TY - JOUR
T1 - Neuroradiology of human prion diseases, diagnosis and differential diagnosis.
AU - Gaudino, Simona
AU - Gangemi, Emma
AU - Colantonio, Raffaella
AU - Botto, Annibale
AU - Ruberto, Emanuela
AU - Calandrelli, Rosalinda
AU - Martucci, Matia
AU - Vita, Maria Gabriella
AU - Masullo, Carlo
AU - Colosimo, Cesare
AU - Cerase, Alfonso
PY - 2017
Y1 - 2017
N2 - Human transmissible spongiform encephalopathies (TSEs), or prion diseases, are invariably fatal conditions associated with a range of clinical presentations. TSEs are classified as sporadic [e.g. sporadic Creutzfeldt-Jakob disease (sCJD), which is the most frequent form], genetic (e.g. Gerstmann-Straussler-Scheinker disease, fatal familial insomnia, and inherited CJD), and acquired or infectious (e.g. Kuru, iatrogenic CJD, and variant CJD). In the past, brain imaging played a supporting role in the diagnosis of TSEs, whereas nowadays magnetic resonance imaging (MRI) plays such a prominent role that MRI findings have been included in the diagnostic criteria for sCJD. Currently, MRI is required for all patients with a clinical suspicion of TSEs. Thus, MRI semeiotics of TSEs should become part of the cultural baggage of any radiologist. The purposes of this update on the neuroradiology of CJD are to (i) review the pathophysiology and clinical presentation of TSEs, (ii) describe both typical and atypical MRI findings of CJD, and (iii) illustrate diseases mimicking CJD, underlining the MRI key findings useful in the differential diagnosis.
AB - Human transmissible spongiform encephalopathies (TSEs), or prion diseases, are invariably fatal conditions associated with a range of clinical presentations. TSEs are classified as sporadic [e.g. sporadic Creutzfeldt-Jakob disease (sCJD), which is the most frequent form], genetic (e.g. Gerstmann-Straussler-Scheinker disease, fatal familial insomnia, and inherited CJD), and acquired or infectious (e.g. Kuru, iatrogenic CJD, and variant CJD). In the past, brain imaging played a supporting role in the diagnosis of TSEs, whereas nowadays magnetic resonance imaging (MRI) plays such a prominent role that MRI findings have been included in the diagnostic criteria for sCJD. Currently, MRI is required for all patients with a clinical suspicion of TSEs. Thus, MRI semeiotics of TSEs should become part of the cultural baggage of any radiologist. The purposes of this update on the neuroradiology of CJD are to (i) review the pathophysiology and clinical presentation of TSEs, (ii) describe both typical and atypical MRI findings of CJD, and (iii) illustrate diseases mimicking CJD, underlining the MRI key findings useful in the differential diagnosis.
KW - Creutzfeldt–Jakob disease
KW - Differential diagnosis
KW - Diffusion-weighted imaging
KW - Neurodegenerative diseases
KW - Creutzfeldt–Jakob disease
KW - Differential diagnosis
KW - Diffusion-weighted imaging
KW - Neurodegenerative diseases
UR - http://hdl.handle.net/10807/93412
U2 - 10.1007/s11547-017-0725-y
DO - 10.1007/s11547-017-0725-y
M3 - Article
SP - N/A-N/A
JO - Radiologia Medica
JF - Radiologia Medica
SN - 0033-8362
ER -