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American Journal of Neuroradiology 25:1046-1048, June-July 2004
© 2004 American Society of Neuroradiology

Case Report
PEDIATRICS

MR Imaging of Brain-Stem Hypoplasia in Horizontal Gaze Palsy with Progressive Scoliosis

Andrea Rossia, Martin Catalac, Roberta Biancheria, Raffaella Di Comiteb and Paolo Tortori-Donatia

a Department of Pediatric Neuroradiology, G. Gaslini Children’s Research Hospital, Genoa, Italy
b Division of Infantile Neuropsychiatry, G. Gaslini Children’s Research Hospital, Genoa, Italy
c Laboratory of Histology, Embryology, and Cytogenetics, Faculty of Medicine Pitiè-Salpêtriere, Paris, France

Address correspondence to Andrea Rossi, MD, Department of Pediatric Neuroradiology, G. Gaslini Children’s Research Hospital, Largo G. Gaslini 5, I-16147 Genoa, Italy

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.






Copyright © 2008 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X