MR imaging of brain-stem hypoplasia in horizontal gaze palsy with progressive scoliosis

Andrea Rossi, Martin Catala, Roberta Biancheri, Raffaella Di Comite, Paolo Tortori-Donati

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)1046-1048
Number of pages3
JournalAmerican Journal of Neuroradiology
Volume25
Issue number6
Publication statusPublished - Jun 2004

ASJC Scopus subject areas

  • Clinical Neurology
  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology

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