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[The first patient with the new variant of Creutzfeldt-Jakob's disease in The Netherlands].
Ned Tijdschr Geneeskd. 2005 Dec 31; 149(53):2949-54.NT

Abstract

A Dutch woman died at the age of 26 years, after a disease duration of 18 months, due to the new variant of Creutzfeldt-Jakob's disease (vCJD). She had never travelled to the United Kingdom and there was no history of potential iatrogenic exposure. However, she had worked in the catering and food production industry for the previous 6 years and had frequently consumed raw meat. The disease course showed the classical clinical picture of vCJD, which was confirmed by post-mortem examination of the brain. Contrary to classical sporadic CJD, patients with the variant disease are usually younger and present predominantly with psychiatric symptoms. Sensory complaints like pain and dysaesthesiae usually follow soon. Only later are these symptoms followed by rapidly progressive neurological symptoms and signs. All patients genotyped so far are homozygous for methionine on codon 129 of the prion protein gene. Recognition of the disease is of particular importance because of possible transmission via blood and tissues. In patients with rapidly progressive psychiatric symptoms and unexplained neurological signs, particularly sensory complaints, one must consider the possibility of vCJD.

Authors+Show Affiliations

Jansen C
Afd. Pathologie, Universitair Medisch Centrum Utrecht, Postbus 85.500, 3508 GA Utrecht. c.jansen@lab.azu.nl
Houben MP
No affiliation info available
Hoff JI
No affiliation info available
Sanchez-Juan P
No affiliation info available
Rozemuller AJ
No affiliation info available
van Duijn CM
No affiliation info available

MeSH

AdultBrainCreutzfeldt-Jakob SyndromeDiagnosis, DifferentialDisease ProgressionFatal OutcomeFemaleHumansNetherlands

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

dut

PubMed ID

16425845

Citation

Jansen, C, et al. "[The First Patient With the New Variant of Creutzfeldt-Jakob's Disease in the Netherlands]." Nederlands Tijdschrift Voor Geneeskunde, vol. 149, no. 53, 2005, pp. 2949-54.
Jansen C, Houben MP, Hoff JI, et al. [The first patient with the new variant of Creutzfeldt-Jakob's disease in The Netherlands]. Ned Tijdschr Geneeskd. 2005;149(53):2949-54.
Jansen, C., Houben, M. P., Hoff, J. I., Sanchez-Juan, P., Rozemuller, A. J., & van Duijn, C. M. (2005). [The first patient with the new variant of Creutzfeldt-Jakob's disease in The Netherlands]. Nederlands Tijdschrift Voor Geneeskunde, 149(53), 2949-54.
Jansen C, et al. [The First Patient With the New Variant of Creutzfeldt-Jakob's Disease in the Netherlands]. Ned Tijdschr Geneeskd. 2005 Dec 31;149(53):2949-54. PubMed PMID: 16425845.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [The first patient with the new variant of Creutzfeldt-Jakob's disease in The Netherlands]. AU - Jansen,C, AU - Houben,M P W A, AU - Hoff,J I, AU - Sanchez-Juan,P, AU - Rozemuller,A J M, AU - van Duijn,C M, PY - 2006/1/24/pubmed PY - 2006/2/14/medline PY - 2006/1/24/entrez SP - 2949 EP - 54 JF - Nederlands tijdschrift voor geneeskunde JO - Ned Tijdschr Geneeskd VL - 149 IS - 53 N2 - A Dutch woman died at the age of 26 years, after a disease duration of 18 months, due to the new variant of Creutzfeldt-Jakob's disease (vCJD). She had never travelled to the United Kingdom and there was no history of potential iatrogenic exposure. However, she had worked in the catering and food production industry for the previous 6 years and had frequently consumed raw meat. The disease course showed the classical clinical picture of vCJD, which was confirmed by post-mortem examination of the brain. Contrary to classical sporadic CJD, patients with the variant disease are usually younger and present predominantly with psychiatric symptoms. Sensory complaints like pain and dysaesthesiae usually follow soon. Only later are these symptoms followed by rapidly progressive neurological symptoms and signs. All patients genotyped so far are homozygous for methionine on codon 129 of the prion protein gene. Recognition of the disease is of particular importance because of possible transmission via blood and tissues. In patients with rapidly progressive psychiatric symptoms and unexplained neurological signs, particularly sensory complaints, one must consider the possibility of vCJD. SN - 0028-2162 UR - https://www.unboundmedicine.com/medline/citation/16425845/[The_first_patient_with_the_new_variant_of_Creutzfeldt_Jakob's_disease_in_The_Netherlands]_ DB - PRIME DP - Unbound Medicine ER -