Veselinovic Nikola, Pavlovic Aleksandra M, Petrovic Boris, Ristic Aleksandar, Novakovic Ivana, Svabic Medjedovic Tamara, Pavlovic Dragan, Sternic Nada
*Clinic for Neurology, Faculty of Medicine ‡Faculty for Special Education and Rehabilitation, University of Belgrade, Belgrade, Serbia †Diagnostic Center VAMED-VMR, Novi Sad, Serbia.
Cogn Behav Neurol. 2014 Mar;27(1):48-50. doi: 10.1097/WNN.0000000000000015.
Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disease caused by conformational alteration of the ubiquitous prion protein. Sporadic CJD appears to progress faster if the basal ganglia are shown to be affected on magnetic resonance imaging. Transcranial B-mode sonography (TCS) enables visualization of differences in tissue echogenicity, which can be associated with changes in the cerebral metabolism of various metals. These metabolic changes are considered 1 of the potential mechanisms of the brain damage in CJD; TCS hyperechogenicity may reflect changes in metal homeostasis in CJD. We report a 63-year-old woman who presented with typical sporadic CJD. One month after she fell ill, a magnetic resonance imaging scan of her brain showed diffuse cortical but no obvious basal ganglia involvement. However, TCS revealed moderate hyperechogenicity of both lentiform nuclei. The patient's disease progressed quickly and she died 2 months later. TCS may show basal ganglia alteration early in the disease course of patients with quickly progressing CJD, thus aiding in premortem diagnosis.
克雅氏病（CJD）是一种由普遍存在的朊病毒蛋白构象改变引起的致命性神经退行性疾病。如果磁共振成像显示基底神经节受影响，散发性克雅氏病似乎进展更快。经颅B型超声检查（TCS）能够可视化组织回声性的差异，这可能与各种金属的脑代谢变化有关。这些代谢变化被认为是克雅氏病脑损伤的潜在机制之一；TCS高回声可能反映克雅氏病中金属稳态的变化。我们报告了一名63岁患有典型散发性克雅氏病的女性。她患病一个月后，脑部磁共振成像扫描显示弥漫性皮质受累，但基底神经节无明显受累。然而，TCS显示双侧豆状核中度高回声。患者病情进展迅速，2个月后死亡。TCS可能在快速进展性克雅氏病患者的病程早期显示基底神经节改变，从而有助于生前诊断。
