Middle interhemispheric variant of holoprosencephaly syntelencephaly

Abstract

Middle interhemispheric holoprosencephaly (MIH) or syntelencephaly is a variant of holoprosencephaly in which the posterior frontal and parietal lobes are fused, while the frontal poles and the occipital lobes are separated by the interhemispheric fissure. We describe a two and half year old girl who presented with spastic diplegia, in whom the magnetic resonance imaging revealed MIH. Her ambulation was limited by the spasticity of her limbs but her language, social and adaptive skills were much ahead of her motor abilities. Etiopathogenesis of MIH and its differences from the ‘classic’ forms of holoprosencephaly are also discussed.

Keywords

Syntelencephaly - middle interhemispheric variant - holoprosencephaly