A RARE CASE OF BILATERAL DUANE RETRACTION SYNDROME WITH BILATERAL CEREBELLAR ATROPHY

Duane's retraction syndrome is characterized by globe retraction and palpebral fissure narrowing on adduction, with restriction of abduction or adduction or both. The proposed mechanism for the impaired ocular motility is congenital miswiring of the lateral and medial rectus muscles. Electromyographic studies show decreased or absent firing of the lateral rectus and medial rectus during attempted adduction causing globe retraction. It may be associated with other ocular as well as systemic congenital anomalies such as sensorineural deafness, upper limbs defect, facioauriculovertebral anomalies, cardiac and genitourinary anomalies. We report a case of rare association of bilateral Duane retraction syndrome with bilateral cerebellar atrophy in a 28 year-old male. History, the ocular motility examination and neurological examination revealed that our patient has congenital bilateral Duane retraction syndrome with bilateral cerebellar signs. We concluded that there is novel association between Duane retraction syndrome with chronic progressive bilateral cerebellar degeneration.