HORIZONTAL GAZE PALSY WITH PROGRESSIVE SCOLIOSIS: CASE REPORT

Résumé

Purpose: horizontal gaze palsy with progressive scoliosis (HGPPS) is an autosomal recessive disorder characterized by congenital absence of horizontal gaze, progressive scoliosis, and failure of the corticospinal and somatosensory axon tracts to decussate in the medulla. Case description: man with sporadic HGPPS, 26 years old, of non-consanguineous parents. Pupils were normal and there was full vertical motility. Horizontal saccades and pursuit movements were absent, and vestibulo-ocular reflex testing produced no horizontal eye movements. While in his adolescence, the patient presented scoliosis and underwent surgical treatment to avoid its progression. Conclusions:  the HGPPS is a rare syndrome. Any patient with absence of horizontal gaze should be evaluated comprehensively to rule out other syndromic associations.