Pubblicazioni

Autori:

Manara, Renzo; Fragiacomo, Federica; Ladogana, Anna; Vaianella, Luana; Camporese, Giulia; Zorzi, Giovanni; Vicinanza, Sabrina; Zanusso, Gianluigi; Pocchiari, Maurizio; Cagnin, Annachiara

Titolo:

MRI abnormalities in Creutzfeldt-Jakob disease and other rapidly progressive dementia

Anno:

2024

Tipologia prodotto:

Articolo in Rivista

Tipologia ANVUR:

Articolo su rivista

Lingua:

Inglese

Formato:

A Stampa

Referee:

Sì

Nome rivista:

Journal of Neurology

ISSN Rivista:

0340-5354

N° Volume:

271

Numero o Fascicolo:

1

Intervallo pagine:

300-309

Parole chiave:

Creutzfeldt–Jakob disease; Dementia; Magnetic resonance imaging; Prion

Breve descrizione dei contenuti:

Objective: To investigate brain MRI abnormalities in a cohort of patients with rapidly progressive dementia (RPD) with and without a diagnosis of Creutzfeldt-Jakob disease (CJD). Methods: One hundred and seven patients with diagnosis of prion disease (60 with definite sCJD, 33 with probable sCJD and 14 with genetic prion disease) and 40 non-prion related RPD patients (npRPD) underwent brain MRI including DWI and FLAIR. MRIs were evaluated with a semiquantitative rating score, which separately considered abnormal signal extent and intensity in 22 brain regions. Clinical findings at onset, disease duration, cerebrospinal-fluid 14-3-3 and t-tau protein levels, and EEG data were recorded. Results: Among patients with definite/probable diagnosis of CJD or genetic prion disease, 2/107 had normal DWI-MRI: in one patient a 2-months follow-up DWI-MRI showed CJD-related changes while the other had autopsy-proven CJD despite no DWI abnormalities 282 days after clinical onset. CJD-related cortical changes were detected in all lobes and involvement of thalamus was common. In the npRPD groups, 6/40 patients showed DWI alterations that clustered in three different patterns: (1) minimal/doubtful signal alterations (limbic encephalitis, dementia with Lewy bodies); (2) clearly suggestive of alternative diagnoses (status epilepticus, Wernicke or metabolic encephalopathy); (3) highly suggestive of CJD (mitochondrial disease), though cortical swelling let exclude CJD. Conclusions: In the diagnostic work-up of RPD, negative/doubtful DWI makes CJD diagnosis rather unlikely, while specific DWI patterns help differentiating CJD from alternative diagnoses. The pulvinar sign is not exclusive of the variant form.

Pagina Web:

https://doi.org/10.1007/s00415-023-11962-1

Id prodotto:

136176

Handle IRIS:

11562/1113089

ultima modifica:

10 gennaio 2024

Citazione bibliografica:

Manara, Renzo; Fragiacomo, Federica; Ladogana, Anna; Vaianella, Luana; Camporese, Giulia; Zorzi, Giovanni; Vicinanza, Sabrina; Zanusso, Gianluigi; Pocchiari, Maurizio; Cagnin, Annachiara, MRI abnormalities in Creutzfeldt-Jakob disease and other rapidly progressive dementia «Journal of Neurology» , vol. 271 , n. 1 , 2024 , pp. 300-309

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