methylenetetrahydrofolate reductase deficiency

ORPHA: 395

Overview

medical condition

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 methylenetetrahydrofolate reductase deficiency, sourced from HPO and Orphanet clinical annotations.

Atypical behaviorGait disturbanceMorphological central nervous system abnormalityLower limb spasticityHomocystinuriaHyperhomocystinemiaUpper motor neuron dysfunctionCystathioninemiaLower limb muscle weaknessAbnormal circulating enzyme concentration or activityPsychotic episodesSeizureAtaxiaMental deteriorationSpastic paraparesisAbnormal periventricular white matter morphologyHypomethioninemiaPeripheral neuropathyBrain imaging abnormalityPsychosisIntellectual disabilityLethargyGlobal developmental delayHemiparesisSpecific learning disabilityPsychotic mentationFailure to thriveThromboembolic strokeAbnormal thrombosisBilateral tonic-clonic seizureApneaHeadacheAbnormal cerebral white matter morphologyDeep venous thrombosisAtrophy of the spinal cordFocal-onset seizureFeeding difficulties in infancyGeneralized neonatal hypotoniaBrain atrophyCognitive impairmentHydrocephalusMicrocephalyAbnormality of the eyeNystagmusOptic atrophyStrokeEncephalopathyVentriculomegalyGeneralized non-motor (absence) seizureGeneralized myoclonic seizure

Classification & Codes

Orphanet Code

ORPHA:395
methylenetetrahydrofolate reductase deficiency
OrphanetORPHA:395
Treatments0 drug(s)
Symptoms on record50 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO