Two further cases of Creutzfeldt-Jakob disease (CJD) in association with cadaveric dura mater grafts are described. The clinical features of all such reported cases resemble more closely those of sporadic CJD, in contrast with kuru and the cases of CJD which have arisen after therapy with human pituitary-derived growth hormone. This observation may reflect the route of inoculation of the agent.

BACKGROUND There is evidence that homocysteine contributes to various neurodegenerative disorders. OBJECTIVE To assess the values of homocysteine in patients with Creutzfeldt-Jakob disease (CJD) in both cerebrospinal fluid (CSF) and plasma. METHODS STUDY DESIGN Case control study. Total homocysteine was quantified in CSF and plasma samples of CJD patients (n=13) and healthy controls (n=1...

Creutzfeldt-Jakob disease (CJD) exists in inherited, acquired (variant and iatrogenic), and spontaneous (sporadic) forms. Although iatrogenic and variant forms of CJD usually affect relatively young persons, all forms may affect elderly persons, especially sporadic CJD. Sporadic CJD is a rare cause of dementia among middle-aged and elderly persons, and typical cases are clinically fairly distin...

Creutzfeldt-Jakob disease (CJD) is the most frequent human Prion-related disorder (PrD). The detection of 14-3-3 protein in the cerebrospinal fluid (CSF) is used as a molecular diagnostic criterion for patients clinically compatible with CJD. However, there is a pressing need for the identification of new reliable disease biomarkers. The pathological mechanisms leading to accumulation of 14-3-3...

Small amounts of brain tissue (2 g) infected with Creutzfeldt-Jakob disease (CJD) can be fractionated by using a simple 1-day method that includes lysis with N-lauroylsarcosine. Unique fibrils have been identified previously in scrapie- and CJD-infected tissue. These fibrils were abundant in final fractions. Preparations from human CJD autopsy material and from experimental hamster and guinea p...

The population of neurons and the neuronal size in the nucleus basalis of Meynert (nbM) were studied in 20 patients with definite Creutzfeldt-Jakob disease (CJD). When compared with a normal control group, the 20 CJD brains showed a significant loss of neurons and reduction of neuronal size, mainly in the middle level of the nbM and mostly affecting the right side. Since these findings show som...

The Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) was established in October 1993 after the identification of probable iatrogenic CJD in recipients of human pituitary hormones. Since this time and with the recommendations of the Allars inquiry into CJD in Australia, the registry has performed surveillance of CJD in Australia with retrospective ascertainment to 1970 and ongoing...