Asymmetric cortical high signal on diffusion weighted-MRI in a case of Creutzfeldt-Jakob disease

Arquivos de Neuro-Psiquiatria

Ricardo Nitrini¹  Renata Areza-fegyveres¹  Vilma R. Martins²  Rosa Maria R.p.s. Castro²  Michele C. Landemberger²  Nancy Huang¹  Luiz A. Bacheschi¹  Luiz E. Bacheschi¹  Cláudia C. Leite¹  Carlos A. Buchpiguel¹  Sérgio Rosemberg¹ 

[1] ,University of São Paulo School of Medicine Hospital das ClínicasSão Paulo SP ,Brazil

关键词: Creutzfeldt-Jakob disease;    prion disease;    MRI;    diffusion-weighted MRI;    doença de Creutzfeldt-Jakob;    prion;    ressonância magnética;    difusão;   

DOI  :  10.1590/S0004-282X2005000300028

来源: SciELO

【 摘 要 】

High signal in the cerebral cortex and/or basal ganglia on diffusion-weighted magnetic resonance imaging (DW-MRI) has been described as a good diagnostic marker for sporadic Creutzfeldt-Jakob disease (sCJD). We report a case of sCJD with atypical clinical evolution and unusual DW-MRI findings. A 53-year-old man was seen with a 2-year history of a rapidly progressive dementia and cerebellar ataxia. Cerebrospinal fluid analysis, including the test for 14-3-3 protein, was normal. EEG did not show periodic activity. However, DW-MRI showed gyriform hyperintensity involving practically the entire cortical ribbon of the left hemisphere, whilst being limited to the posterior cingulate gyrus in the right hemisphere. DNA analysis showed no mutations or insertions in the prion protein gene, and homozigozity for methionine in codon 129. A subsequent brain biopsy confirmed the diagnosis of CJD. Thus, high signal on DW-MRI may be limited to the cerebral cortex and may present a very asymmetric distribution in sCJD.

【 授权许可】

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