Affiliations: Developmental Pediatrics Unit, Department of Child Health, Christian Medical College, Vellore, Tamil Nadu, India | Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
Note: [] Corresponding author: Dr. Samuel P. Oommen, Developmental Pediatrics Unit, Department of Child Health and Neonatology, Christian Medical College, Vellore, Tamil Nadu, India. Tel.: +91 416 228 3260; Fax: +91 416 223 2035; E-mail: docspo91@yahoo.com, devpaed@cmcvellore.ac.in.
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.