Gerstmann-Straussler-Scheinker Disease

Overview

An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA, spastic paraparesis, extrapyramidal signs, and DEMENTIA. Clinical onset is in the third to sixth decade of life and the mean duration of illness prior to death is five years. Several kindreds with variable clinical and pathologic features have been described. Pathologic features include cerebral prion protein amyloidosis, and spongiform or neurofibrillary degeneration. (From Brain Pathol 1998 Jul;8(3):499-513; Brain Pathol 1995 Jan;5(1):61-75)

This information is general and should not be treated as medical advice. Always speak directly with a qualified healthcare professional before making decisions about treatment, diagnosis, procedures, medicines or care.

Quick facts about Gerstmann-Straussler-Scheinker Disease

Topic type

Condition

Category

Neurology

MeSH classification

C01.207.800.350|C10.228.228.800.350|C10.574.500.425|C10.574.843.400|C16.320.400.350

Preparing for an appointment about Gerstmann-Straussler-Scheinker Disease

If you are speaking with a healthcare professional about Gerstmann-Straussler-Scheinker Disease, it can help to prepare a few details in advance.

• Write down your symptoms and when they started.
• Bring details of any medicines, supplements or previous treatments.
• Ask what tests or assessments may be needed.
• Ask what treatment or management options may be suitable.
• Ask when you should seek urgent medical help or follow-up care.

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