Adrenomyeloneuropathy

ICD-10: E71.522ORPHA: 139399

Contents

1. Overview
2. Available Treatments
3. Clinical Presentation
4. Classification & Codes

Overview

Adrenomyeloneuropathy (AMN) is an adult form of the peroxisomal disease X-linked adrenoleukodystrophy (X-ALD, see this term), characterized by spastic paraparesia and often associated with peripheral adrenal insufficiency in males

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 Adrenomyeloneuropathy, sourced from HPO and Orphanet clinical annotations.

Peripheral axonal degenerationAbnormal spinal cord morphologyDistal sensory impairmentAbnormal circulating fatty-acid concentrationProgressive spastic paraparesisDistal lower limb muscle weaknessAbnormality of peripheral nervous system electrophysiologyImpaired continenceAxonal degenerationAdrenocortical abnormalityAbnormality of skin pigmentationSpasticityHyperreflexiaSpastic gaitFine hairBowel incontinenceUrinary bladder sphincter dysfunctionBabinski signAtrophy of the spinal cordDorsal column degenerationCerebral dysmyelinationAtrophy/Degeneration involving the corticospinal tractsVery long chain fatty acid accumulationLeg muscle stiffnessPeripheral neuropathyIntra-oral hyperpigmentationAdrenocorticotropic hormone excessFatigueFemale sexual dysfunctionAbnormal libidoMale sexual dysfunctionDelayed somatosensory central conduction timeErectile dysfunctionLip hyperpigmentationBrain imaging abnormalityUrinary urgencyUrinary retentionUrinary incontinenceAtypical behaviorAdrenal insufficiencyDysarthriaFrontal baldingMemory impairmentBack painFunctional motor deficitPrimary adrenal insufficiencyDysesthesiaDecreased circulating vitamin B12 concentrationCognitive impairment

Classification & Codes

ICD-10 Code

E71.522

Orphanet Code

ORPHA:139399

Adrenomyeloneuropathy

ICD-10	`E71.522`
Orphanet	`ORPHA:139399`
Treatments	0 drug(s)
Symptoms on record	49 signs
Status	published

Factual Authority

Last Updated3/20/2026

Clinical DataHPO · Orphanet

Drug DataFDA · EMA · CDSCO