A 14 yo male child, birth history uneventful.Complaints multiple episodes of seizures since 10 months with history of developmental delay and clinical impression of cerebral palsy with epilepsy
On MRI,
The interhemispheric fissure not visualized in fronto parietal region with midline fusion of the cerebral hemispheres, single lateral ventricle cavity.
The sylvian fissure extending across the midline. Body of corpus callosum is deficient.
Normal 3rd and 4th ventricle.
The fused superior frontal cortex is thick , has a agyric / polymicrogyric pattern.
Diagnosis : Syntelencephaly.
A varient of semilobar holoprosencephaly _ Congenital malformation of brain also known as Middle Interhemisheric variant.
A rare malformation in which the cerebral hemisphere fails to divide in the posterior frontal and parietal region.
Contributed by Dr Mitusha Verma SR DNB Radiology Nanavati.
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