mitochondrial myopathy with reversible cytochrome c oxidase deficiency

ORPHA: 254864

Overview

human disease

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 mitochondrial myopathy with reversible cytochrome c oxidase deficiency, sourced from HPO and Orphanet clinical annotations.

Generalized hypotoniaMuscle weaknessMyopathyRagged-red muscle fibersCytochrome C oxidase-negative muscle fibersIncreased muscle glycogen contentIncreased muscle lipid contentHyporeflexiaRespiratory distressSevere lactic acidosisMildly elevated creatine kinaseDecreased activity of mitochondrial complex IMacroglossiaHigh palateAbnormality of the nervous systemAbnormality of the liverAbnormality of the cardiovascular systemPoor suckHepatomegalyDecreased circulating carnitine concentrationRespiratory failure requiring assisted ventilationVentilator dependence with inability to weanNasogastric tube feeding in infancyDelayed gross motor development

Classification & Codes

Orphanet Code

ORPHA:254864
mitochondrial myopathy with reversible cytochrome c oxidase deficiency
OrphanetORPHA:254864
Treatments0 drug(s)
Symptoms on record24 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO