Smolinski Lukasz, Ziemssen Tjalf, Akgun Katja, Antos Agnieszka, Skowrońska Marta, Kurkowska-Jastrzębska Iwona, Członkowska Anna, Litwin Tomasz
Second Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland.
Center of Clinical Neuroscience, Department of Neurology, University Clinic Carl Gustav Carus & Dresden University of Technology, Dresden, Germany.
Mov Disord. 2022 Dec;37(12):2446-2451. doi: 10.1002/mds.29229. Epub 2022 Sep 27.
Although brain atrophy is common in neurological Wilson's disease, longitudinal studies are lacking.
The objective of this study was to measure longitudinal brain atrophy rate and to relate it to the change in neurological impairment in Wilson's disease.
We included patients with brain imaging done at diagnosis and at least 12 months later. The atrophy rate was measured as percentage change in ventricular volume, whereas the change in neurological impairment was scored on the Unified Wilson's Disease Rating Scale.
Of 57 patients, 36 had neurological presentation, 17 had hepatic presentation, and 4 were presymptomatic. The annualized atrophy rate was significantly greater in patients with the neurological presentation than in other patients (P = 0.001). In the neurological presentation, the atrophy rate correlated with the change in impairment (rho = 0.39, P = 0.018) and was significantly greater in those with worsening after diagnosis than in those without worsening (P < 0.001).
Brain atrophy rate appears as a promising marker of neurodegeneration in Wilson's disease. © 2022 International Parkinson and Movement Disorder Society.
尽管脑萎缩在神经型威尔逊病中很常见，但缺乏纵向研究。
本研究的目的是测量纵向脑萎缩率，并将其与威尔逊病神经功能损害的变化相关联。
我们纳入了在诊断时及至少12个月后进行脑成像检查的患者。萎缩率以脑室体积的百分比变化来衡量，而神经功能损害的变化则根据统一威尔逊病评定量表进行评分。
57例患者中，36例有神经症状表现，17例有肝脏症状表现，4例为症状前患者。有神经症状表现的患者年化萎缩率显著高于其他患者（P = 0.001）。在有神经症状表现的患者中，萎缩率与功能损害的变化相关（rho = 0.39，P = 0.018），且诊断后病情恶化的患者的萎缩率显著高于未恶化的患者（P < 0.001）。
脑萎缩率似乎是威尔逊病神经退行性变的一个有前景的标志物。© 2022国际帕金森病和运动障碍协会。
