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Molekulare Charakterisierung der Prionerkrankungen und Bildgebung

Förderkennzeichen: ARG 07/008

Projektlaufzeit: 01.03.08 - 28.02.10

With respect to sporadic CJD (CJD), six molecular subtypes (MM1, MM2, MV1, MV2, VV1 and VV2) have been described. These subtypes vary concerning age of disease onset, disease duration, early symptoms, diagnostic findings (EEG and CSF) and neuropathological lesion profiles. It has been proposed that MRI signal alterations in cortical and subcortical structures vary in distinct CJD subtypes, potentally representing different strain characteristics. MRI lesion pattern might help to determine the molecular CJD type in sporadic CJD. Genetic cases of prion dseases were not analyzed with this respect and no data on this subject are available. The high incidence of patients with E200K mutation in Argentina opens a unique opportunity to study these cases with modern brain imaging techniques. Genetic and sporadic CJD cases from Argentina and Germany will be analyzed for their MRI characteristics and clinical and pathological features. A database will be created and will include infromation on clinical data, brain imaging analysis (seven cortical and three subcortical regions), histopathology (PrP deposition, neuronal loss, astrogliosis, spongiform changes), PRNP analysis and Western blotting

Projektpartner Deutschland:

Nationales TSE Referenzzentrum
Prof. Dr.
Inga
Zerr

Neurologische Klinik
Robert-Koch-Str. 40
37075 Göttingen

Tel:
0551-396636
Fax:
0551-397020
E-Mail:

Ausländischer Projektpartner:

Referal center for CJD and other TSE
Prof. Dr.
Ana Lia
Taratuto

Dept. of Neuropathol
Montaneses 2325
C1428 AQK Buenos Aires

Tel:
+54-11-5777-3200
Fax:
+54-11-5777-3209
E-Mail:

Redaktion: 02.09.10

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