Tay-Sachs disease

MeSH: D013661ORPHA: 845

Overview

Human medical condition

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 Tay-Sachs disease, sourced from HPO and Orphanet clinical annotations.

Muscle weaknessProgressive spasticitySkeletal muscle atrophyGM2-ganglioside accumulationAbnormality of glycolipid metabolismAbnormal circulating enzyme concentration or activityVisual impairmentAtypical behaviorShort attention spanDysarthriaGait disturbanceHyperreflexiaDysphagiaGliosisPostural instabilityIncoordinationClumsinessFrequent fallsDevelopmental regressionFasciculationsDistal muscle weaknessMuscle spasmDifficulty climbing stairsPoor fine motor coordinationHypointensity of cerebral white matter on MRILower limb muscle weaknessQuadriceps muscle atrophyCherry red spot of the maculaAspiration pneumoniaAbnormal thalamic MRI signal intensityAbnormality of eye movementBlindnessOptic atrophyPsychosisDepressionAnxietySeizureCerebellar atrophyGeneralized hypotoniaDysmetriaDystoniaMyoclonusTremorAbsent speechLimited elbow extensionVentriculomegalyExaggerated startle responseGlobal brain atrophyDroolingMemory impairmentFocal impaired awareness seizureInability to walkLimited knee extensionFunctional motor deficitProgressive macrocephalyDistal upper limb muscle weaknessSpeech articulation difficultiesTypical absence seizureAnkle clonusGastrostomy tube feeding in infancyLaryngeal dystoniaHip flexor weaknessManiaHearing impairmentPrecocious pubertyIncreased serum beta-hexosaminidaseDecerebrate rigidityVegetative state

Classification & Codes

MeSH Code

D013661

Orphanet Code

ORPHA:845
Tay-Sachs disease
MeSHD013661
OrphanetORPHA:845
Treatments0 drug(s)
Symptoms on record68 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO