Sato K, Sato S, Ohta S, Mori H, Matsuoka S, Shirai T, Kanazaea A, Mizuno Y
Department of Neurology, Juntendo University School of Medicine.
No To Shinkei. 2000 Jul;52(7):643-53.
We report a 67-year-old man with progressive disturbance of gait. He was well until the spring of 1993 (62 years of the age), when he noted an onset of unsteady gait. He also noted that he started to have a difficulty in playing tennis, in which he became unable to hit the ball with his racket. He also noted parkinsonian features such as bradykinesia and loss of hand dexterity. He was treated with levodopa, which did not improve his symptoms. His MRI revealed marked atrophy of the cerebellum and the pons. The criss-cross high signal lesion was seen in the center of the pons. The third ventricle was dilated. The putamen was unremarkable. His subsequent course was complicated by easy to fall, difficulty in swallowing with episodes of aspiration pneumonia. He also developed nocturnal apneustic episodes. He was admitted to our hospital on November 15, 1998, when he was 67 years of the age. He had low grade fever and low blood pressure (98/70). He was anemic but not icteric. Tumors were palpated in his jaw, anterior chest, and in the left arm. He was alert but unable to convey his desire because of dyspnea and tracheostomy. His gaze was slightly restricted in the horizontal direction and markedly so in the vertical direction. Motor functions were difficult to evaluate. His clinical course was complicated by atelectasis of the right lung and pleural effusion. He developed marked edema and oliguria. He developed sudden bradycardia and expired on December 26, 1998. He was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had multiple system atrophy. Majority of the audience agreed with this diagnosis. Post-mortem examination revealed a lung cancer in the right lung (undifferentiated adenocarcinoma) with metastases to the liver, kidneys, lymph nodes, pericardium, pleura, skin, bone marrow, and the brain. Neuropathologic examination revealed marked atrophy of the pons and the cerebellum. The putamen showed brownish discoloration and atrophic changes. The substantia nigra showed marked neuronal loss and gliosis. Oligodendrocytic inclusion bodies (alpha-synuclein positive) were seen in the putamen, globus pallidus, substantia nigra, pontine nucleus, cerebellar white matter, internal capsule, cerebral peduncle, and the spinal cord. These findings are consistent with the pathologic diagnosis of multiple system atrophy. What was interesting to us was the presence of neurofibrillary tangles in the substantia nigra, nucleus ruber, globus pallidus, and subthalamic nucleus. Tuft-shaped astrocytes were also seen. This patient appears to be a rare example of combination of MSA and PSP.
我们报告一名67岁男性，患有进行性步态障碍。1993年春（62岁）之前他身体状况良好，当时他注意到步态不稳。他还发现自己打网球开始变得困难，无法用球拍击球。他也出现了帕金森氏症特征，如运动迟缓及手部灵活性丧失。他接受了左旋多巴治疗，但症状未改善。他的磁共振成像（MRI）显示小脑和脑桥明显萎缩。脑桥中央可见交叉高信号病变。第三脑室扩张。壳核无明显异常。他随后的病程出现了易跌倒、吞咽困难并伴有吸入性肺炎发作的情况。他还出现了夜间呼吸暂停发作。1998年11月15日，67岁的他入住我院。他有低热和低血压（98/70）。他有贫血但无黄疸。在他的颌部、前胸和左臂可触及肿块。他意识清醒，但因呼吸困难和气管切开无法表达自己的需求。他的目光在水平方向略有受限，在垂直方向明显受限。运动功能难以评估。他的临床病程出现了右肺肺不张和胸腔积液。他出现了明显水肿和少尿。他突发心动过缓，于1998年12月26日死亡。在一次神经科病例讨论会（CPC）上对他进行了讨论，主要讨论者得出结论，该患者患有多系统萎缩。大多数与会者同意这一诊断。尸检显示右肺有肺癌（未分化腺癌），已转移至肝脏、肾脏、淋巴结、心包、胸膜、皮肤、骨髓和脑。神经病理学检查显示脑桥和小脑明显萎缩。壳核呈现褐色变色及萎缩性改变。黑质显示明显的神经元丢失和胶质细胞增生。在壳核、苍白球、黑质、脑桥核、小脑白质、内囊、大脑脚和脊髓中可见少突胶质细胞包涵体（α-突触核蛋白阳性）。这些发现与多系统萎缩的病理诊断一致。令我们感兴趣的是，在黑质、红核、苍白球和丘脑底核中存在神经原纤维缠结。还可见束状星形胶质细胞。该患者似乎是多系统萎缩（MSA）和进行性核上性麻痹（PSP）合并的罕见病例。
