Kim Younhee, Koide Reiji, Kawata Akihiro
Department of Neurology, Tokyo Metropolitan Neurological Hosptal.
Brain Nerve. 2015 May;67(5):635-8. doi: 10.11477/mf.1416200192.
A 37-year-old male patient presented with psychiatric symptoms, dysarthria, limb dystonia, increased tendon reflexes, and a Kayser-Fleischer ring in his late teens. Laboratory examinations showed decreased concentrations of serum copper and ceruloplasmin, and increased urinary copper levels. Magnetic resonance imaging (MRI) showed high-signal-intensity lesions in the bilateral putamen, globus pallidus, thalamus, and brainstem on T2-weighted images (T2WI). Based on the MRI results and laboratory data, we diagnosed this patient with Wilson's disease (WD). He was treated with trientine hydrochloride and zinc acetate. Four months after the initiation of treatment, the patient'symptoms began to improve. On a follow-up MRI that was obtained 6 years after treatment, the high-signal-intensity lesions on the T2WI had disappeared completely. However, the low-signal-intensity lesions in the basal ganglia had spread to the caudate nuclei. Here, we discuss the characteristics of the MRI changes in WD following treatment. The Pathological basis for the low-signal-intensity lesions on T2WI in WD remains unclear. Our results suggest that this lesion may reflect the accumulation of materials other than copper.
一名37岁男性患者在十几岁后期出现精神症状、构音障碍、肢体肌张力障碍、腱反射亢进以及Kayser-Fleischer环。实验室检查显示血清铜和铜蓝蛋白浓度降低,尿铜水平升高。磁共振成像(MRI)在T2加权像(T2WI)上显示双侧壳核、苍白球、丘脑和脑干有高信号强度病变。根据MRI结果和实验室数据,我们诊断该患者为威尔逊病(WD)。他接受了盐酸曲恩汀和醋酸锌治疗。治疗开始四个月后,患者症状开始改善。在治疗6年后进行的随访MRI检查中,T2WI上的高信号强度病变已完全消失。然而,基底节的低信号强度病变已蔓延至尾状核。在此,我们讨论WD治疗后MRI变化的特征。WD中T2WI上低信号强度病变的病理基础仍不清楚。我们的结果表明,这种病变可能反映了除铜以外物质的积累。