| Autor: |
Cannizzaro, Miryam, Pieri, Valentina, Curti, Davide Gusmeo, Gueye, Mor, Rugarli, Giulia, Napoli, Giovanni, Cardamone, Rosalinda, Falini, Andrea, Perani, Daniela, Zucconi, Marco, Casoni, Francesca, Giaccone, Giorgio, Moda, Fabio, Di Fede, Giuseppe, Magnani, Giuseppe, Caso, Francesca, Filippi, Massimo |
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| Zdroj: |
Journal of Neurology; Jun2023, Vol. 270 Issue 6, p3246-3251, 6p |
| Abstrakt: |
Miryam Cannizzaro1 and Valentina Pieri1 contributed equally to this work Introduction Prion diseases constitute a group of rapid and fatal neurodegenerative disorders caused by the conversion and accumulation of the normal prion protein (PrP SP C sp ), which is rich in -helical structures, into an abnormal, misfolded form (PrP SP Sc sp ), which is enriched in -sheet structures. Indeed, in our patient, 1-month follow-up brain MRI evidenced cortical and basal ganglia DWI-hyperintensity, suggesting the hypothesis of a prion disease, which was already suggested by the 18FDG-PET and then supported by the positive CSF RT-QuIC, despite its sensitivity in sFI being reported around 60% [[3]]. The presence of negative brain MRI in rapidly evolving dementias such as prion diseases is uncommon, but it can occur in the early phases of the disease [[12]], so short-term follow-up is suggested. Because of sFI rarity and its overlapping symptoms with other neurogenerative diseases, this disease often represents a diagnostic challenge for clinicians, leading to delayed or post-mortem diagnosis [[3]]. [Extracted from the article] |
| Databáze: |
Complementary Index |
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