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Duane Syndrome

Symptoms

-present from birth but may take several years to be diagnosed

-holding head in abnormal position

-crossing or misalignment of eyes

-closing one eye to see better

-double vision

-headaches

-difficulty seeing on side of affected eye

Cause

-genetic and environmental disturbances during embryonic development

-can be autosomal dominant or a single occurance in a family

-Mutations in the CHN1 (chimerin 1) gene located on chromosome 2

CHN1 gene

Type 2

-Provides instructions for making proteins; a1-chimaerin and a2-chimaerin

-Critial during formation of nerves in head and face

-The mutation overactivates the a2-chimaern

disrupting the growth of neurons resulting in muscles surround the eye not developing properly

-inability to move eye inward

What is Duane Syndrome?

Type 3

inability to move

eye in or out

Type 1

inability to move

eye outward

-miswiring of the eye muscles

-eye muscles may contract when

they should not or not contract when they should

Treatments

-limited ability to move the eye inward

or outward

-Surgery: goal is to eliminate or improve unacceptable head turns and misaligning eyes and reduce severe retraction.

-Visual Therapy

80% of cases only 1 eye is affected, usually left eye

Quality of Life

-Almost completely normal

-special seating in class for children

-special rear-view mirrors for driving

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