Ishida Shimon, Doi Yoshimitsu, Yamane Kazushi, Sugino Masakazu, Kimura Fumiharu, Hanafusa Toshiaki, Fukui Hideo, Tamai Hiroshi
The First Department of Internal Medicine, Osaka Medical College, Japan.
Intern Med. 2012;51(13):1759-63. doi: 10.2169/internalmedicine.51.7341. Epub 2012 Jul 1.
A 38-year-old woman with Wilson's disease developed neurological deterioration after 25 years of low-dose penicillamine administration. She showed an akinetic-rigid syndrome and cerebellar motor ataxia. Brain MRI showed increased signal intensity at the bilateral pons, midbrain, putamen, and thalamus. 123I-IMP-SPECT revealed a diffuse reduction of cerebral blood flow at the bilateral cerebral hemisphere including the basal ganglia. After the patient's regimen was changed to zinc therapy, her neurological condition gradually improved, and she showed almost complete recovery within two years. Serial MRI and SPECT studies showed a marked improvement in the lesions.
一名38岁的威尔逊氏病女性患者,在接受低剂量青霉胺治疗25年后出现神经功能恶化。她表现出运动不能-强直综合征和小脑性运动共济失调。脑部磁共振成像显示双侧脑桥、中脑、壳核和丘脑信号强度增加。123I-异碘安非他明单光子发射计算机断层扫描显示双侧大脑半球包括基底神经节的脑血流量弥漫性减少。在将患者的治疗方案改为锌疗法后,她的神经状况逐渐改善,并且在两年内几乎完全康复。系列磁共振成像和单光子发射计算机断层扫描研究显示病变有明显改善。