PT  - JOURNAL ARTICLE
AU  - Hahn, J.S.
AU  - Barnes, P.D.
AU  - Clegg, N.J.
AU  - Stashinko, E.E.
TI  - Septopreoptic Holoprosencephaly: A Mild Subtype Associated with Midline Craniofacial Anomalies
AID  - 10.3174/ajnr.A2123
DP  - 2010 Oct 01
TA  - American Journal of Neuroradiology
PG  - 1596--1601
VI  - 31
IP  - 9
4099  - http://www.ajnr.org/content/31/9/1596.short
4100  - http://www.ajnr.org/content/31/9/1596.full
SO  - Am. J. Neuroradiol.2010 Oct 01; 31
AB  - SUMMARY: HPE is a congenital brain malformation characterized by failure of the prosencephalon to divide into 2 hemispheres. We have identified 7 patients who have a mild subtype of HPE in which the midline fusion was restricted to the septal region or preoptic region of the telencephalon. This subtype, which we call septopreoptic HPE, falls in the spectrum of lobar HPE, but lacks significant frontal neocortical fusion seen in lobar HPE. Other imaging characteristics include thickened or dysplastic fornix, absent or hypoplastic anterior CC, and single unpaired ACA. The SP was fully formed in 4, partially formed in 2, and absent in 1. Mild midline craniofacial malformation, such as SMMCI and CNPAS were found in 86% and 71%, respectively. Patients outside of infancy often manifested language delay, learning disabilities, or behavioral disturbances, while motor function was relatively spared. ACanterior commissureACAanterior cerebral arteryCCcorpus callosumCNPAScongenital nasal piriform aperture stenosisFSPGRfast-spoiled gradient-recalledHPEholoprosencephalyIHFinterhemispheric fissureMPRmultiplanar reconstructedSMMCIsingle median maxillary central incisorSPseptum pellucidumSPGRspoiled gradient-recalledV3third ventricle