at least 3 sources including Nelson Textbook of Pediatrics (14th and 15th editions), the Birth Defects Encyclopedia (1990 and 1994 editions) and from at least one other source (journal articles, review articles, textbooks). One may download the entire Database as Shareware . An editable Registered version is also available through the author. AARSKOG SYNDROME
Agenesis Of The Vermis agenesis of the vermis This article submitted by Julie Leibel on 9/14/96. Author's Email jleibel@cyberatl.net My 41/2 year old twin son was diagnosed with agenesis of the vermis. Here is a list of responses that have been posted to this article agenesis of cerebellar vermis (9/15/96) 142 PM http://neuro-www.mgh.harvard.edu/neurowebforum/GeneralFeedbackArticles/Agenesiso
Agenesis Of The Cerebellar Vermis HOME agenesis of the cerebellar vermis. INTERACTIONS OF TASK DEMANDS,PERFORMANCE, AND NEUROLOGY; agenesis of the cerebellar vermis; http://www.bdid.com/acvermis.htm
Dandy Walker neural anomalies include "agenesis of the posterior cerebellar vermis, agenesis of the corpus callosum, aqueductal http://www.cvtcollege.org/~lpenrose/dw.html
Extractions: Abstract Dandy Walker Syndrome is known by several different names. It is the result of abnormal development of the cerebellum and fourth ventricle and is often associated with hydrocephalus. It occurs 1 in 25,000 babies and females are affected more often. The disorder was first described in 1954, and was named Dandy Walker Syndrome in 1954 .There are certain characteristics associated with it. Specific symptoms may appear early in infancy or in older children. The prognosis is only moderately favorable. There are specific imaging findings of Dandy Walker Syndrome. Key Words Dandy Walker Syndrome, cerebellum, fourth ventricle, hydrocephalus, ultrasound Introduction Dandy Walker is known by several names such as Dandy Walker Syndrome, Dandy Walker Malformation, Dandy Walker Cyst, and Dandy Walker Variant (1). "The Dandy Walker Syndrome is a spectrum of disorders resulting from abnormal development of the cerebellum with associated maldevelopment of the fourth ventricle (2)." Body It occurs 1 in 25,000 babies and more often in females. It accounts for approximately 1-4% of hydrocephalus cases (3). In 1914, the disorder was first described by Dandy and Blackfan (4). In 1954, Benda designated the disorder as Dandy Walker Syndrome and also reported a familial occurrence (5). The cause is thought to from "abnormal embryogenesis of the roof of the fourth ventricle before the sixth or seventh gestational week (6).
What Is A Dandy-Walker Malformation? a shunt. The extent of agenesis of the cerebellar vermis may varywidely, as can it's effects on the patient. The Cerebellum has http://www.geocities.com/Heartland/Hollow/4868/dandy2.htm
Extractions: Our Understanding of Dandy-Walker Malformations Dandy-Walker is a name given to a range of conditions. Dandy-Walker complex may be a more appropriate term. Different names we have heard include Dandy-Walker Cyst, Dandy-Walker Malformation, and Dandy-Walker Syndrome. It seems that each refers to something a bit different. The distinctions are not clear, however. The three primary indications of Dandy-Walker are cyst formation and associated enlargement of the 4th ventricle , The resulting hydrocephalus, and partial or complete agenesis of the cerebellar vermis . Approximately 90 % of Dandy-Walker patients have hydrocephalus, which is typically treated with a shunt . The extent of agenesis of the cerebellar vermis may vary widely, as can it's effects on the patient. The Cerebellum has several functions: controlling Subconcious Skeletal Muscle Movements, Equilibrium and Posture , and may play a role in the development of the sensations of Anger and Pleasure . The cerebellar vermis plays an important role in providing the link between the two hemispheres of the cerebellum . Agenesis of this structure may affect those functions controlled by the Cerebellum . The severity depends on the magnitude of the agenesis and other factors. Apparently similar levels of agenesis can produce widely different outcomes. Other abnormalities possible within the Dandy-Walker complex include agenesis of the corpus callosum , spinal cord defects, "cranial encephalocele" (protusion of tissue outside the back of the skull, may contain brain tissue), cleft palate, enlarged kidney with cysts, or other brain abnormalities. Typically, the more abnormalities, the poorer the prognosis.
Upper Motor Neuron System Hypotonia Large posterior fossa due to a large cisterna magna and hypoplasia or agenesis of the cerebellar vermis. http://www.pediatricneuro.com/alfonso/pg117.htm
Extractions: Dandy-Walker malformation is most likely due to slow flow of cerebrospinal fluid from the fourth ventricle to the cisterna magna. Dandy-Walker malformation is characterized by agenesis or hypoplasia of the cerebellar vermis and cystic dilation of the posterior fossa (Figure 117.1). Figure 117.1. Schematic representation of Dandy-Walker malformation. Large posterior fossa due to a large cisterna magna and hypoplasia or agenesis of the cerebellar vermis. The tentorium is positioned high because of the large size of the posterior fossa (Figure 117.2). Apneic spells and nystagmus may occur. Macrocephaly with prominent occiput may be present. Other congenital brain abnormalities that occur in association with Dandy-Walker syndrome are agenesis of the corpus callosum, neuronal heterotopia, and aqueductal stenosis. Hydrocephalus is not present at birth but usually develops after 3 months of age. Magnetic resonance imaging of the brain is diagnostic (Figure 117.2).
Rhomb-update.rtf ABSTRACT agenesis of the cerebellar vermis (paleocerebellar agenesis) withfusion of the cerebellar hemispheres (rhombencephalosynapsis) is a rare http://www.indiana.edu/~pietsch/rhomb-update.html
Extractions: web contact: pietsch@indiana.edu 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
Rhomb.html diagnosed in vivo by magnetic resonance imaging (MRI) is presented that demonstratesfusion of the dentate nuclei and agenesis of the cerebellar vermis. http://www.indiana.edu/~pietsch/rhomb.html
Extractions: web contact: pietsch@indiana.edu Rhombencephalosynapsis (RHS) is a rare congenital condition in which the cerebellum is severely underdeveloped. According to Savolaine et al, who first observed RHS with MRI , only fourteen cases have been reported since its discovery in 1914. The rhombencephalon is the embryonic hindbrain rudiment, from which the cerebellum buds off (as the metencephalon) and differentiates. In RHS the central part of the cerbellum (vermis) fails to develop and the two cerebellar hemispheres fuse into a single mass: Thus ' syn apsis', which implies fusion. This search was conducted at Indiana University , Bloomington, Indiana and is presented with the generous co-operation and kind permission of SilverPlatter MEDLINE EXPRESS (R) 1991-1995 1 of 7 TI: [Rhombencephalosynapsis] TO: Rhombenzephalosynapsis. AU: Boltenstern-M; Konrad-A; Jost-W; Uder-M; Kujat-C AD: Institut fur Neuroradiologie, Universitatskliniken des Saarlandes, Homburg/Saar. SO: Rofo-Fortschr-Geb-Rontgenstr-Neuen-Bildgeb-Verfahr. 1995 Jul; 163(1): 91-3
Cerebellar Disorders to be confused with Dandy's syndrome, indicating bilateral vestibular loss), thereis partial or complete agenesis of the cerebellar vermis, cystic formation http://www.tchain.com/otoneurology/disorders/central/cerebellar.htm
Extractions: Return to Education Index Last update: 6/26/01. This page is meant to provide a general outline of cerebellar disorders. More specific and detailed material is found in links. The cerebellum is part of the brain. It lies under the cerebrum, towards the back, behind the brainstem and above the brainstem. The cerebellum is largely involved in "coordination". Persons whose cerebellum doesn't work well are generally clumsy and unsteady. The main clinical features of cerebellar disorders include incoordination, imbalance, and troubles with stabilizing eye movements. There are two distinguishable cerebellar syndromes midline and hemispheric. Midline syndromes are characterized by imbalance. Persons are unsteady, they are unable to stand in Romberg with eyes open or closed, and are unable to well perform tandem gait. Severe midline disturbance causes "trunkal ataxia" a syndrome where a person is unable to sit on their bed without steadying themselves. Some persons have "titubation" or a bobbing motion of the head or trunk. Midline cerebellar disturbances also often affect eye movements. There may be nystagmus, ocular dysmetria and poor pursuit. Hemispheric cerebellar syndromes are characterized by incoordination of the limbs. There may be decomposition of movement, dysmetria, and rebound. Dysdiadochokinesis is the irregular performance of rapid alternating movements. Intention tremors may be present on an attempt to touch an object. A kinetic tremor may be present in motion. The finger-to-nose and heel-to-knee tests are classic tests of hemispheric cerebellar dysfunction. While reflexes may be depressed initially with hemispheric cerebellar syndromes, this cannot be counted on. Speech may be dysarthric, scanning, or have irregular emphasis on syllables.
Untitled Document DandyWalker, central cyst communicating with fourth ventricle agenesis or dysgenesis.malformation, of cerebellar vermis spraying of cerebellar hemisphere. http://www.hamchoon.co.kr/news/news_2002_1.html
Agenesis Of The Corpus Callosum - Page 16 The DandyWalker malformation consists of an enlarged posterior fossa with a highposition of the tentorium, hypo- or agenesis of the cerebellar vermis and a http://www.rbrs.org/database/83-1/page16.html
Extractions: Clinical history A 3-day-old female neonate was referred with macrocephaly and an episode of convulsions. The mother had had no major illness and the pregnancy and labour were uneventful. Apart from a mild hypothermia, the vital signs were normal. The clinical neurological examination showed hypotonia. The EEG was abnormal and showed asymmetric potentials. Radiological diagnosis On sagittal SE T1-weighted MR image of the brain (fig. 1) , there is complete absence of the corpus callosum, vermian hypoplasia, a cystic dilated fourth ventricle and a high position of the tentorium. The remaining upper portion of the vermis is tilted upward. Axial SE T2-weighted MR image of the brain (fig. 3) demonstrates a parallel configuration of the lateral ventricles. The diagnosis was Dandy-Walker malformation with an associated agenesis of the corpus callosum Figure 1 Figure 2 Figure 3 Discussion The Dandy-Walker malformation consists of an enlarged posterior fossa with a high position of the tentorium, hypo- or agenesis of the cerebellar vermis and a cystic dilatation of the 4
Indian Pediatrics - Case Reports to describe this syndrome which included episodic hyperpnea, abnormal eye movements,ataxia and mental retardation with agenesis of cerebellar vermis in 4 http://www.indianpediatrics.net/sept2001/sept-1045-1049.htm
Extractions: 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
Indian Pediatrics - Editorial case does not fit into WWS as there is no absence of cerebellar vermis, there is andBatWing appearance of lateral ventricles suggestive of agenesis of corpus http://www.indianpediatrics.net/may2000/may-542-545.htm
Extractions: Revision Accepted: November 19, 1999 In 1965, Aicardi and colleagues reported a new syndrome characterized by agenesis of corpus callosum (ACC) with cortical heterotopia, infantile spasm, chorioretinopathy, mental retardation with or without associated vertebral anomalies. Amongst these, ACC, infantile spasms, mental retardation and chorioretinal lacunae are the constant findings(1). It is a rare neuro-ophthalmic disorder with progressive mental deterioration. All the patients described till date have been females except two male subjects(2,3). We describe a male baby who had all the essential features suggestive of Aicardis Syndrome along with ventricular septal defect and lissencephaly.
ICP Monitors be minimally or maximally dilated, and there may or may not be dysgenesis of theinferior cerebellar vermis. 2. Complete or incomplete agenesis of the vermis. http://www.ucch.org/sections/neurosurg/NeuroReview/11-Pediatrics/PFossaCysts.htm
Extractions: Congenital CSF Abnomalities of the Posterior Fossa Classification 1. Dandy-Walker malformation. 2. Isolated fourth ventricle. 3. Pulsion diverticulum. 4. Mega cisterna magna, ex-vacuo cysts. I. Dandy-Walker Malformation Definition Epidemiology Dandy-Walker syndrome which consists of the following features. 1. Posterior fossa cyst with the fourth ventricle as its floor. 2. Complete or incomplete agenesis of the vermis. The inferior vermis is usually always involved. 3. Dilatation of fourth ventricle. 4. Enlarged posterior fossa. 5. Torcula elevated and located at the skull vertex. 6. Other associated anomalies. i) Hydrocephalus. ii) Agenesis of corpus callosum. iii) Nuclear dysplasia of brainstem. iv) Cerebral and cerebellar heterotopias. Pathogenesis 1. Foraminal Atresia i) Dandy-Walker. Elastic, diverticulum of IVth ventricle. ii) Chiari. Inelastic, hindbrain herniation. iii) Arachnoid cyst. Splitting of the roof with two layers. iv) Syringomyelia. Pulsation into central canal, water hammer effect. 2. Teratogenic Theory Clinical Features hydrocephalus are present such as an enlarged head, bulging fontanelle, and
Extractions: Program Nr: 973 Prenatally diagnosed isolated Dandy-Walker variant associated with 4p- and 6p- karyotypes. L. Hunnisett , A. Toi , E. Winsor , S. Blaser , D. Chitayat 1) Prenatal Diagnosis Program, The University Health Network; 2) Department of Diagnostic Imaging, The University Health Network; 3) Department of Laboratory Medicine, University Health Network; 4) Department of Radiology, Hospital for Sick Children; 5) University of Toronto, Toronto, Ontario, Canada.
Bombay Hospital Journal - Case Reports - Facial Hemangioma In 2 The malformation consists of a cystic dilatation of the 4th ventricle and morphologicalabnormalities of cerebellar vermis such as agenesis (25%) and http://www.bhj.org/journal/1998/4001_jan/cr_185.htm
