MRI lesion profiles in sporadic Creutzfeldt-Jakob disease

被引：129

作者：

Meissner, B. ^{[1
]}

Kallenberg, K. ^{[2
]}

Sanchez-Juan, P. ^{[3
,4
]}

Collie, D. ^{[5
]}

Summers, D. M. ^{[5
]}

Almonti, S. ^{[6
]}

Collins, S. J. ^{[7
]}

Smith, P. ^{[8
]}

Cras, P. ^{[9
]}

Jansen, G. H. ^{[10
]}

Brandel, J. P.

Coulthart, M. B. ^{[10
]}

Roberts, H. ^{[7
]}

Van Everbroeck, B. ^{[9
]}

Galanaud, D.

Mellina, V. ^{[6
]}

Will, R. G. ^{[5
]}

Zerr, I. ^{[1
]}

机构：

[1] Univ Gottingen, Dept Neurol, Natl TSE Reference Ctr, D-37075 Gottingen, Germany

[2] Univ Gottingen, Dept Neuroradiol, D-37075 Gottingen, Germany

[3] Fdn Marques de Valdecilla IFIMAV, Santander, Spain

[4] Ctr Invest Biomed Red Enfermedades Neurodegenerat, Santander, Spain

[5] Western Gen Hosp, CJD Surveillance Unit, Edinburgh EH4 2XU, Midlothian, Scotland

[6] Ist Super Sanita, Dept Cell Biol & Neurosci, I-00161 Rome, Italy

[7] Univ Melbourne, Dept Pathol, Australian Natl Creutzfeldt Jakob Dis Registry, Parkville, Vic 3052, Australia

[8] Mercy Private Radiol, Melbourne, Vic, Australia

[9] Univ Antwerp, Born Bunge Inst, Dept Neurol, Neurobiol Lab, Antwerp, Belgium

[10] Publ Hlth Agcy Canada, Prion Dis Program, Creutzfeldt Jakob Dis Surveillance Syst, Ottawa, ON, Canada

来源：

NEUROLOGY | 2009年 / 72卷 / 23期

关键词：

CLINICAL-DIAGNOSIS; TESTS; SENSITIVITY; FEATURES; SUBTYPE; PATTERN; CJD;

D O I：

10.1212/WNL.0b013e3181a96e5d

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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), PrPSc 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. Neurology (R) 2009; 72: 1994-2001

引用

页码：1994 / 2001

页数：8