Kearns-Sayre syndrome

MeSH: D007625ORPHA: 480

Overview

spontaneous occuring or inherited mitochondrial myopathy with a typical onset before 20 years of age

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with Kearns-Sayre syndrome, sourced from HPO and Orphanet clinical annotations.

Pigmentary retinopathyProgressive external ophthalmoplegiaThird degree atrioventricular blockHearing impairmentPtosisAnterior hypopituitarismAtaxiaHypotoniaReduced tendon reflexesIncreased CSF protein concentrationRagged-red muscle fibersSkeletal muscle atrophyEMG abnormalityProgressive intervertebral space narrowingNeck muscle weaknessDementiaDiabetes mellitusDelayed pubertyHypoparathyroidismMuscle weaknessTremorCardiomyopathyRenal tubular acidosisDysphagiaDelayed skeletal maturationExercise intoleranceShort statureHemiplegia/hemiparesisChronic kidney diseaseCognitive impairment

Classification & Codes

MeSH Code

D007625

Orphanet Code

ORPHA:480
Kearns-Sayre syndrome
MeSHD007625
OrphanetORPHA:480
Treatments0 drug(s)
Symptoms on record30 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO