2008
DOI: 10.1007/s00247-008-1058-8
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Horizontal gaze palsy with progressive scoliosis: CT and MR findings

Abstract: Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare congenital disorder characterized by absence of conjugate horizontal eye movements and progressive scoliosis developing in childhood and adolescence. We present a child with clinical and neuroimaging findings typical of HGPPS. CT and MRI of the brain demonstrated pons hypoplasia, absence of the facial colliculi, butterfly configuration of the medulla and a deep midline pontine cleft. We briefly discuss the imaging aspects of this rare entity in… Show more

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Cited by 22 publications
(26 citation statements)
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“…However, it is less known that this sign may be elicited in normal infants up to 7 months of age [6] by a sudden change in head position or by shining a bright light in front of infants 20-40 weeks of age and suddenly removing it [7]. Other rare gaze abnormalities include horizontal gaze palsy with progressive scoliosis [8,9] and progressive upward gaze paralysis that may suggest the presence of lipid storage disease [10,11]. However, this may also be a benign phenomenon.…”
Section: Discussionmentioning
confidence: 99%
“…However, it is less known that this sign may be elicited in normal infants up to 7 months of age [6] by a sudden change in head position or by shining a bright light in front of infants 20-40 weeks of age and suddenly removing it [7]. Other rare gaze abnormalities include horizontal gaze palsy with progressive scoliosis [8,9] and progressive upward gaze paralysis that may suggest the presence of lipid storage disease [10,11]. However, this may also be a benign phenomenon.…”
Section: Discussionmentioning
confidence: 99%
“…1). Abnormalities of horizontal gaze may be seen in children due to focal neurologic lesions or congenital syndromes such as Mobius syndrome or horizontal gaze palsy with progressive scoliosis [1,2]. Here, we present the unusual finding of transient horizontal gaze palsy associated with skull fracture and focal traumatic subarachnoid hemorrhage in a young infant.…”
Section: Introductionmentioning
confidence: 96%
“…These radiological findings are typical for HGPPS patients. 6 Both physical and radiological examinations strongly suggest a diagnosis of HGPPS.…”
mentioning
confidence: 99%