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Gerstmann-Straussler-Scheinker syndrome

MeSH: D016098ORPHA: 356

Contents

  1. 1. Overview
  2. 2. Available Treatments
  3. 3. Clinical Presentation
  4. 4. Classification & Codes

Overview

prion disease characterized by adult onset of memory loss, dementia, ataxia, and pathologic deposition of amyloid-like plaques in the brain

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 Gerstmann-Straussler-Scheinker syndrome, sourced from HPO and Orphanet clinical annotations.

Gait ataxiaLower limb muscle weaknessDysesthesiaCognitive impairmentDementiaDysarthriaMental deteriorationAreflexiaReduced tendon reflexesAbnormal cerebellum morphologyAbnormal pyramidal tract morphologyAbnormality of extrapyramidal motor functionSleep disturbanceParesthesiaAbnormal central sensory functionAcroparesthesiaBrain imaging abnormalityLimb myoclonus

Classification & Codes

MeSH Code

D016098

Orphanet Code

ORPHA:356
Gerstmann-Straussler-Scheinker syndrome
MeSHD016098
OrphanetORPHA:356
Treatments0 drug(s)
Symptoms on record18 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO