Isolated Cortical Involvement on MR Imaging in Sporadic Creutzfeldt-Jakob Disease: A Case Report

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare dementing disease and is thought to caused by a prion. It is characterized by rapidly progressive dementia, ataxia, myoclonus, akinetic mutism and eventual death. The detection of 14-3-3 protein in the cerebrospinal fluid (CSF) may support the diagnosis of the CJD. Periodic synchronized sharp wave complexes are usually seen in electroencephalogram (EEG) during middle or late stages of disease. Diffusion-weighted imaging (DWI) is the most sensitive magnetic rezonans sequence technique in the diagnosis of CJD. Brain biopsy or autopsy is required for a definitive diagnosis of CJD. We present 60-year-old man diagnosed as sporadic CJD with isolated cortical involvement. The patient had dementia and myoclonus. The 14-3-3 protein was positive in the CSF. Bilateral asymetric cerebral cortical abnormalities with high signal intensities were seen well on the DWI and to a lesser degree also seen on fluid-attenuated inversion recovery (FLAIR) images. No abnormal signal were seen in the basal ganglia and thalamus. Through this case report we want to emphasize the importance of DWI as a conjunct to the conventional MR sequences in cases in which the CJD is the prediagnosis in order to detect the disease in its earlier stages.

Keywords

• Magnetic rezonans imaging; Creutzfeldt-Jakob disease; isolated cortical involvement; diffusion-weighted imaging

Sporadik Creutzfeldt-Jakob Hastalığında İzole Kortikal Tutulumun Manyetik Rezonans Görüntüleme Bulguları

Abstract

Creutzfeldt-Jakob hastalığı (CJH) nadir bir demansiyel hastalıktır. Priona bağlı olduğu düşünülmektedir. Hızlıilerleyen demans, ataksi, miyoklonus, akinetik mutizm ve sonrasında ölüm ile karakterizedir. Beyin omurilik sıvısı(BOS)’ nda 14-3-3 proteinin tespiti CJH tanısına destek olabilir. Hastalığın orta veya geç evrelerinde elektroensefalografi(EEG)’de genellikle periyodik senkronize keskin dalga kompleksleri görülür. Difüzyon ağırlıklı görüntüleme (DAG)CJH tanısında en duyarlı manyetik rezonans görüntüleme (MRG) tekniğidir. Kesin tanı için beyin biyopsisi veya otopsigereklidir. Biz bu yazıda olası sporadik CJH tanısı konan ve izole kortikal tutulumu olan 60 yaşındaki erkek olguyusunuyoruz. Hastamızda demans bulguları ve myoklonus mevcuttu. BOS’ da 14-3-3 protein pozitifti. Kranial MRG’ deserebral kortikal bölgelerde DAG ve fluid-attenuated inversion recovery (FLAIR) görüntülerde bilateral asimetrik yükseksinyal intensite izlendi. Bazal ganglionlar ve talamuslarda patolojik sinyal saptanmadı. CJH ön tanısı olan olgulardaözellikle hastalığın erken dönemde saptamasında konvansiyonel MRG sekanslarına DAG eklenmelidir

Keywords

• Manyetik rezonans görüntüleme, Creutzfeldt-Jakob hastalığı, İzole kortikal tutulum, Difüzyonağırlıklı görüntüleme

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