Short case...Aicardi syndrome

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Short case...Aicardi syndrome

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Short case...Aicardi syndrome

  1. 1. Short case publication... version 2.4 | Edited by professor Yasser Metwally | October 2008 Short case Edited by Professor Yasser Metwally Professor of neurology Ain Shams university school of medicine Cairo, Egypt Visit my web site at: http://yassermetwally.com 11 years old female patient presented with congenital left sided hemiaplasia and Lennox Gastaut syndrome. The patient's family gave a history of west syndrome during the first year of the patient's life. Fundus examination revealed chorioretinal lacunae. EEG examination revealed 1.5 C/S slow spike/slow wave discharge of Lennox Gastaut syndrome. The patient's scholastic achievement was very poor. DIAGNOSIS: AICARDI SYNDROME ASSOCIATED WITH MULTIPLE CORTICAL DYSPLASIAS THAT INCLUDE HEMIMEGALENCEPHALY, LISSENCEPHALY, HETEROTOPIAS, AND SEPTO-OPTIC DYSPLASIA.
  2. 2. Figure 1. Precontrast MR T1 images showing a huge right sided intraventricular /parenchymal cyst associated with agenesis of the septum pellucidum. The corpus callosum is markedly hypoplastic and deficient. The cerebral cortex is lissencephalic. Notice the right sided hemimegalencephaly and the subependymal nodular heterotopia. Subcortical band heterotopia can also be appreciated. Figure 2. Precontrast MR T1 images showing a huge right sided intraventricular /parenchymal cyst associated with agenesis of the septum pellucidum. The corpus callosum is markedly hypoplastic and deficient. The cerebral cortex is lissencephalic. Notice the right sided hemimegalencephaly and the subependymal nodular heterotopia.
  3. 3. Figure 3. MR T2 images showing a huge right sided intraventricular /parenchymal cyst associated with agenesis of the septum pellucidum. The corpus callosum is markedly hypoplastic and deficient. The cerebral cortex is lissencephalic. Notice the right sided hemimegalencephaly and the subependymal nodular heterotopia. Subcortical band heterotopia can also be appreciated. Figure 4. Precontrast MR T1 images showing a huge right sided intraventricular /parenchymal cyst. The corpus callosum is markedly hypoplastic and deficient. The cerebral cortex is lissencephalic. Notice the subependymal nodular heterotopia and hypoplasia of the optic nerve. The cerebellum and brain stem are also hypoplastic.
  4. 4. Figure 5. Precontrast MR T1 images showing A huge right sided intraventricular /parenchymal cyst. The cerebral cortex is lissencephalic. Notice the right sided hemimegalencephaly and the subependymal nodular heterotopia. Subcortical band heterotopia can also be appreciated. Figure 6. Precontrast MR T1 images showing marked hypoplasia of the cerebellum and brain stem.
  5. 5. Figure 7. Chorioretinal lacunae Criteria that are highly suggestive of Aicardi syndrome Partial or complete callosal agenesis Cortical dysplasia Gross asymmetry of the hemispheres Periventricular or subcortical heterotopias Cysts of the choroid plexus or around the third ventricle is highly suggestive of AS Addendum A new version of short case is uploaded in my web site every week (every Saturday and remains available till Friday.) To download the current version follow the link quot;http://pdf.yassermetwally.com/short.pdfquot;. You can download the long case version of this short case during the same week from: http://pdf.yassermetwally.com/case.pdf or visit web site: http://pdf.yassermetwally.com To download the software version of the publication (crow.exe) follow the link: http://neurology.yassermetwally.com/crow.zip At the end of each year, all the publications are compiled on a single CD-ROM, please contact the author to know more details. Screen resolution is better set at 1024*768 pixel screen area for optimum display For an archive of the previously reported cases go to www.yassermetwally.net, then under pages in the right panel, scroll down and click on the text entry quot;downloadable short cases in PDF formatquot; Also to view a list of the previously published case records follow the following link (http://wordpress.com/tag/case- record/) or click on it if it appears as a link in your PDF reader References 1. Metwally, MYM: Textbook of neurimaging, A CD-ROM publication, (Metwally, MYM editor) WEB-CD agency for electronic publishing, version 9.4a October 2008

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