mitochondrial complex II deficiency

MeSH: C565375ORPHA: 3208

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 complex II deficiency, sourced from HPO and Orphanet clinical annotations.

Lower limb hypertoniaFeeding difficulties in infancyFocal myoclonic seizureModerate global developmental delayNoncompaction cardiomyopathyMild microcephalyVesicoureteral refluxExternal ophthalmoplegiaPigmentary retinopathyBlindnessNystagmusDementiaPoor head controlLoss of ambulationAbnormality of the eyeSpasticityMotor delayAbnormality of the cardiovascular systemHypertrophic cardiomyopathyLeft ventricular hypertrophyWeight lossGeneralized myoclonic seizureMotor deteriorationDevelopmental regressionGeneralized muscle weaknessEasy fatigabilityBabinski signProportionate short statureSevere short statureDistal amyotrophyProximal muscle weaknessSkeletal myopathyAbnormal left ventricular functionHyperactive deep tendon reflexesHyperactive patellar reflexProgressive psychomotor deteriorationUpper limb hyperreflexiaReduced visual acuityIrritabilitySeizureAtaxiaSpastic tetraparesisGeneralized hypotoniaIntrauterine growth retardationSpastic paraparesisFrequent fallsExpressive language delaySkeletal muscle atrophyAbnormal atrioventricular conductionKnee flexion contracture

Classification & Codes

MeSH Code

C565375

Orphanet Code

ORPHA:3208
mitochondrial complex II deficiency
MeSHC565375
OrphanetORPHA:3208
Treatments0 drug(s)
Symptoms on record50 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO