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From: TSS (216-119-139-80.ipset19.wt.net)
Subject: Clinical and genetic features of human prion diseases in Catalonia: 1993-2002
Date: October 11, 2004 at 1:13 pm PST

-------- Original Message --------
Subject: Clinical and genetic features of human prion diseases in Catalonia: 1993-2002
Date: Mon, 11 Oct 2004 15:15:49 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@UNI-KARLSRUHE.DE


##################### Bovine Spongiform Encephalopathy #####################

Clinical and genetic features of human prion diseases in Catalonia:
1993-2002
R. Sanchez-Vallea,b
,
C. Nosc,d
,
J. Yagüeb,e
,
F. Grausa,b
,
A. Domínguezc

and A. Saiza,b

for the Catalan Collaborative Study Group for CJD*

We describe the clinical and genetic characteristics of the 85 definite
or probable human prion diseases cases died between January 1993 and
December 2002 in Catalonia (an autonomous community of Spain, 6 million
population). Seventy-three (86%) cases were sporadic Creutzfeld-Jakob
diseases (sCJD) (49 definite, 24 probable), with a median age at onset
of 66 years. The clinical presentation was dementia in 29 cases, ataxia
in 14 and visual symptoms in five. The median survival was 3 months. The
14-3-3 assay was positive in 93% cases, 62% presented periodic sharp
wave complexes (PSWC) in EEG but only 18% the typical signs on MRI.
Forty-eight sCJD were studied for codon 129 PRNP polymorphism: 69% were
methionine/methionine (M/M), 14.5% valine/valine (V/V) and 16.5% M/V.
Six out of seven V/V cases did not present PSWC and in two survival was
longer than 20 months. Eleven cases (13%) were genetic: five familial
fatal insomnia and six familial CJD (fCJD). Up to four (67%) fCJD lacked
family history of disease, two presented seizures early at onset and one
neurosensorial deafness. The only iatrogenic case was related to a dura
mater graft. No case of variant CJD was registered. The study confirms
in our population the consistent pattern reported worldwide on human
prion diseases. Atypical features were seen more frequently in sporadic
129 V/V CJD and fCJD cases.


http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=1351-5101&date=2004&volume=11&issue=10&spage=649TSS

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