The prion protein (PrP) plays an essential role in the pathogenesis of a group of sporadic,
genetically determined and infectious fatal degenerative diseases, referred to as “prion …

Gerstmann‐Sträussler‐Scheinker disease is an autosomal dominant disorder with a wide
spectrum of clinical presentations including ataxia, spastic paraparesis, extrapyramidal signs, …

… DNA was extracted with phenol/chloroform solutions and the aqueous phase was
precipitated in a solution of O.5 M NaCl/isopropanol (1:10, v/v) overnight at -70C. DNA was …

Neuropathological diagnostic criteria for Creutzfeldt‐Jakob disease (CJD) and other human
transmissible spongiform encephalopathies (prion diseases) are proposed for the following …

… (oil red O); Comparison of the lipid staining patern of the white matter between the frontal …
(oil red O): Degeneration of the frontopontine tract in the basal portion of midbrain. O: lncreased …

It has been recognized that molecular classifications will form the basis for neuropathological
diagnostic work in the future. Consequently, in order to reach a diagnosis of Alzheimer's …

Patients affected with Gerstmann-Sträussler-Scheinker disease from two families, one from
Indiana and one of Swedish origin, have been studied. The patients are clinically …

When 22 members of the BrainNet Europe (BNE) consortium assessed 31 cases with α-synuclein
(αS) immunoreactive (IR) pathology applying the consensus protocol described by …

Deposition of PrP amyloid in cerebral vessels in conjunction with neurofibrillary lesions is the
neuropathologic hallmark of the dementia associated with a stop mutation at codon 145 of …

In 1986, we reported two anatomoclinical observations of a familial condition that we called “fatal
familial insomnia” (FFI). We now present the pedigree as well as the clinical and …