Aicardi syndrome

Case contributed by Magdi Mahsoub
Diagnosis certain

Presentation

Presenting to the emergency department with headache, dizziness, and left-side weakness.

Patient Data

Age: 35 years
Gender: Female
ct
This study is a stack
Axial
non-contrast
This study is a stack
Axial
non-contrast
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 Agenesis of corpus callosum with colpocephaly and high riding third ventricle.

Small nodules isodense to gray matter at the lateral walls of both lateral ventricles suggesting subependymal heterotopia.

Right parasagittal interhemispheric cyst.

Extra-axial cystic lesion at the right side of the posterior cranial fossa causing mass effect on the right cerebellar hemisphere suggesting arachnoid cyst.

 Fusiform focal lesion centered on the right optic nerve.

mri
This study is a stack
Axial
T1
This study is a stack
Axial
T2
This study is a stack
Axial
FLAIR
This study is a stack
Axial
DWI
This study is a stack
Axial
ADC
This study is a stack
Sagittal
T1
This study is a stack
Coronal
T2
This study is a stack
Coronal
FLAIR
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Agenesis of the corpus callosum with widely separated parallel bodies of lateral ventricles, colpocephaly, high riding third ventricle communicating with interhemispheric cistern, Absent cingulate gyrus with radial medial hemispheric gyri on sagittal view, and moose head appearance of lateral ventricles on coronal view.

Right medial frontal polymicrogyria.

Subependymal heterotopia.

Right parasagittal interhemispheric cyst.

Cystic lesion at the right aspect of the posterior cranial fossa causing mass effect on the right cerebellar hemisphere suggesting arachnoid cyst.

Relatively small right eye globe (microphthalmos) with right optic nerve cystic lesion.

Case Discussion

This patient is a known case of Aicardi syndrome with typical radiological features.

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