Nishimura Y, Tsuda N, Ikemoto K, Negoro K, Morimatsu M
Department of Neurology, Yamaguchi University, School of Medicine.
Rinsho Shinkeigaku. 1996 Jun;36(6):764-9.
We report two patients, 73- and 70-year-old men, characterized by progressive hemiparesis and homolateral limb ataxia as the main clinical symptoms; magnetic resonance (MR) imaging of the brain revealed asymmetric cerebral cortical and peduncular atrophy; 99mTc-ECD single photon emission computed tomography (SPECT) of brain showed decreased RI uptake in the cerebral hemisphere correlated with clinical deficits. Brain SPECT of case 1 showed decreased RI uptake in the cortex of the right hemisphere and the left cerebellar hemisphere ("crossed cerebellar diaschisis; CCD"). These findings indicate that ataxia of our patients may depend on the lesions of the corticopontocerebellar tracts, although it is possible that ataxia may be related to lack of spatial orientation associated with parietal lobe lesion. The mechanism of the occurrence of asymmetric cerebral peduncular atrophy would be explained by wallerian degeneration of the pyramidal tract and other cortically originated fibers associated with the cortical degeneration. From these clinical and radiologic features, it seems likely that our two patients are categorized in the "asymmetric cortical degeneration syndromes", and we propose the term "progressive ataxic hemiparesis" for our patients.
我们报告了两名患者，分别为73岁和70岁男性，主要临床症状为进行性偏瘫和同侧肢体共济失调；脑部磁共振成像（MR）显示大脑皮质和脑桥萎缩不对称；脑部99mTc - ECD单光子发射计算机断层扫描（SPECT）显示大脑半球放射性摄取指数（RI）降低，与临床缺损相关。病例1的脑部SPECT显示右半球皮质和左小脑半球放射性摄取指数降低（“交叉性小脑失联络；CCD”）。这些发现表明，我们患者的共济失调可能取决于皮质脑桥小脑束的病变，尽管共济失调也可能与顶叶病变相关的空间定向障碍有关。大脑脚萎缩不对称的发生机制可通过锥体束及其他与皮质变性相关的皮质起源纤维的华勒氏变性来解释。从这些临床和放射学特征来看，我们的两名患者似乎可归类于“不对称皮质变性综合征”，我们为我们的患者提出“进行性共济失调性偏瘫”这一术语。
