Obama Ruriko, Tachikawa Hiroshi, Yoshii Fumihito, Takeoka Tsuneyuki, Shinohara Yukito
Department of Neurology, Tokai University School of Medicine.
Rinsho Shinkeigaku. 2002 Sep;42(9):885-8.
A 48-year-old women was admitted to our hospital because of gradually developed spastic gait. She showed spasticity of the lower extremities with mild weakness. Laboratory tests disclosed decreased WBC and platelet counts and mild increases of transaminase and total bilirubin. Blood manganese level was markedly increased (6.0 micrograms/dl). Abdominal ultrasound showed splenomegaly, and abdominal angiography showed a dilatation of the portal and paraumbilical veins. T1-weighted MR images showed high signal intensities at the bilateral globus pallidus and cerebral peducles, and T2-weighted images showed high signal intensities at the bilateral deep white matter, posterior limbs of the internal capsule and right upper cervical spinal cord. Following the diagnosis of IPH, splenectomy was performed. The blood level of manganese decreased thereafter and her neurological deficits gradually improved. Hepatic diseases often show high signal intensities at the basal ganglia on T1-weighted images, and this seemed to be due to accumulation of manganese in our case. Because demyelination or axonal injury of the spinal cord are found in hepatic disease, we speculate that the high signal intensities at the spinal cord on T2-weighted images of our case reflect hepatic myelopathy, which may also be caused by high blood levels of manganese.
一名48岁女性因逐渐出现的痉挛性步态入住我院。她表现为下肢痉挛伴轻度无力。实验室检查显示白细胞和血小板计数降低，转氨酶和总胆红素轻度升高。血锰水平显著升高（6.0微克/分升）。腹部超声显示脾肿大，腹部血管造影显示门静脉和脐旁静脉扩张。T1加权磁共振图像显示双侧苍白球和大脑脚呈高信号强度，T2加权图像显示双侧深部白质、内囊后肢和右上颈脊髓呈高信号强度。在诊断为肝豆状核变性后，进行了脾切除术。此后血锰水平下降，她的神经功能缺损逐渐改善。肝脏疾病在T1加权图像上常显示基底节区高信号强度，在我们的病例中这似乎是由于锰的蓄积。因为在肝脏疾病中发现脊髓脱髓鞘或轴索损伤，我们推测我们病例中T2加权图像上脊髓的高信号强度反映了肝性脊髓病，这也可能是由高血锰水平引起的。
