maternally-inherited progressive external ophthalmoplegia

ORPHA: 663

Overview

Orphanet summary for this disease is currently under development. However, other data related to the disease are accessible from the Additional Information menu located on the right side of this page

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 maternally-inherited progressive external ophthalmoplegia, sourced from HPO and Orphanet clinical annotations.

Progressive external ophthalmoplegiaMuscle abnormality related to mitochondrial dysfunctionPtosisHypothyroidismBrisk reflexesRestrictive ventilatory defectIncreased circulating lactate concentrationRespiratory insufficiency due to muscle weaknessRagged-red muscle fibersAxial muscle weaknessEMG abnormalityMildly elevated creatine kinaseAbnormal mitochondria in muscle tissueProgressive proximal muscle weaknessDepressionMild intellectual disability

Classification & Codes

Orphanet Code

ORPHA:663
maternally-inherited progressive external ophthalmoplegia
OrphanetORPHA:663
Treatments0 drug(s)
Symptoms on record16 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO