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MRI lesion profiles in sporadic Creutzfeldt-Jakob disease.
- Source :
-
Neurology [Neurology] 2009 Jun 09; Vol. 72 (23), pp. 1994-2001. - Publication Year :
- 2009
-
Abstract
- Background: With respect to sporadic Creutzfeldt-Jakob disease (sCJD), six molecular subtypes (MM1, MM2, MV1, MV2, VV1, and VV2) have been described, which vary with respect to age at disease onset, disease duration, early symptoms, and neuropathology. MRI signal alterations were reported to correlate with distinct Creutzfeldt-Jakob disease (CJD) subtypes. This multicenter, international study aimed to describe the brain MRI findings associated with each of the sCJD molecular subtypes.<br />Methods: Pathologically confirmed sCJD cases with codon 129 genotype (MM, MV, and VV), PrP(Sc) type, and fluid-attenuated inversion recovery (FLAIR) or diffusion-weighted imaging (DWI) were collected in seven countries. All MRI scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus, and cerebellum.<br />Results: MRI scans were evaluated in 211 CJD patients (98 MM1, 23 MM2, 19 MV1, 30 MV2, 9 VV1, and 32 VV2). Basal ganglia hyperintensities occurred most frequently in MV2, VV2, and MM1 subtypes (79, 77, and 70%). Wide cerebral cortical signal increase was most common in VV1, MM2, and MV1 subtypes (86, 77, and 77%). Thalamic hyperintensities occurred most often in VV2 (45%) and MV2 (43%). The most consistent finding across most subtypes was high signal in basal ganglia, with these abnormalities found in 63% (FLAIR) and 71% (DWI).<br />Conclusion: Cortical signal increase and hyperintensities in the basal ganglia and thalamus are detected by MRI across all molecular sporadic Creutzfeldt-Jakob disease subtypes. Our findings argue that characteristic MRI lesion patterns may occur for each molecular subtype.
- Subjects :
- Basal Ganglia anatomy & histology
Basal Ganglia pathology
Basal Ganglia physiopathology
Brain anatomy & histology
Brain physiopathology
Cerebral Cortex anatomy & histology
Cerebral Cortex pathology
Cerebral Cortex physiopathology
Codon
Creutzfeldt-Jakob Syndrome classification
Creutzfeldt-Jakob Syndrome genetics
DNA Mutational Analysis
Diffusion Magnetic Resonance Imaging methods
Disease Progression
Genetic Testing
Genotype
Humans
Nerve Fibers, Myelinated pathology
Observer Variation
Odds Ratio
PrPSc Proteins genetics
Predictive Value of Tests
Sensitivity and Specificity
Thalamus anatomy & histology
Thalamus pathology
Thalamus physiopathology
Brain pathology
Creutzfeldt-Jakob Syndrome pathology
Magnetic Resonance Imaging methods
Subjects
Details
- Language :
- English
- ISSN :
- 1526-632X
- Volume :
- 72
- Issue :
- 23
- Database :
- MEDLINE
- Journal :
- Neurology
- Publication Type :
- Academic Journal
- Accession number :
- 19506221
- Full Text :
- https://doi.org/10.1212/WNL.0b013e3181a96e5d