A Case Report on Kearns Sayre Syndrome: A Rare Oculomyoencephalopathy
Journal: International Journal of Science and Research (IJSR) (Vol.10, No. 6)Publication Date: 2021-06-05
Authors : Abhishikta Chakraborty; Utkarsh Rai; Jawahar Jyoti Kuli;
Page : 269-271
Keywords : KSS; Ptosis; Hearing loss; conductive hearing loss; Sensorineural Hearing Loss; cardiac abnormalities; red ragged fibers; EOM restriction; skeletal muscle biopsy;
Abstract
A 22 year short-statured female presented with progressive drooping of both upper eyelids, gradually progressive restriction of eye movements in all gazes. No complaint of diplopia or diminution of vision, no diurnal variation; no history of prior ocular surgery or trauma. She complained of generalised weakness and mild hearing loss. BCVA was 6/6 OU. Chin & Brow elevation present, B/L mild ptosis with preserved Bell?s phenomenon. B/L EOM movement limited in all gazes. Direct & consensual reflexes normal. B/L pigmentary retinopathy was present. Audiometry showed B/L sensorineural deafness. ECG showed AV conduction delay with LVH. Multidisciplinary management for ptosis correction, vision & hearing aids was given.
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Last modified: 2021-07-05 13:46:22