A RARE CASE OF BILATERAL DUANE RETRACTION SYNDROME WITH BILATERAL CEREBELLAR ATROPHY
Abstract
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.
Keyword :Duane retraction syndrome,
cerebellar atrophy, abduction, adduction
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