tremor-ataxia-central hypomyelination syndrome

ORPHA: 447896

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 tremor-ataxia-central hypomyelination syndrome, sourced from HPO and Orphanet clinical annotations.

Hypogonadotropic hypogonadismVertical supranuclear gaze palsyMyopiaAbnormality of ocular smooth pursuitNystagmusHypodontiaOligodontiaDelayed eruption of teethDelayed pubertyAtaxiaMild intellectual disabilitySpasticityGlobal developmental delayDysmetriaCerebellar hypoplasiaDystoniaHyperreflexiaDysphagiaHypoplasia of the corpus callosumIntention tremorAbnormal basal ganglia morphologyPostural tremorClumsinessDevelopmental regressionPositive Romberg signLeukodystrophySpastic dysarthriaUpper motor neuron dysfunctionCNS hypomyelinationBabinski signShort statureAutonomic bladder dysfunctionHigh myoinositol in brain by MRSOptic atrophyCerebral cortical atrophyImpaired vibration sensation in the lower limbsDroolingImpaired distal proprioceptionPeripheral neuropathyDeeply set eyeFocal-onset seizure

Classification & Codes

Orphanet Code

ORPHA:447896
tremor-ataxia-central hypomyelination syndrome
OrphanetORPHA:447896
Treatments0 drug(s)
Symptoms on record41 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO