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December 1996

Apolipoprotein E Phenotype Frequency and Cerebrospinal Fluid Concentration Are Not Associated With Creutzfeldt-Jakob Disease

Author Affiliations

From the Neurologische Klinik und Poliklinik (Drs Zerr, Poser, and Weber) and Abteilung Klinische Chemie (Drs Helmhold and Armstrong), Georg-August-Universität Göttingen, Göttingen, Germany; and Neurologische Klinik, Marienkrankenhaus Hamburg, Hamburg, Germany (Dr Weber).

Arch Neurol. 1996;53(12):1233-1238. doi:10.1001/archneur.1996.00550120041014

Objective:  To analyze the distribution of apolipoprotein E (Apo E) phenotypes between patients with Creutzfeldt-Jakob disease (CJD) and control subjects.

Setting:  University hospital, base of the German National CJD Surveillance Study.

Design:  Prospective case-control study.

Subjects:  Sixty-two patients with definite or probable CJD, 90 patients with initial suspected CJO, and 51 controls matched for age, sex, and place of residence.

Main Outcome Measures:  Phenotyping of Apo E in serum by isoelectric focusing, assessment of the gels by 3 independent investigators, measurement of of Apo E in cerebrospinal fluid using an enzyme-linked immunosorbent assay, and calculation of Kaplan-Meier cumulative survival plots.

Results:  The most frequent phenotype was E 3-3 with 56% in patients and 59% in controls, followed by E 3-4 with a frequency of 29% vs 25%, respectively. The phenotype E 3-2 was much rarer (13% vs 16%, respectively). Patients with definite CJD had a mean (SD) Apo E concentration of 3.4 (2.0) mg/L; patients with probable CJD, 3.1 (1.6) mg/L; patients with possible CJD, 3.8 (2.2) mg/L; and subjects with other diseases, 3.0 (1.7) mg/L. Mean (SD) disease duration for patients with E 3-2 was 11.8 (9.8) months; for patients with E 3-3,12.0 (9.02) months; and for patients with E 3-4,14.2 (12.3) months.

Conclusions:  We found no significant difference in the distribution of Apo E phenotypes between patients with CJD and controls. The concentration of Apo E in cerebrospinal fluid cannot be taken as a biochemical marker for the disease. The Apo E phenotype had no influence on the duration of CJD. Our data do not support an association of Apo E4 with either the duration or time at onset of CJD.

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