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MRI lesion profiles in sporadic Creutzfeldt–Jakob disease

AUTHORS' AFFILIATIONS
From the National TSE Reference Center (B.M., I.Z.), Department of Neurology, Georg-August University Goettingen, Germany; Department of Neuroradiology (K.K.), Georg-August University Goettingen, Germany; Fundación "Marqués de Valdecilla" IFIMAV and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (P.S.-J.), Santander, Spain; CJD Surveillance Unit (D.C., D.M.S., R.G.W.), Western General Hospital, Edinburgh, UK; Istituto Superiore di Sanità (S.A., V.M.), Department of Cell Biology and Neurosciences, Rome, Italy; Australian National Creutzfeldt-Jakob Disease Registry (S.J.C., H.R.), Department of Pathology, the University of Melbourne, Parkville, Australia; Mercy Private Radiology (P.S.), East Melbourne, Australia; Laboratory of Neurobiology (P.C., B.V.E.), Department of Neurology, Born Bunge Institute, University of Antwerp, Belgium; Creutzfeldt-Jakob Disease Surveillance System (G.H.J., M.B.C.), Prion Diseases Program, Public Health Agency of Canada, Ottawa, Canada; and Cellule Nationale de référence des maladies de Creutzfeldt-Jakob Groupe Hospitalier Pitié-Salpêtrière (J.P.B., D.G.), Paris, France.

Address correspondence and reprint requests to Dr. Inga Zerr, National TSE Reference Center, Department of Neurology, Georg-August University Goettingen, Robert-Koch-Str. 40, 37075 Goettingen, Germany epicjd{at}med.uni-goettingen.de

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.

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.

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).

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.

Abbreviations: AL = anterolateral; AUC = area under the receiver operating characteristic curve; BG = basal ganglia; CI = confidence interval; CJD = Creutzfeldt–Jakob disease; DWI = diffusion-weighted imaging; FLAIR = fluid-attenuated inversion recovery; MD = mediodorsal; OR = odds ratio; ROC = receiver operating characteristic; sCJD = sporadic Creutzfeldt–Jakob disease.

Supplemental data at www.neurology.org

Authors' affiliations are listed at the end of the article.

Supported by grants from the Federal Ministry of Education and Research (BMBF 01GI0301 and KZ: 0312720), the Federal Ministry of Health (BMG Az325-4471-02/15), the Robert Koch-Institute through funds of the Federal Ministry of Health (grant 1369-341), the Department of Health (121/7369), the European Union (TSELAB QLK2-CT-2002-81523), the Department of Health and the Scottish Executive Department of Health (The UK National CJD Surveillance Unit), the National Registry of CJD and Related Disorders of the Istituto Superiore di Sanità, Rome, Italy, the Commonwealth Department of Health and Ageing, the Fonds voor Wetenschappelijk Onderzoek, and the Born Bunge Institute.

Disclosure: The authors report no disclosures.

Received October 14, 2008. Accepted in final form March 12, 2009.

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