Living with Gerstmann-Straussler-Scheinker syndrome
A rare inherited prion disease that slowly damages the part of the brain that controls balance and coordination, with memory and thinking changes.
Understanding Gerstmann-Straussler-Scheinker syndrome
Prion diseases are a group of conditions that affect the nervous system. The main feature of GSS is progressive damage to the cerebellum, the part of the brain that controls coordination, balance, and muscle tone, along with varying degrees of dementia. It usually starts in adulthood.
Signs & symptoms
Symptoms vary from person to person. GARD lists these among the most common:
- Trouble with balance and coordinated walking (gait ataxia)
- Dementia and mental decline
- Difficulty speaking clearly (dysarthria)
- Weakness in the legs
- Pins and needles feelings (paresthesia)
- Sleep problems
Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.
Diagnosis
- 1Talk with your doctor
Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.
- 2See a specialist
Specialists who may be involved include brain and nervous system specialists and genetic specialists.
- 3Consider genetic testing
Changes in PRNP are linked to this condition. Genetic testing and counseling can help confirm a diagnosis and explain what it means for your family.
Care & treatment
- Care team
- Brain and nervous system specialists (neurologists); Genetic specialists (geneticists)
- Expert care
- Ask about multidisciplinary care centers for rare conditions
- Treatment
- GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
- Research
- Clinical trials and observational studies may be options. See the research section below.
General information, not medical advice. Your care team makes the decisions.
Everyday life
Living with a rare condition is easier with support.
- Connect with Creutzfeldt-Jakob Disease Foundation, Inc
- Keep notes on symptoms, changes and questions for appointments
- Ask about patient registries that help research
- Look into financial aid and travel resources for care visits
Quick facts
Changes in the PRNP gene cause this condition. It is inherited in an autosomal dominant way, which means one changed copy of the gene is enough.
Also known as: cerebellar ataxia, progressive dementia, and amyloid deposits in cns; cerebellar ataxia, progressive dementia, and amyloid deposits in the central nervous system; encephalopathy subacute spongiform gerstmann-straussler type; gerstmann-straussler disease; gerstmann-straussler-scheinker disease; gsd
Patient organizations
- Creutzfeldt-Jakob Disease Foundation, IncGerstmann-Straussler-Scheinker syndrome · United States
You are more than your diagnosis
Perspectives from people navigating life with Gerstmann-Straussler-Scheinker syndrome.
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