61. ACR Learning File Web The findings of a gray matter lined cleft extending through the entire hemisphereinto the lateral ventricle is diagnostic of schizencephaly. Return to top. http://www.learningfile.com/learning_file/viewcase.php?section=nu&case_num=705

62. Cephalic Disorders - Types - Neurologychannel Colpocepaly, Holoprosencephaly, Hydranencephaly, Iniencephaly, Lissencephaly, Megalencephaly,Microcephaly, Porencephaly, schizencephaly, Other Rare Cephalic http://www.neurologychannel.com/cephalicdisorders/hydranencephaly.shtml

Home Search SiteMap Ask the Dr. ... Medical Store advertisement advertisement CEPHALIC DISORDERS Overview Anencephaly Colpocephaly Holoprosencephaly ... Other Types CONDITIONS ADHD ALS Alzheimer's Disease Autism ... Carpal Tunnel Syndrome Cephalic Disorders Cerebral Palsy Charcot-Marie-Tooth Disease Dementia Encephalitis Epilepsy Essential Tremor ... Guillain-Barre Syndrome Headache Huntington's Disease Hydrocephalus Lou Gehrig's Disease ... Traumatic Brain Injury Vertigo TREATMENT OPTIONS Botulinum Toxin Therapy Epidural Injection Trigger Point Injection Vagus Nerve Stimulation RESOURCES Clinical Trials Links Videos ABOUT US Healthcommunities.com Pressroom Testimonial Hydranencephaly Hydranencephaly is a rare and extreme form of porencephaly. The hemispheres of the cerebrum are absent and replaced by sacs filled with cerebrospinal fluid (CSF). In most cases, the cerebellum and brainstem are formed normally. Causes Vascular injuries, infections, or trauma after the 12th week of pregnancy result in malformations of the brain. Symptoms Some infants with hydranencephaly may have seizures, myoclonus (sudden, rapid, involuntary twitches or jerks), and respiratory problems at birth. Most appear normal and have normal spontaneous reflexes, such as sucking, swallowing, crying, and moving the arms and legs. Within a few weeks these infants become irritable and develop hypertonia, an abnormal increase in muscle tone. Seizures and hydrocephalus usually follow within a few months, and there may be symptoms of visual impairment, lack of growth, deafness, blindness, paralysis, and intellectual deficits.

63. Cleveland Clinic Health System - Health Information Search Results The following information is available for the topic schizencephaly.Please select one. Care Treatment. , schizencephaly. New Search. http://www.cchs.net/health/getcontents.asp?DocID=do-query&TopicId=1353

64. IComm: File Not Found! Scheie Syndrome .. National MPS Society http//members.aol.com/mpssociety.schizencephaly .. http://www.icomm.ca/geneinfo/s.htm

File Not Found We're sorry, but the file you're looking for can't be found. You might want to try starting at our front page or a list of iComm accountholders , or you can search the site This site was last updated on 30 August 2002. iComm Questions regarding the iComm web site should be sent to our webmaster

65. Abstract Diagnostic Neuroradiology. Morphological features and associated anomalies ofschizencephaly in the clinical population detailed analysis of MR images. http://link.springer-ny.com/link/service/journals/00234/contents/01/00719/s00234

Diagnostic Neuroradiology Morphological features and associated anomalies of schizencephaly in the clinical population: detailed analysis of MR images N. Hayashi , Y. Tsutsumi and A. J. Barkovich Department of Radiology, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-0033, Japan Department of Radiology, National Okura Hospital, Tokyo, Japan Department of Radiology, Section of Neuroradiology, University of California San Francisco, San Francisco, USA Abstract. Keywords. Schizencephaly - Porencephaly - Polymicrogyria - MRI E-mail: naoto-tky@umin.ac.jp Phone: 81-3-5800-8666 Fax: 81-3-5800-8935

66. Abstract © SpringerVerlag 2002. Diagnostic Neuroradiology. Polymicrogyriawithout porencephaly/schizencephaly. MRI analysis of the spectrum http://link.springer-ny.com/link/service/journals/00234/contents/02/00793/s00234

Diagnostic Neuroradiology Polymicrogyria without porencephaly/schizencephaly. MRI analysis of the spectrum and the prevalence of macroscopic findings in the clinical population N. Hayashi , Y. Tsutsumi and A. J. Barkovich Department of Radiology, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan Section of Neuroradiology, University of California San Francisco, San Francisco, California, USA Department of Radiology, National Okura Hospital, Tokyo, Japan Abstract. Keywords. Polymicrogyria - MRI - Developmental anomaly E-mail: naoto-tky@umin.ac.jp Phone: +81-3-58008666 Fax: +81-3-58008935

67. Dorlands Medical Dictionary schizencephaly (schiz·en·ceph·a·ly) (skiz²schwansef¢schwa-le)schiz- + Gr. enkephalos brain schizencephalic porencephaly. http://www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspzQzpgzEzzSzppdocszSzuszS

68. CancerGene EMX2 Faiella A;Brunelli S;Granata T;D'Incerti L;Cardini R;Lenti C;Battaglia G;BoncinelliE A number of schizencephaly patients including 2 brothers are heterozygous http://caroll.vjf.cnrs.fr/cancergene/CG1887.html

Infobiogen Search CancerGene CancerGene Homepage Search CancerGene Citations CancerGene Card Symbol Aliases Name empty spiracles homolog 2 (Drosophila) Locus OMIM GDB SwissProt LocusLink Keywords Mutation Diseases Endometrial Neoplasms Note To examine the potential function of EMX2 in endometrial tumorigenesis, Noonan et al. (2001, UI:21433821 ) investigated 20 primary tumors and 6 endometrial cancer cell lines for mutations. Two primary tumors had mutations. Inactivation or reduced expres sion of EMX2 in cancers, coupled with increased expression in the quiescent endometrium, indicate that this homeodomain gene is involved in maintenance of the differentiated state. Selected MEDLINE References: [Link to NCBI] [Link to CancerGene Citation Database] Noonan FC;Goodfellow PJ;Staloch LJ;Mutch DG;Simon TC Antisense transcripts at the EMX2 locus in human and mouse( small star, filled ). (In Process Citation) Genomics 2003 Jan;81(1):58-66. Noonan FC;Mutch DG;Ann Mallon M;Goodfellow PJ Characterization of the homeodomain gene EMX2: sequence conservation, expression analysis, and a search for mutations in endometrial cancers. Genomics 2001 Aug;76(1-3):37-44.

69. Mioti: Medical Condition Condition schizencephaly. NINDS schizencephaly Information Page. Informationfrom the National Institute of Neurological Disorders and Stroke. General Topics. http://www.mioti.com/cat/condition/condition.asp?Cat=Schizencephaly

70. Schizencephaly schizencephaly NINDS schizencephaly http//www.ninds.nih.gov Information sheetcompiled the National Institute of Neurological Disorders and Stroke. http://www.medlina.com/schizencephaly.htm

Academic Medicine Addiction Aging AIDS-HIV ... Women's Health The Web MEDLINA.com (partial) CDC WHO FDA NIH CATEGORIES Search: All Products Books Magazines Popular Music Classical Music Video DVD Baby Electronics Software Outdoor Living Wireless Phones Keywords: Home Up Anencephaly Chiari Malformations ... Lissencephaly Schizencephaly Porencephaly Schizencephaly NINDS: Schizencephaly - http://www.ninds.nih.gov Information sheet compiled the National Institute of Neurological Disorders and Stroke. Pediatric Database - http://www.icondata.com A definition of schizencephaly, the epidemiology, pathogenesis, clinical features, investigations and management. Cleveland Clinic - http://www.clevelandclinic.org A description of schizencephaly including treatments and prognosis and further reading. SUBCATEGORIES Up powered by A merica M edica, I nc. info@medlina.com New York City

71. CLASSIFIEDS.TERADEX.COM - Health/Fitness/Diseases/Genetic Disorders/Schizencepha CLASSIFIEDS.TERADEX.COM Health/Fitness/Diseases/Genetic Disorders/schizencephaly. CLASSIFIEDS/ Health/Fitness / Diseases / Genetic Disorders / schizencephaly. http://classifieds.teradex.com/Health_Fitness/Diseases/Genetic_Disorders/Schizen

preloadImages('as_free_x-mail','','as_ad-space'); Add your Web Site Add Search to your Site Search: Current Group Entire Site Results per page: Output format: Long Short URL Match: All Any Boolean CLASSIFIEDS Health/Fitness Diseases Genetic Disorders ... Schizencephaly Page 1 of 1 Submit Classifieds Ad Edit Ads Ad Headline User ID ... Classified Ads Open adlistings Submit Classifieds Ads! Sign Up User Name: Password: Edit in Real Time, Classifieds Ad Placement E-Mail: webmaster@teradex.com Aaex Corp.

72. Katalog :  : Health : Conditions_and_Diseases : Genetic_Disorders : Schi Translate this page NINDS schizencephaly - Information sheet compiled the National Instituteof Neurological Disorders and Stroke. Pediatric Database http://www.netz-tipp.de/kat/Health/Conditions_and_Diseases/Genetic_Disorders/Sch

Katalog Suchmaschinen-Eintrag - kostenlos! (Anzeige) Web: Suche im ganzen Netz Spezial: Suche im Katalog Startseite Buchhandel Bibliothek Zeitung ... Suchmaschinen Kostenloser Newsletter. Bitte tragen Sie hier Ihre E-Mail-Adresse ein! Startseite Werbung Presse Rechtliches ... Impressum Partner: Voreintrag Ladezeit Link-Check Linkparade HTML-Check Startseite Welt Deutsch Anzeige: Verdienen Sie Geld mit Ihren ungenutzten Domains! Welt English Health ... Genetic Disorders : Schizencephaly Health: Conditions and Diseases: Neurological Disorders: Brain Diseases Health: Conditions and Diseases: Rare Disorders NINDS: Schizencephaly - Information sheet compiled the National Institute of Neurological Disorders and Stroke. Pediatric Database - A definition of schizencephaly, the epidemiology, pathogenesis, clinical features, investigations and management. Seite (URL) vorschlagen Eintrag in weiteren Suchmaschinen Help build the largest human-edited directory on the web. Submit a Site Open Directory Project Become an Editor In einigen Teilen modifizierte Version des DMOZ. Anzeige Sind die Domains xyz.de oder xyz.com wieder frei?

73. The 18-23-week Scan - Chapter 2.09 The 1823-week scan. Gianluigi Pilu Kypros H. Nicolaides. DESTRUCTIVE CEREBRALLESIONS. These lesions include hydranencephaly, porencephaly and schizencephaly. http://www.fetalmedicine.com/18-23scanbook/Chapter2/chap02-09.htm

The 18-23-week scan DESTRUCTIVE CEREBRAL LESIONS These lesions include hydranencephaly, porencephaly and schizencephaly. In hydranencephaly , there is absence of the cerebral hemispheres with preservation of the mid-brain and cerebellum. In porencephaly , there are cystic cavities within the brain that usually communicate with the ventricular system, the subarachnoid space or both. Schizencephaly is associated with clefts in the fetal brain connecting the lateral ventricles with the subarachnoid space. Prevalence Destructive cerebral lesions are found in about 1 per 10,000 births. Etiology Hydranencephaly is a sporadic abnormality that may result from widespread vascular occlusion in the distribution of the internal carotid arteries, prolonged severe hydrocephalus, or an overwhelming infection such as toxoplasmosis or cytomegalovirus. Porencephaly may be caused by infarction of the cerebral arteries or hemorrhage into the brain parenchyma. Schizencephaly may be a primary disorder of brain development or it may be due to bilateral occlusion of the middle cerebral arteries. Diagnosis Complete absence of echoes from the anterior and middle fossae distinguishes hydranencephaly from severe hydrocephalus in which a thin rim of remaining cortex and the mid-line echo can always be identified. In porencephaly, there are one or more cystic areas in the cerebral cortex, which usually communicates with the ventricle; the differential diagnosis is from intracranial cysts (arachnoid, glyo-ependymal), that are usually found either within the scissurae or in the mid-line and compress the brain. In schizencephaly, there are bilateral clefts extending from the lateral ventricles to the subarachnoid space. Schizencephaly is usually associated with absence of the cavum septum pellucidum.

74. Lissencephaly Microlissencephaly (MLIS), NormanRoberts Syndrome (NRS), Lissencephaly with CerebellarHypoplasia (LCH), Polymicrogyria (PMG), schizencephaly (SCH), Muscle-Eye http://www.my-journey.net/pages/lissencephaly.html

Lissencephaly Isolated Lissencephaly Sequence (ILS), X-Linked Lissencepahly (XLIS), Subcortical Band Heterotopia (SBH), Miller-Dieker Syndrome (MDS), Microcephaly, Microlissencephaly (MLIS), Norman-Roberts Syndrome (NRS), Lissencephaly with Cerebellar Hypoplasia (LCH), Polymicrogyria (PMG), Schizencephaly (SCH), Muscle-Eye-Brain (MEB) Disease, and Walker-Warburg Syndrome (WWS), Chromosome 17p13.3 deletion Lissencephaly Network, Inc. 716 Autumn Ridge Fort Wayne, IN 46804-6402 Phone: (219) 432-4310 Contact: Dianna Fitzgerald Fax: (219) 749-6337 Web site: http://www.lissencephaly.org E-mail: lissnet@lissencephaly.org or pjoliver@ghg.net see Genetics Page (recurrence rates for different types) and Lissencephaly Types and Genetics Lissencephaly Network c/o Debbie Bloor 1549 Regent Street Regina, Saskatchewan S4N 1S1 CANADA Phone: 306-569-0146 Lissencephaly Launch Pad , United Kingdom (parents web site) 39 Barlows Road Edgbaston Birmingham B15 2PN England Tel: +44 (0)121 455 0981 Web Page: http://homepage.ntlworld.com/foliot/liss/

75. Health Library - Schizencephaly SelfHelp Resources Information about national and local self-help organizationsand support groups. schizencephaly. Self Help Clearinghouse. Schiz Kidz Buddies. http://www.baylorhealth.com/library/healthguide/selfhelp/topic.asp?hwid=shc29sch

76. Health Library - Schizencephaly Logo. Customized health information for you and your family Site Search AdvancedSearch. schizencephaly. Self Help Clearinghouse. Schiz Kidz Buddies. http://www.stjosephhealth.org/library/healthguide/SelfHelp/topic.asp?hwid=shc29s

77. Health Conditions And Diseases Genetic Disorders Schizencephaly Polish Yellow Pa Health Conditions and Diseases Genetic Disorders schizencephaly552312 Polish Yellow Pages YP.pl. http://www.yellowpages.pl/ca/552312/Health/Conditions_and_Diseases/Genetic_Disor

>English Version Polskie Firmy: Firmy wg SIC Szukaj firmy Szukaj w Internecie: Polska ¦wiat Katalog Stron Biznes Serwisy YellowPages: Rejestracja DOMEN Strony WWW DNS ZOOM ... Sklep Partner idealo.de NETSPRINT Reporter.pl Portofolio DVD Search TOP CHARTS Webkatalog Multi szukanie - mainSeek Polska - Netsprint ¦wiatowy Katalog Stron Katalog Firm Teleadreson Szukaj domeny TOP Health Schizencephaly Cleveland Clinic - A description of schizencephaly including treatments and prognosis and further reading. NINDS: Schizencephaly - Information sheet compiled the National Institute of Neurological Disorders and Stroke. Pediatric Database - A definition of schizencephaly, the epidemiology, pathogenesis, clinical features, investigations and management. Help build the largest human-edited directory on the web. Submit a Site Open Directory Project Become an Editor P³atna reklama REJESTRACJA DOMEN KATALOG STRON PROFESJONALNY DNS SKLEP ... e-mail

78. NEURORADIOLOGY axial space with temporal lobe atrophy. Diagnosis schizencephaly (opentype). Discussion Clinical Findings Patients may present with http://www.indianradiologist.com/neuroradiology9.htm

NEURORADIOLOGY Case 9 : A 13-year-old male presents with seizure disorder. CT scan was performed. What is the diagnosis? SCROLL FOR ANSWER Imaging Findings Study shows a cleft in the temporal lobe, communicating with a prominent extra-axial space with temporal lobe atrophy. Diagnosis Schizencephaly (open type) Discussion Clinical Findings Patients may present with seizure disorders, mild to moderate developmental delay and mental retardation. Etiology/Pathophysiology Failure of the normal migration of neurons from the germinal matrix zone at 1-5 months of gestation. A gray matter lined parenchymal cleft develops which extends from the subarachnoid space to the subependyma of the lateral ventricles. Schizencephaly occurs when there is a failure of the normal migration of neurons from the germinal matrix zone at 1-5 months of gestation. Some theories suggest that possible causes of this are in-utero stroke, early gestational viral infections, and recent Italian research has linked Schizencephaly with a mutated gene called the Homeobox gene EMX-2. If the gene EMX-2 is missing or defective, nerve cell growth and migration will not occur normally and this will lead to the formation of the 'clefts' associated with Schizencephaly.

79. BRI - Publications - ABSTRACTS Twins with different temporal lobe malformations schizencephaly and arachnoidcyst. schizencephaly is generally regarded as unrelated to arachnoid cysts. http://www.brain.org.au/publications/GDJ_abs.html

Epilepsia 1999 Dec;40(12):1816-20 Occurrence of hippocampal sclerosis: is one hemisphere or gender more vulnerable? Briellmann RS Jackson GD , Mitchell LA, Fitt GJ, Kim SE, Berkovic SF Brain Imaging Research Institute, Department of Neurology, Austin and Repatriation Medical Center, Heidelberg, Victoria, Australia. PMID: 10612350, UI: 20077597 Neurology 1999 Oct 22;53(7):1462-7 Motor cortex localization using functional MRI and transcranial magnetic stimulation. Macdonell RA, Jackson GD , Curatolo JM, Abbott DF , Berkovic SF, Carey LM, Syngeniotis A , Fabinyi GC, Scheffer IE PMID: 10534252, UI: 20001819 Neurology 1999 Sep 11;53(4):715-22 Clinical and imaging features of cortical malformations in childhood. Leventer RJ, Phelan EM, Coleman LT, Kean MJ, Jackson GD Harvey AS Department of Neurology, Royal Children's Hospital, Melbourne, Australia. PMID: 10489031, UI: 99417156 Radiology 1999 Feb;210(2):529-38 Presurgical evaluation of the motor hand area with functional MR imaging in patients with tumors and dysplastic lesions. Achten E

80. Discus26 CASE 40 DISCUSSION schizencephaly is an uncommon structural disorder of cerebralcortical development, characterized by congenital clefts spanning the cerebral http://www.medicine.cmu.ac.th/dept/radiology/pedrad/discus40.html

CASE 40 DISCUSSION Schizencephaly is an uncommon structural disorder of cerebral cortical development, characterized by congenital clefts spanning the cerebral hemispheres from the pial surface to the lateral ventricles and lined by cortical gray matter [1]. This lesion, which occurs between the third and fourth month of gestation, may be due to an antenatal environmental incident or may be of a genetic origin. The clinical features of schizencephaly are extremely variable. The severity of symptoms is related to the amount of the brain involved. Children with unilateral schizencephaly present with hemiparesis and mild mental delay, while children with bilateral cleft are tetraparetic with severe mental deficits [1]. The clefts are divided into those with closed lips and those with open lips. In the clefts with closed lips, the walls oppose one another directly, obliterating the CSF space within the cleft at that point. When the lips are open, CSF fills the cleft all the way from the lateral ventricle to the subarachnoid spaces surrounding the hemispheres. The clefts, which can be unilateral or bilateral, are most commonly located near the pre- and post-central gyri. The septum pellucidum is absent in about 80% of patients with schizencephaly [2]. These abnormalities can be seen with any imaging modality, but appear best demonstrated with magnetic resonance imaging. Imaging studies of schizencephaly show a cleft through the affected hemisphere. Gray matter, typically characterized by a bumpy outer surface and an irregular gray matter-white matter junction, lines the cleft. The gyral pattern of the cortex adjacent to the cleft is usually abnormal, with sulci that radiate into the cleft. The gray matter lining the cleft may extend into the ventricle in the form of subependymal heterotopia [2]. On proton density and T2-weighted images, the lining of the cleft is isointense to gray matter. On MRI differentiation of schizencephaly from a porencephalic cyst is based on demonstration of gray matter along the entire length of the cleft in schizencephaly, whereas with porencephaly the cleft extends through both gray and white matter so that there will also be white matter abutting the CSF cyst [3].

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