aceruloplasminemia

ICD-10: E83.1MeSH: C536004ORPHA: 48818

Overview

adult-onset disorder of neurodegeneration with brain iron accumulation

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 aceruloplasminemia, sourced from HPO and Orphanet clinical annotations.

Abnormality of the nervous systemIncreased circulating ferritin concentrationHypochromic microcytic anemiaRefractory anemiaAbnormal circulating enzyme concentration or activityAceruloplasminemiaRetinal degenerationMacular degenerationDiabetes mellitusAtaxiaDysarthriaDystoniaGait ataxiaLimb ataxiaChoreaInvoluntary movementsAbnormal retinal pigmentationDecreased circulating ceruloplasmin concentrationDecreased circulating copper concentrationElevated hepatic iron concentrationDecreased circulating iron concentrationCognitive impairmentFacial grimacingTorticollisNystagmusBlepharospasmApathyParkinsonismTremorCongestive heart failureRigidityAkinesiaMemory impairmentAbnormal corpus striatum morphologyAbnormal pancreas morphologyCraniofacial dystoniaIron accumulation in brainAbnormal thalamic MRI signal intensityAbnormal dentate nucleus morphology

Classification & Codes

ICD-10 Code

E83.1

MeSH Code

C536004

Orphanet Code

ORPHA:48818
aceruloplasminemia
ICD-10E83.1
MeSHC536004
OrphanetORPHA:48818
Treatments0 drug(s)
Symptoms on record39 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO