PT  - JOURNAL ARTICLE
AU  - Rossi, Andrea
AU  - Catala, Martin
AU  - Biancheri, Roberta
AU  - Di Comite, Raffaella
AU  - Tortori-Donati, Paolo
TI  - MR Imaging of Brain-Stem Hypoplasia in Horizontal Gaze Palsy with Progressive Scoliosis
DP  - 2004 Jun 01
TA  - American Journal of Neuroradiology
PG  - 1046--1048
VI  - 25
IP  - 6
4099  - http://www.ajnr.org/content/25/6/1046.short
4100  - http://www.ajnr.org/content/25/6/1046.full
SO  - Am. J. Neuroradiol.2004 Jun 01; 25
AB  - Summary: We present the MR imaging findings of a girl with horizontal gaze palsy and progressive scoliosis (HGPPS). HGPPS is a rare congenital disorder characterized by absence of conjugate horizontal eye movements and accompanied by progressive scoliosis developing in childhood and adolescence. MR imaging depicted brain-stem hypoplasia with absence of the facial colliculi, presence of a deep midline pontine cleft (split pons sign), and a butterfly configuration of the medulla. These MR imaging features suggest the diagnosis of HGPPS and correlate with the clinical findings. We hypothesize that maldevelopment of dorsomedial brain-stem structures plays a crucial role in the pathogenesis of HGPPS.