Cerebrospinal fluid levels of amyloid β-protein precursor are low in gerstmann-sträussler-scheinker disease, Indiana kindred

We studied levels of amyloid β-protein precursor (β PP) in CSF from four patients affected with Gerstmann-Sträussler-Scheinker disease and one asymptomatic gene-carrier from an Indiana kindred. Levels of βPP were decreased in all affected patients with the lowest levels occurring in the clinically most severely affected patients. Correlation of neuropathology with CSF data in three patients suggested that low βPP in CSF may be associated with βPP accumulation in neurites surrounding cortical and cerebellar amyloid deposits.