This document summarizes a case study of an 11-year-old female patient presented with congenital left sided hemiaplasia and Lennox Gastaut syndrome. MRI images showed abnormalities including a large right-sided cyst, agenesis of the septum pellucidum, lissencephaly, hemimegalencephaly, and heterotopias. The patient was diagnosed with Aicardi syndrome associated with multiple cortical dysplasias. The author provides criteria for suggestive of Aicardi syndrome and information on accessing additional case studies and publications on the author's website.

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. 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. 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. 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. 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