BACKGROUND AND PURPOSE: To establish radiological features in the atypical MV2 subtype of sCJD compared with the classical MM1 subtype, as well as region- and sequence-dependent inter-observer correlation. METHODS: MRI hyperintensity of basal ganglia (BG), cortex and thalamus was evaluated in 31 MM1 and 32 MV2 patients. Each MR scan was analyzed independently by two neuroradiologists blinded to PRNP genotype/prion protein type. RESULTS: Cumulative T2-sensitivity for BG hyperintensity was higher in the MV2 subtype (84% for both observers versus 61% in observer 1/42% in observer 2 in MM1 patients). Significant inter-observer agreement was found for BG and thalamus on T2, FLAIR, PD and DWI, but for cortex only on DWI. Thalamic changes were significantly more frequent in MV2 than in MM1 patients (cumulative sensitivity 86% vs. 12.5% on DWI). DISCUSSION: The high frequency of thalamic hyperintensity in the MV2 subtype allowed differentiation from MM1 patients. Good inter-observer agreement was found for BG and thalamus in all sequences. DWI showed the highest inter-observer correlation independent of the investigated brain region and was therefore not only highly sensitive but also relatively independent of investigator bias. Since inter-observer correlation for cortical hyperintensity in T2, FLAIR and PD is relatively low, the cortical changes should not be over-interpreted with these sequences.
BACKGROUND AND PURPOSE: To establish radiological features in the atypical MV2 subtype of sCJD compared with the classical MM1 subtype, as well as region- and sequence-dependent inter-observer correlation. METHODS: MRI hyperintensity of basal ganglia (BG), cortex and thalamus was evaluated in 31 MM1 and 32 MV2 patients. Each MR scan was analyzed independently by two neuroradiologists blinded to PRNP genotype/prion protein type. RESULTS: Cumulative T2-sensitivity for BG hyperintensity was higher in the MV2 subtype (84% for both observers versus 61% in observer 1/42% in observer 2 in MM1patients). Significant inter-observer agreement was found for BG and thalamus on T2, FLAIR, PD and DWI, but for cortex only on DWI. Thalamic changes were significantly more frequent in MV2 than in MM1patients (cumulative sensitivity 86% vs. 12.5% on DWI). DISCUSSION: The high frequency of thalamic hyperintensity in the MV2 subtype allowed differentiation from MM1patients. Good inter-observer agreement was found for BG and thalamus in all sequences. DWI showed the highest inter-observer correlation independent of the investigated brain region and was therefore not only highly sensitive but also relatively independent of investigator bias. Since inter-observer correlation for cortical hyperintensity in T2, FLAIR and PD is relatively low, the cortical changes should not be over-interpreted with these sequences.
Authors: Damien Galanaud; S Haik; M G Linguraru; J-P Ranjeva; B Faucheux; E Kaphan; N Ayache; J Chiras; P Cozzone; D Dormont; J-P Brandel Journal: AJNR Am J Neuroradiol Date: 2010-04-29 Impact factor: 3.825
Authors: Alberto Bizzi; Riccardo Pascuzzo; Janis Blevins; Marina Grisoli; Raffaele Lodi; Marco E M Moscatelli; Gianmarco Castelli; Mark L Cohen; Lawrence B Schonberger; Aaron Foutz; Jiri G Safar; Brian S Appleby; Pierluigi Gambetti Journal: JAMA Neurol Date: 2020-09-01 Impact factor: 18.302
Authors: Wen-Quan Zou; Gianfranco Puoti; Xiangzhu Xiao; Jue Yuan; Liuting Qing; Ignazio Cali; Miyuki Shimoji; Jan P M Langeveld; Rudy Castellani; Silvio Notari; Barbara Crain; Robert E Schmidt; Michael Geschwind; Stephen J Dearmond; Nigel J Cairns; Dennis Dickson; Lawrence Honig; Juan Maria Torres; James Mastrianni; Sabina Capellari; Giorgio Giaccone; Ermias D Belay; Lawrence B Schonberger; Mark Cohen; George Perry; Qingzhong Kong; Piero Parchi; Fabrizio Tagliavini; Pierluigi Gambetti Journal: Ann Neurol Date: 2010-08 Impact factor: 10.422
Authors: Alberto Bizzi; Riccardo Pascuzzo; Janis Blevins; Marco E M Moscatelli; Marina Grisoli; Raffaele Lodi; Fabio M Doniselli; Gianmarco Castelli; Mark L Cohen; Aymeric Stamm; Lawrence B Schonberger; Brian S Appleby; Pierluigi Gambetti Journal: Ann Neurol Date: 2020-12-22 Impact factor: 10.422
Authors: I Zerr; K Kallenberg; D M Summers; C Romero; A Taratuto; U Heinemann; M Breithaupt; D Varges; B Meissner; A Ladogana; M Schuur; S Haik; S J Collins; Gerard H Jansen; G B Stokin; J Pimentel; E Hewer; D Collie; P Smith; H Roberts; J P Brandel; C van Duijn; M Pocchiari; C Begue; P Cras; R G Will; P Sanchez-Juan Journal: Brain Date: 2009-09-22 Impact factor: 13.501