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From: TSS (216-119-139-80.ipset19.wt.net)
Subject: Familial Prion Diseases in the Basque Country (Spain)
Date: October 11, 2004 at 1:46 pm PST

-------- Original Message --------
Subject: Familial Prion Diseases in the Basque Country (Spain)
Date: Mon, 11 Oct 2004 15:40:41 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@UNI-KARLSRUHE.DE


Familial Prion Diseases in the Basque Country (Spain)
Juan J. Zarranza, Anton Digonb, Begoña Atarésc, José M. Arteagoitiaf,
Nieves Carrerag, Iñaki Fernández-Mancholag, Manuel Fernández-Martíneza,
Covadonga Fernández-Maizteguia, Isabel Forcadasa, Luis Galdosd, Agustín
Ibáñezd, Elena Lezcanoa, José F. Martí-Massóg, María M. Mendibea, Miguel
Urtasung, Juan M. Utergah, Nieves Saracibare, Fernando Velascoa, Luis
González de Galdeanof

aService of Neurology, Hospital Cruces, Department of Neurosciences,
University of the Basque Country, Baracaldo,
bService of Neurology, Hospital Santiago Apóstol,
cService of Pathology, Hospital Txagorritxu,
dService of Neurology, Hospital Txagorritxu, Department of
Neurosciences, University of the Basque Country, and
eService of Pathology, Hospital Santiago, Vitoria,
fService of Epidemiology, Health Department, Basque Government, Lakua,
gService of Neurology, Hospital Donostia, Department of Neurosciences,
University of the Basque Country, San Sebastián, and
hService of Neurology, Hospital Basurto, Department of Neurosciences,
University of the Basque Country, Bilbao, Spain

Neuroepidemiology 2005;24:103-109 (DOI: 10.1159/000081057)

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Abstract

In 1995, a surveillance system for prion diseases was set up in the
Basque Country, an autonomous region in northern Spain (2.1 million
inhabitants). In the period from January 1993 to December 2003, we
diagnosed 21 patients with familial prion diseases prospectively and
another 4 patients retrospectively. They represent 35% of all the cases
referred to the epidemiological registry. Two main possible explanations
for this unusual high incidence of familial prion diseases are proposed:
first, comprehensive case ascertainment by public health neurologists;
second, a probable cluster of the D178N mutation within families of
Basque origin related to a still unconfirmed common ancestor. Further
genetic and genealogical studies should resolve this issue.

Copyright © 2005 S. Karger AG, Basel

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Author
Contacts

Prof. Juan J. Zarranz
Service of Neurology, Hospital de Cruces, Department of Neurosciences
University of the Basque Country
ES-48903 Baracaldo, Vizcaya (Spain)
Tel. +34 94 6006500, Fax +34 94 6006075, E-Mail
jjzarranz@hcru.osakidetza.net

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Article
Information

Published online: September 24, 2004
Number of Print Pages : 7
Number of Figures : 2, Number of Tables : 1, Number of References : 22

http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=NED20050241_2103

TSS





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