Unusual findings in Creutzfeldt-Jakob disease. Case report

Abstract

We present a definite case of Creutzfeldt-Jakob disease (CJD) with visual evoked response (VER) and CSF oligoclonal band abnormalities and discuss the neurobiological significance and diagnostic value of these findings.

Immunoglobulins and virus-specific antibodies in patients with Creutzfeldt-Jakob disease*

“…The reports were mainly based on the clinical diagnosis of Ôprobable CJDÕ (15)(16)(17)(18)(19)(20)(21)(22), while, to the best of our knowledge, this study is the first comprehensive analysis of a large number of neuropathologically confirmed sporadic CJD. The reports were mainly based on the clinical diagnosis of Ôprobable CJDÕ (15)(16)(17)(18)(19)(20)(21)(22), while, to the best of our knowledge, this study is the first comprehensive analysis of a large number of neuropathologically confirmed sporadic CJD.…”

“…The results of standard CSF investigations such as cell count, blood-CSF barrier function, immunoglobulins have been reported earlier in the literature in small case series and not well-defined groups. The reports were mainly based on the clinical diagnosis of Ôprobable CJDÕ (15)(16)(17)(18)(19)(20)(21)(22), while, to the best of our knowledge, this study is the first comprehensive analysis of a large number of neuropathologically confirmed sporadic CJD. Moreover, no observations have been described on frequency of intrathecal synthesis of antibodies against neurotropic viruses before.…”

“…It is widely accepted that in CJD there are no major pathological findings in CSF routine investigations, but to our knowledge there has never been a study about this issue except for reports with small numbers of cases or case reports (15)(16)(17)(18)(19)(20)(21)(22). To date no data is available on intrathecally synthesized antibodies against neurotropic viruses like varicella zoster (VZV) and herpes simplex (HSV) in CSF of patients with CJD.…”

CSF analysis in patients with sporadic CJD and other transmissible spongiform encephalopathies

“…Standard CSF examination has an important utility in identifying these conditions in patients with suspected transmissible spongiform encephalopathies (TSEs). There have been a number of studies showing that there are no substantial inflammatory changes within the CSF of patients with CJD, but most of these reports have only included small numbers of cases, single case reports or brief descriptions of results [4–9]. In this study, we describe the results of the white cell count, total protein and oligoclonal IgG found in neuropathologically confirmed cases of sporadic, genetic and variant CJD.…”