Rapidly progressive Creutzfeldt-Jakob disease in patients with Familial Mediterranean Fever.
BACKGROUND
The largest cluster of familial Creutzfeldt-Jakob disease (fCJD) exists in Jews of Libyan origin. Familial Mediterranean fever (FMF) is an inflammatory disease also common in this population.


OBJECTIVE
We hypothesized that FMF, as a pro-inflammatory condition, may affect the course of CJD.


METHODS
Three hundred and seventy-two consecutive patients diagnosed clinically and genetically as CJD were included in the study. Two hundred and thirty-six had fCJD, and 136 had sporadic disease (sCJD). Review of the patient's records revealed three patients with FMF-CJD co-morbidity. In addition, 50 DNA samples of patients with CJD were genotyped as homozygote, heterozygote, and non-carriers of the FMF mutation. The demographic and clinical variables of the groups were compared.


RESULTS
The three patients with FMF had an earlier age of onset and significantly shorter disease duration than the patients without FMF. Heterozygote carriers did not differ in disease onset and duration from patients without FMF.


CONCLUSIONS
The shorter disease duration of CJD patients with FMF may indicate the importance of pro-inflammatory factors in the disease.
Appel
S A
SA
Department of Neurology, The Sagol Neuroscience Center at the Chaim Sheba Medical Center affiliated to the Sackler Faculty of Medicine, Tel-Aviv University, Tel-Hashomer, Israel.
Chapman
J
J
Kahana
E
E
Rosenmann
H
H
Prohovnik
I
I
Pras
E
E
Reznik-Wolf
H
H
Cohen
O S
OS
D000328:Adult; D017668:Age of Onset; D015897:Comorbidity; D007562:Creutzfeldt-Jakob Syndrome / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology; D003598:Cytoskeletal Proteins / Q000235:genetics; D018450:Disease Progression; D010505:Familial Mediterranean Fever / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology; D005260:Female; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D007585:Jews / Q000235:genetics; D008002:Libya; D008297:Male; D008875:Middle Aged; D009154:Mutation; D016133:Polymerase Chain Reaction; D000071198:Pyrin
D003598:Cytoskeletal Proteins; C000604192:MEFV protein, human; D000071198:Pyrin