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         Schizencephaly:     more detail
  1. The Official Parent's Sourcebook on Schizencephaly: A Revised and Updated Directory for the Internet Age by ICON Health Publications, 2004-02-13
  2. Schizencephaly: An entry from Thomson Gale's <i>Gale Encyclopedia of Neurological Disorders</i> by Peter, MD Lin, 2005
  3. The Official Parent's Sourcebook On Schizencephaly by Icon Health Publications, 2004

21. Schizencephaly
schizencephaly is a rare developmental disorder characterized by abnormal slits, or clefts, in the cerebral hemispheres. schizencephaly
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*~Schizencephaly~* Directory: Cephalic Disorders Home Anecephaly Cebocephaly ... Research SCHIZENCEPHALY is a rare developmental disorder characterized by abnormal slits, or clefts, in the cerebral hemispheres. Schizencephaly is a form of porencephaly. Individuals with clefts in both hemispheres, or bilateral clefts, are often developmentally delayed and have delayed speech and language skills and corticospinal dysfunction. Individuals with smaller, unilateral clefts (clefts in one hemisphere) may be weak on one side of the body and may have average or near-average intelligence. Patients with schizencephaly may also have varying degrees of microcephaly, mental retardation, hemiparesis (weakness or paralysis affecting one side of the body), or quadriparesis (weakness or paralysis affecting all four extremities), and may have reduced muscle tone (hypotonia). Most patients have seizures and some may have hydrocephalus. In schizencephaly, the neurons border the edge of the cleft implying a very early disruption in development. There is now a genetic origin for one type of schizencephaly. Causes of this type may include environmental exposures during pregnancy such as medication taken by the mother, exposure to toxins, or a vascular insult. Often there are associated heterotopias (isolated islands of neurons) which indicate a failure of migration of the neurons to their final position in the brain.

22. Schizencephaly - Information / Diagnosis / Treatment / Prevention
home genetic disorders schizencephaly schizencephaly. Information • Diagnosis • Treatment • Prevention. External links (marked
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Health News: Search millions of published articles for news on Schizencephaly Modern Medicine Aging The Ardell Wellness Report HealthFacts Medical Post Medical Update Men's Health and the National Women's Health Report Note: Subscription required to access the full text of articles. Web Directory: 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. Schizencephaly.com

23. Open Lipped Schizencephaly
Open Lipped schizencephaly. Differential Diagnosis schizencephaly versus porencephaly. Lining by gray matter distinguishes between the two.
http://www.med.uc.edu/neurorad/webpage/eqa.html
Open Lipped Schizencephaly Findings:
A large CSF intensity cleft extends from the left lateral ventricle to the frontal cortex, lined by gray matter. Sulcation is abnormal on the right, with thickened cortex and pachygyria. Differential Diagnosis:
schizencephaly versus porencephaly. Lining by gray matter distinguishes between the two. Discussion:
-schizencephaly is the most common migrational anomaly occurring at approximately 6 weeks gestation.
-other migrational anomalies: lissencephaly, polymicrogyria, pachygyria
-infarct vs. developmental, TORCH
-50% have septo-optic dysplasia
-80% rule- bilateral, open, symmetric BACK TO UNKNOWNS BACK TO CATEGORIES HOME NEXT CASE

24. Disease Directory : Genetic Disorders : Schizencephaly
Arquivos de NeuroPsiquiatria - B schizencephaly /B B report - schizencephaly report of eleven cases. ABSTRACT schizencephaly. What is schizencephaly?
http://www.diseasedirectory.net/Genetic_Disorders/Schizencephaly/default.aspx
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... Genetic Disorders : Schizencephaly

25. Disease Directory : Schizencephaly.com
Diseases Genetic Disorders Smith Lemli Opitz Syndrome schizencephaly.com. Date Added 2/4/2004 12450 PM URL http//www.schizencephaly.com.
http://www.diseasedirectory.net/detailed/10404.aspx
Wednesday, June 02, 2004 Genetic Disorders
Aarskog Syndrome

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... Smith Lemli Opitz Syndrome : Schizencephaly.com
Directory Listing
Title: Schizencephaly.com
Description: Dedicated to the family, friends, and caregivers of individuals with Schizencephaly. FAQs, forum, and resources.
Date Added: 2/4/2004 1:24:50 PM
URL: http://www.schizencephaly.com

26. Uhrad.com - Neuroradiology Imaging Teaching Files
uhrad.com Neuroradiology Imaging Teaching Files. Case Thirteen - Bilateral Closed-lip schizencephaly. Diagnosis Bilateral closed-lip schizencephaly.
http://www.uhrad.com/mriarc/mri013.htm
uhrad.com - Neuroradiology Imaging Teaching Files
Case Thirteen - Bilateral Closed-lip Schizencephaly
Click on Images for Enlarged View Clinical History: Four-year-old female with new onset of febrile seizures associated with loss of vision. Radiologic Findings: On the CT of the brain nodular irregularity of both lateral ventricular walls suggestive of heterotopia. T1 weighted axial images and gradient echo volume sagittal images were obtained. There is a gray matter lined cleft extending from the level of the parieto-occipital junction to the occipital horns of the lateral ventricles. There is marked gray matter heterotopia lining the occipital horns, atria, and the bodies of the lateral ventricles bilaterally. The gyral pattern of the occipital and pareietal lobes are abnormal with a thickened nodular pattern suggesting areas of polymicrogyria. Diagnosis: Bilateral closed-lip schizencephaly Discussion: Schizencephaly, also known as agenetic porencephaly, refers to gray matter lined clefts that extend through the entire hemisphere from the ependymal lining of the lateral ventricles to the pial covering of the cortex. The gray matter lining is dysplastic. The clefts can be unilateral or bilateral and are commonly located near the pre- or post-central gyri. Two types of schizencephaly are identified for prognostic purposes. In closed-lip schizencephaly, the cleft walls oppose each other causing obliteration of the CSF space within the cleft. In open-lip schizencephaly, CSF fills the cleft from the lateral ventricles to the subarachnoid space that surrounds the hemispheres. Patients typically present with seizures, hemiparesis, and some degree of developmental delay.

27. Schizencephaly
schizencephaly,. Print this article, Also, see schizencephaly. NW. The Encyclopaedia of Medical Imaging Volume VII. schizencephaly, Fig.
http://www.amershamhealth.com/medcyclopaedia/medical/Volume VII/SCHIZENCEPHALY.A
Amershamhealth.com Search for: Type a word or a phrase. All forms of the word are searchable. Browse entry words starting with: A B C D ... Other characters Schizencephaly, neuronal migration abnormality which occurs early in gestational life, possibly around the 7th to 8th week. It may be related to a vascular event. The resulting abnormality is a seam of grey matter extending from the pial surface of the brain down towards the ependyma of the lateral ventricle. This results in a grey matter lined cleft which can be described as open lipped, where there is a cerebrospinal fluid (CSF) track between the two sides, or closed lipped, where no CSF is identified. There is usually an associated abnormal contour of the lateral ventricle where the cleft meets the ventricular surface. In a large proportion of patients (70-80%) there will also be an absence of the septum pellucidum ( Fig.1 ). The abnormality is often found in the parietal lobe and can be bilateral ( Fig.2 ). The ventricular system may also be slightly dilated and the grey matter lining the cleft may be thickened. Children often present with seizures or developmental delay, but can have remarkably few symptoms. Also, see schizencephaly
NW
The Encyclopaedia of Medical Imaging Volume VII Schizencephaly, Fig. 1

28. Schizencephaly
schizencephaly,. Print this article, a greymatter-lined cleft that extends through the entire cerebral hemisphere from the ependymal
http://www.amershamhealth.com/medcyclopaedia/medical/Volume VI 1/SCHIZENCEPHALY.
Amershamhealth.com Search for: Type a word or a phrase. All forms of the word are searchable. Browse entry words starting with: A B C D ... Other characters Schizencephaly, a grey-matter-lined cleft that extends through the entire cerebral hemisphere from the ependymal surface of the lateral ventricle to the cerebral cortex. Two types of schizencephaly are recognized: a "closed-lip" schizencephaly in which the cleft walls are contiguous and an"open-lip" schizencephaly in which the walls are separated by wide subarachnoid spaces. The grey matter lining the cleft is usually polymicrogyric (see polymicrogyria ). The clefts can be unilateral or bilateral, symmetrical or asymmetrical and can appear anywhere in the brain. These lesions are believed to be due to in utero vascular injuries and their preferential location in the perisylvian areas follows the frontoparietal distribution of the middle cerebral artery. The clinical condition of patients relates to the amount and location of involved brain: patients with a narrow, unilateral cleft usually present with seizures and mild focal neurological deficits and they are otherwise developmentally normal; patients with bilateral clefts often have severe developmental delay with early intractable epilepsy and severe motor dysfunction. On imaging studies the key feature in the diagnosis of any type of schizencephaly is the presence of a ventricular dimple at the ependymal surface of the cleft. This can be the only CT feature in cases of closed or minimally open lip schizencephaly. On MR images the thickness of the transhemispheric cleft and the polymicrogyric aspect of the grey matter lining the cleft are better discernible (

29. Schizencephaly - Medical Dictionary Definitions Of Popular Medical Terms
schizencephaly A developmental disorder of the brain characterized by abnormal slits, or clefts, in the cerebral hemispheres. schizencephaly
http://www.medterms.com/script/main/art.asp?articlekey=15542

30. Schizencephaly - Condition, Disease, Or Disorder Article
schizencephaly Article relating to particular medical disease, condition, or disorder. schizencephaly. What is schizencephaly? schizencephaly
http://www.stopgettingsick.com/Conditions/condition_template.cfm/2489/283/1
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Schizencephaly What is Schizencephaly? Schizencephaly is an extremely rare developmental disorder characterized by abnormal slits, or clefts, in the brain's cerebral hemispheres. Schizencephaly is a form of porencephaly in which there is a cyst or cavity in the cerebral hemispheres. Individuals with clefts in both hemispheres (bilateral clefts) are commonly developmentally delayed and have delayed speech and language skills and corticospinal dysfunction. Individuals with smaller, unilateral clefts (clefts in only one hemisphere) are often paralyzed on one side of the body and may have normal intelligence. Patients with schizencephaly may also have varying degrees of microcephaly (abnormally small head), mental retardation, hemiparesis or quadriparesis (partial or complete paralysis), and reduced muscle tone (hypotonicity). Most patients have seizures. Some may have hydrocephalus. Is there any treatment?

31. Genetic Disorders, Schizencephaly
Category Home Health Conditions and Diseases Genetic Disorders schizencephaly. * Site Title · The name of the site. (eg
http://www.iseekhealth.com/directory/index.php?method=show_link_exchange&directo

32. KinderStart - Health/Medical/Dental : Major Diseases/Conditions : Schizencephaly
Vacations . Home Health/Medical/Dental Major Diseases/Conditions schizencephaly. Web Pages. The following links are in English
http://www.kinderstart.com/healthmedicaldental/majordiseasesconditions/schizence
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33. TheFetus.net - Schizencephaly, Type II -Vernon D. Byrd, MD*, Philippe Jeanty, MD
199105-12-10 schizencephaly, type II © Byrd http//www.thefetus.net/. schizencephaly, type II. The precise etiology of schizencephaly remains unknown.
http://www.thefetus.net/page.php?id=130

34. TheFetus.net - Schizencephaly, Unilateral -Bañuelos Acosta, MD
200009-01-12 schizencephaly, unilateral © Acosta http//fwww.thefetus.net/. schizencephaly, unilateral. Bañuelos Acosta, MD. Puerto Vallarta, Mexico.
http://www.thefetus.net/page.php?id=131

35. A Genetic Basis For Schizencephaly
A Genetic Basis for schizencephaly. Lhx2 may play a role in schizencephaly, septooptic dysplasia, and Joubert Syndrome. by Allen Ho.
http://hcs.harvard.edu/~husn/BRAIN/vol8-spring2001/schiz.htm
Volume 8 [Spring 2001]
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A Genetic Basis for Schizencephaly Lhx2 may play a role in Schizencephaly, septo-optic dysplasia, and Joubert Syndrome by Allen Ho is a LIM homeodomain gene expressed by proliferating cells in the developing cerebral cortex. Based on its expression pattern and similarities to knockout mice, was investigated as a candidate gene for the human disorder schizencephaly, a rare cortical malformation characterized by a full-thickness cleft within the cerebral hemispheres. Septo-optic dysplasia, a disorder often associated with schizencephaly, and Joubert Syndrome, a cerebellar disorder, were also investigated. The human genomic sequence was defined and PCR protocols were developed to amplify DNA samples from both blood and paraffin-embedded brain tissue. The human gene was sequenced in nine patients with one or more of these malformations, with no definite mutations found in the known functional domains sequenced so far. A heterozygous sequence alteration is reported involving a hydrophobic to hydrophilic amino acid change in a hydrophobic polyalanine stretch with unknown functional significance. Also, two other reported heterozygous sequence alterations are identified involving base changes not predicted to result in a change to the protein coding sequence.

36. IRSC - Rare Disorders, Schizencephaly
Rare Disorders schizencephaly.
http://www.irsc.org:8080/irsc/irscmain.nsf/sub?readform&cat=Rare Disorders&subca

37. IRSC - Rare Disorders, Schizencephaly
IRSC Link to IRSC. Rare Disorders schizencephaly. IRSC Home Rare Disorders schizencephaly. Categories. Online Community, Web Pages,
http://www.irsc.org:8080/irsc/irscmain.nsf/sub?readform&cat=Rare Disorders&subca

38. Christopher A. Walsh Laboratory - Schizencephaly
schizencephaly refers to a particular abnormality in the way that the brain developed during pregnancy, resulting in a cleft or a slit in the brain.
http://www.walshlab.org/schizencephaly.html
Schizencephaly refers to a particular abnormality in the way that the brain developed during pregnancy, resulting in a cleft or a slit in the brain. A cleft in schizencephaly extends from the surface of the brain all the way through to the ventricle, which is a normal fluid-filled space deep within the brain. The ventricle is directly connected to the surface of the brain in schizencephaly, which is not the case in the normal brain. The cleft can be bilateral or unilateral. The walls of the cleft can be touching each other or separated. Closed lip schizencephaly is the term used when the walls are touching each other, and open lip schizencephaly means that the walls are separated.
There may be many different causes of schizencephaly. Sometimes schizencephaly may result from problems during pregnancy, such as infection or exposure to certain drugs or toxins. In other cases, schizencephaly may be due to a mutation in a gene(s). Mutations in the EMX2 gene have been reported in a small number of patients, but this is not likely to be the case for most individuals with schizencephaly.
We are examining both the genetic and non-genetic causes of schizencephaly. Because many individuals with schizencephaly have not been found to have mutations within the EMX2 gene, our laboratory is searching for additional genes that may cause schizencephaly. Identification of the gene(s) will inform researchers of proteins that are important in brain development. Researchers can then study these proteins to learn how they function and this may lead to better treatment for individuals with schizencephaly in the future.

39. Christopher A. Walsh Laboratory - Schizencephaly
You’ve been told that your child has a condition called schizencephaly. This brochure gives you some general information about schizencephaly.
http://www.walshlab.org/schizencephaly_i.html
What is schizencephaly?
Schizencephaly is a medical term that refers to a particular abnormality in the way that the brain developed during pregnancy. Schizencephaly means that there is a cleft or a slit in the brain. A cleft in schizencephaly extends from the surface of the brain all the way through to the ventricle, which is a normal fluid-filled space deep within the brain. The ventricle is thus directly connected to the surface of the brain in schizencephaly, which is not the case in the normal brain. The cleft can be bilateral (on both sides of the brain) or unilateral (on one side of the brain). The walls of the cleft can be touching each other or separated. Closed lip schizencephaly is the term used when the walls are touching each other, and open lip schizencephaly means that the walls are separated.
Schizencephaly can occur by itself or it can occur in association with other abnormalities of the brain. For example, the brain tissue lining the walls of a cleft in schizencephaly often demonstrate polymicrogyria, another developmental abnormality. How your child may be affected by schizencephaly depends on many things, including where the cleft occurs, what type of schizencephaly he or she has, and whether or not other problems are associated with it.
How is schizencephaly diagnosed?

40. Unilateral Open-lip Schizencephaly With Absence Of Septum Pellucidum - Eurorad -
teaching files,radiology,Unilateral openlip schizencephaly with absence of septum pellucidum,A 3 year-old male patient presented with partial complex
http://www.eurorad.org/case.cfm?uid=1081

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