late-infantile/juvenile Krabbe disease

ORPHA: 206443

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 late-infantile/juvenile Krabbe disease, sourced from HPO and Orphanet clinical annotations.

TremorSlurred speechPes cavusNeuromuscular dysphagiaHemiplegiaFebrile seizure (within the age range of 3 months to 6 years)TetraplegiaUpper limb muscle weaknessAttention deficit hyperactivity disorderAxial hypotoniaImpaired tactile sensationFeeding difficultiesAcroparesthesiaIrritabilityIncreased CSF protein concentrationReduced tissue galactocerebrosidase activityHearing impairmentEsotropiaVisual lossAbnormality of visual evoked potentialsAtypical behaviorDecreased nerve conduction velocitySpastic diplegiaMental deteriorationMotor delayGait disturbanceLower limb spasticityClumsinessSpastic paraparesisFrequent fallsLoss of speechDevelopmental regressionUpper motor neuron dysfunctionLoss of ambulationFunctional motor deficitDelayed brainstem auditory evoked response conduction timeSensorimotor neuropathyPeripheral neuropathyEEG with persistent abnormal rhythmic activityAbnormal CNS myelinationVisual impairmentBlindnessEmotional labilitySeizureAtaxiaDysarthria

Classification & Codes

Orphanet Code

ORPHA:206443
late-infantile/juvenile Krabbe disease
OrphanetORPHA:206443
Treatments0 drug(s)
Symptoms on record46 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO