DUANES RETRACTION SYNDROME AND SPASTIC PARAPARESIS

Duane's retraction syndrome (DRS) is a neurogenic eye movement abnorma lity frequently associated with other congenital defects involving ocu lar, skeletal and neural structures. The authors report on a patient w ho had DRS associated with a severe spastic paraparesis, which progres sed from childhood until young adulthood with subsequent stabilization . The patient had severe limitation of abduction of the left eye, narr owing of the palpebral fissure, and globe retraction in adduction. Neu rological examination revealed a severe spastic paraparesis. Extensive clinical neurophysiological evaluation, including electromyography, n erve conduction studies, somatosensory evoked responses and transcrani al magnetic stimulation, was consistent with a severe myelopathy. Magn etic resonance imaging of the spinal cord demonstrated significant thi nning of the cervical and thoracic spinal cord. This previously unrepo rted association is unlikely coincidental, given the rarity of both fi ndings, and may be explained by the abnormal development of neural str uctures during a critical period of gestation.