metachromatic leukodystrophy, adult form

ORPHA: 3092711 Treatment Available

Overview

human disease

Available Treatments (1)

DrugFormStatusCountriesLead Time
atidarsagene autotemcel
Orphan Cold Chain
IV infusion, cell suspensionEMA Approved690d

Clinical Presentation

Signs and symptoms associated with metachromatic leukodystrophy, adult form, sourced from HPO and Orphanet clinical annotations.

Optic atrophyEmotional labilityDementiaShort attention spanHallucinationsDelusionDecreased nerve conduction velocityDysarthriaHyporeflexiaGait disturbanceGeneralized hypotoniaMuscle weaknessDystoniaClumsinessMemory impairmentFrequent fallsDevelopmental regressionLeukodystrophyIncreased CSF protein concentrationAbnormal glycosphingolipid metabolismAbnormal social behaviorPunctate periventricular T2 hyperintense fociUrinary incontinenceAbnormality of visual evoked potentialsDepressionCholecystitisSpasticityAbnormality of metabolism/homeostasisChoreaIntention tremorLoss of speechProgressive spastic quadriplegiaBulbar signsBowel incontinenceAbdominal distentionEMG: chronic denervation signsBabinski signProgressive peripheral neuropathyProgressive gait ataxiaProgressive psychomotor deteriorationReduced visual acuityBilateral sensorineural hearing impairmentSchizophreniaSeizureOrthostatic hypotension due to autonomic dysfunctionDecerebrate rigidityVegetative stateNeoplasm of the gallbladder

Classification & Codes

Orphanet Code

ORPHA:309271
metachromatic leukodystrophy, adult form
OrphanetORPHA:309271
Treatments1 drug(s)
Symptoms on record48 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO