Teaching Video NeuroImages: Horizontal gaze palsy with progressive scoliosis

Abstract

A 10-year-old girl presented with abnormal eye movement since birth. She was born from consanguineous parents. Ocular ductions showed complete horizontal gaze palsy, partial limitation of upgaze, and synergistic convergence (figure, A; video 1). Her pupils were round and isocoric. She showed severe thoracolumbar scoliosis. Uncrossed corticospinal and dorsal column–medial lemniscal pathways were demonstrated during intraoperative neuromonitoring of scoliosis surgery. Brain MRI revealed a bifid shape of the pons and medulla oblongata (figure, B–D), which is a typical finding of horizontal gaze palsy with progressive scoliosis.1,2 Bilateral oculomotor, trochlear, and abducens nerves were normally identified (figure, E).