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MR Imaging of Brain-Stem Hypoplasia in Horizontal Gaze Palsy with Progressive Scoliosis AJNR 25:1046-1048, Rossi, A.,et al, 2004 See this aricle in Pubmed Article 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.

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(click to filter results - removes previous filter) brainstem,hypoplasia congenital malformation gaze palsy gaze palsy,congenital horizontal gaze palsy,horizontal gaze palsy,horizontal-bilateral HGPPS MRI,abnormal scoliosis

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