Search

Objectives: The detection of the protein 14-3-3 in the CSF has been shown to be a reliable and sensitive marker for sporadic Creutzfeldt-Jakob disease (CJD). Other brain-specific proteins such as neuron specific enolase (NSE), S-100b, and tau protein have also been reported to be increased in the CSF of patients with sporadic CJD. In 1996 a variant of CJD (vCJD) was described which is likely to be causally linked to the bovine spongiform encephalopathy agent. This study reports and compares the findings of CSF brain specific protein analysis in 45 patients with vCJD and in 34 control patients.Methods: The CSF from 45 patients with vCJD and 34 controls were investigated for the presence of 14-3-3 by SDS-polyacrylamide gel electrophoresis (SDS-PAGE) and western blotting with chemiluminescent detection. Tau protein, S-100b, and NSE concentrations in CSF were measured using enzyme immunoassays.Results: Protein 14-3-3 was detected in the CSF of 22/45 patients with vCJD and in 3/34 controls. The mean concentrations of NSE, S-100b, and tau protein in CSF were significantly raised in patients with vCJD compared with controls. The positive predictive value of CSF 14-3-3 was 86% and the negative predictive value was 63%. These values are lower than those reported for sporadic CJD. An increased CSF tau had a positive predictive value of 93% and a negative predictive value of 81%. The combination of CSF 14-3-3 and/or increased CSF tau had a positive predictive value of 91% and a negative predictive value of 84%.Conclusions: CSF protein 14-3-3 is not as useful a marker for vCJD as it is for sporadic CJD. Increased concentration of CSF tau was found to be a sensitive marker of vCJD but as concentrations may be increased in many forms of non-CJD dementia, this may limit its usefulness as a diagnostic test. [ABSTRACT FROM AUTHOR]

An attempt was made to ascertain all cases of Creutzfeldt-Jakob disease occurring in England and Wales during the 5 year period 1980-1984. The mean annual mortality rate was 0.49/million; women were more frequently affected than men. The age-specific mortality rate reached a peak in the seventh decade. A case-control study involving 92 of the 122 definite and probable cases ascertained failed to confirm the reality of previously suspected aetiological agents in the environment. Although there was no confirmed instance of familial Creutzfeldt-Jakob disease in the case-control study, dementia in close relatives was significantly more common than in controls. [ABSTRACT FROM AUTHOR]

A 62 year old woman presented with subacute dementia and hemiparesis which progressed to death with terminal seizures over 5 months. A cerebral biopsy specimen and necropsy showed the features of neoplastic angioendotheliosis which proved to be lymphomatous. Cerebral CT showed multiple low density areas in the white matter with striking enhancement after high-dose intravenous contrast administration. There was temporary clinical improvement with prednisolone therapy. The clinical features, cerebral CT appearances and partial response to treatment are discussed, with a review of the relevant literature. [ABSTRACT FROM AUTHOR]

Five patients with serious ocular complications of benign intracranial hypertension are described. Optic nerve sheath fenestration resulted in resolution of papilloedema with, in three instances, improvement in vision. Benign intracranial hypertension may not always be benign for vision and fenestration operations may prevent or reverse visual deterioration by an effect on the optic nerve rather than by reducing intra-cranial pressure. [ABSTRACT FROM AUTHOR]

Between 1970 and 2003, seven cases of human dura mater-associated Creutzfeldt-Jakob disease (CJD) were identified in the UK. Furthermore, we identified a case of CJD in a porcine dura graft recipient. The mean incubation period of the human dura mater cases was 93 (range 45–177) months. The clinico-pathological features of the cases are described and compared with cases previously reported in the world literature. [ABSTRACT FROM AUTHOR]

Objective: To investigate whether phosphorylated tau protein (tau-pT181) is increased in variant Creutzfeldt-Jakob disease (vCJD) and if the tau-pT181/tau protein ratio is useful for distinguishing between patients with and without CD. Methods: CSF tau protein and tau-pT181 were measured in 50 patients with sporadic CD (sCJD), 51 patients with vCJD, 46 sCJD controls, and 37 vCJD controls, using Innotest hTau and Innotest P-Thr181, Innogenetics. Results: Concentrations of CSF tau protein were increased in sCJD (5120 v 367 pg/ml in controls, p<0.001) and vCJD (952 v 106 pg/ml, p<0.001); tau-pT181 was also raised in sCJD (61 v 35 pg/ml in controls, p=0.002) and vCJD(114 v 33 pg/ml, p<0.001). Median concentrations of tau-pT181 were higher in vCJD than in sCJD (p<0.001). The tau- pT181/tau protein ratio was lower than in controls in both sCJD (12 v 128 (p<0.001)) and vCJD (119 v 279 (p<0.001)). Mean tau-pT181/tau protein ratio was 10-fold higher in vCJD than in sCJD. Raised CSF tau protein had the highest efficiency for distinguishing sCJD and vCJD from controls. Conclusions: CSF tau-pT181 concentrations are raised in vCJD and are higher than in sCJD. Measurement of CSF tau-pT181 /tau protein ratio does not improve the diagnostic efficiency of CSF tau protein alone for either vCJD or sCJD. The higher concentration of CSF tau-pT181 found in vCJD suggests that unexplained pathogenic factors influence the phosphorylation of tau protein in vCJD patients. [ABSTRACT FROM AUTHOR]