Alexander disease type I

ORPHA: 363717

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 Alexander disease type I, sourced from HPO and Orphanet clinical annotations.

Abnormal cerebral white matter morphologyFocal T2 hyperintense basal ganglia lesionSeizureCerebellar atrophyFailure to thriveScoliosisProgressive macrocephalyAbnormal pyramidal signAbnormal thalamic MRI signal intensityRosenthal fibersHydrocephalusAtaxiaSpasticityDysarthriaGlobal developmental delayHyperreflexiaDysphagiaCachexiaHypotoniaGeneralized hypotoniaVomitingPalatal tremor

Classification & Codes

Orphanet Code

ORPHA:363717
Alexander disease type I
OrphanetORPHA:363717
Treatments0 drug(s)
Symptoms on record22 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO