Syntelencephaly

Case contributed by Dr Dalia Ibrahim

Presentation

Convulsions.

Patient Data

Age: Infant
Gender: Male
MRI

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

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Case information

rID: 90878
Published: 3rd Jul 2021
Last edited: 5th Jul 2021
Inclusion in quiz mode: Included

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