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

Patients with suspected Creutzfeldt–Jakob disease (CJD) often have routine cerebrospinal fluid (CSF) analysis performed to exclude treatable inflammatory conditions; however, little information is available about the range of results obtained for CSF tests in patients with sporadic CJD and other transmissible spongiform encephalopathies (TSE). Data from 450 patients with sporadic CJD and 47 patients with other TSEs were collected as part of an EC‐supported multinational study. Raised white cell counts of >5 cells/μl were found in three of 298 patients with sporadic CJD, with two cell counts of 7 cells/μl and one of 20 cells/μl. Total protein concentrations of >0.9 g/l were found in five of 438 patients with sporadic CJD, although none had a concentration of >1 g/l. CSF oligoclonal IgG was detected in eight of 182 sporadic CJD patients. Of the patients with other TSEs, six had elevated cell counts ranging from 6 to 14 cells/μl but none had total protein concentrations of >0.9 g/l and one patient had detectable oligoclonal IgG. None of the patients with sporadic CJD or other TSEs had abnormalities in all three tests.

[1]  A. Giovagnoli,et al.  Unusual findings in Creutzfeldt-Jakob disease. Case report , 1988, The Italian Journal of Neurological Sciences.

[2]  H. Kretzschmar,et al.  Immunoglobulins and virus‐specific antibodies in patients with Creutzfeldt–Jakob disease * , 2005, Acta neurologica Scandinavica.

[3]  A. Godbolt,et al.  The prevalence of oligoclonal bands in the CSF of patients with primary neurodegenerative dementia , 2004, Journal of Neurology.

[4]  H. Reiber,et al.  Dynamics of brain-derived proteins in cerebrospinal fluid. , 2001, Clinica chimica acta; international journal of clinical chemistry.

[5]  B. Steinhoff,et al.  How to improve the clinical diagnosis of Creutzfeldt-Jakob disease. , 1999, Brain : a journal of neurology.

[6]  O. Gefeller,et al.  Detection of 14‐3‐3 protein in the cerebrospinal fluid supports the diagnosis of Creutzfeldt‐Jakob disease , 1998, Annals of neurology.

[7]  C. Gibbs,et al.  The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies. , 1996, The New England journal of medicine.

[8]  J. Bell,et al.  A new variant of Creutzfeldt-Jakob disease: neuropathological and clinical features. , 1996, Cold Spring Harbor symposia on quantitative biology.

[9]  K. Jellinger,et al.  Neuropathological Diagnostic Criteria for Creutzfeldt‐Jakob Disease (CJD) and Other Human Spongiform Encephalopathies (Prion Diseases) , 1995, Brain pathology.

[10]  P. Brown,et al.  Human spongiform encephalopathy: The national institutes of health series of 300 cases of experimentally transmitted disease , 1994, Annals of neurology.

[11]  R. Hackler,et al.  Age-related alterations of the blood-brain-barrier (bbb) permeability to protein molecules of different size. , 1993, Zeitschrift fur Gerontologie.

[12]  R. Nau,et al.  Inverse correlation between disappearance of intrathecally injected 111In-DTPA from CSF with CSF protein content and CSF-to-serum albumin ratio , 1993, Journal of the Neurological Sciences.

[13]  K. Blennow,et al.  Protein Analysis in Cerebrospinal Fluid , 1993 .

[14]  K. Blennow,et al.  Protein analysis in cerebrospinal fluid. II. Reference values derived from healthy individuals 18-88 years of age. , 1993, European neurology.

[15]  J. Lott,et al.  Estimation of reference intervals for total protein in cerebrospinal fluid. , 1989, Clinical chemistry.

[16]  E. Hedley‐Whyte,et al.  Ataxic Creutzfeldt‐Jakob disease , 1985, Neurology.

[17]  J. Conly,et al.  Cerebrospinal fluid as a diagnostic body fluid. , 1983, The American journal of medicine.

[18]  J. Olsson.  Brain and CSF proteins in Creutzfeldt-Jakob disease. , 1980, European neurology.

[19]  M. Monari,et al.  Cerebrospinal fluid and serum immunoglobulins and C3 in Creutzfeldt‐Jakob disease , 1979, Neurology.