Presentation
Convulsions.
Patient Data
Age: Infant
Gender: Male
From the case:
Syntelencephaly
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Cerebral hemispheres show:
- fusion of the middle portions of both cerebral hemispheres with continuous white matter across middle part of interhemispheric region
- absent mid-body of the corpus callosum with present genu and splenium
- present interhemispheric fissure and azygous ACA
- bilateral frontal and pareital parafalcine polymicrogyri
Lateral ventricles show hypoplastic frontal horns with absent septum pellucidum
The mesencephalon and the pituitary gland have a normal morphology
The posterior fossa appear normally formed
Case Discussion
Syntelencephaly is a variant of semilobar holoprosencephaly , also known as middle Interhemisheric variant. It's characterized by an abnormal midline connection of the cerebral hemispheres between the posterior frontal and parietal regions.
This case is courtesy o Dr Mostafa El Khashab, MD of radiodiagnosis