DR. SWASTI PANDEY

DR. SHANKAR NARAYAN A, DR. TANMAY SRIVASTAV, DR. KIRTI VERMA

Abstract

Purpose- Kearns Sayre Syndrome is due to mitochondrial myopathy with systemic and ocular findings like cardiac conduction defects,ataxia ,T1DM,ptosis,pigmentary retinopathy and chronic progressive external ophthalmoplagia.Method: A 29 yr old girl presented with complaints of drooping of bilateral upper eyelids and restriction of eyeball movements in all directions of gaze.On taking history,parents reported onset of drooping of eyelids at 2 years and 6 years of age,right followed by left eye.She had surgery for same at 8 years of age.The movements of eyeball initially were restricted in lateral and medial gazes that progressively became fully restricted by the time she turned 14.She also at the same time presented with 2-3 episodes of loss of consciousness ,tremors and palpitations and was referred to Neurology and Endocrine.

Result: On Systemic workup ataxia,dysarthria and T1DM was found.

Conclusion: Ptosis due to Kearns Sayre is rare and should be differentiated for proper management.