Journal of Pediatric Neuroradiology 2012; 01(04): 299-303
DOI: 10.3233/PNR-2012-038
Georg Thieme Verlag KG Stuttgart – New York

Middle interhemispheric variant of holoprosencephaly syntelencephaly

Samuel P. Oommen
a   Developmental Pediatrics Unit, Department of Child Health, Christian Medical College, Vellore, Tamil Nadu, India
,
Anitha Jasper
b   Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
,
Gibikote Sridhar
b   Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
,
Jeyanthi Govindarajan
b   Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
› Author Affiliations

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Further Information

Publication History

16 February 2011

27 July 2011

Publication Date:
28 July 2015 (online)

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.