Matsubayashi S, Sato K, Takase M, Mori H, Suda K, Kondo T, Mizuno Y
Department of Neurology, Juntendo University, School of Medicine, Bunkyo, Japan.
No To Shinkei. 1996 Feb;49(2):185-93.
We report a 83 year-old woman with dementia. She was apparently well until December of 1993 when she was 81-year-old. At that time, she was operated or her cataract. Her post operative course was uneventful, however, shortly after her operation, she had an onset of memory loss and abnormal behavior. She showed a fluctuating course in her mental disturbance. In 1995, her dementia worsened with nocturnal agitation. She was admitted to our service on June 12, 1995. She was alert and her blood pressure was 140/100 mmHg. She showed recent memory loss and disorientation to time. Motor wise, she was unable to stand unsupported. Her gait with support showed small steps and a wide base. She was bradykinetic and ataxic in her finger-to-nose and heel-to-knee test, however, no rigidity or tremor was noted. Her MRI showed T2-high signal lesions in both medial thalamic areas, in the right occipital lobe, and in the bilateral cerebral white matters as well as in the basal ganglia. She was discharged for out-patient follow up on July 3, 1995. Four days after the discharge, she showed declining responses to stimuli and she developed dyspnea on July 14, 1995. She was admitted again on the same day. Her body temperature was 38.5 degrees C and moist rales were heard in the left lung field. She appeared drowsy and no verbal response was obtained; no apparent motor palsy was noted. Blood count showed leukocytosis (14,300/ml). Blood gas analysis under 61 of oxygen inhalation through a mask was as follows: pH 7.460, PCO2 39.6 mmHg, PO2 67 mmHg, and HCO3-28.5 mEq/l. Two days after admission, she developed a convulsion in her left arm and she became unconscious. Her EEG showed periodically recurring lateralized epileptic discharges on the right fronto-central areas. Her subsequent course was complicated by status epilepticus and respiratory distress. She died on July 26, 1995. She was discussed in a neurological CPC. The chief discussant arrived at a conclusion that she suffered from multi-infarct dementia. Bilateral thalamic infarctions were considered to have played a significant role in her dementia. Post-mortem examination revealed subcortical leukoencephalopathy of Binswanger's type and cerebral infarctions in the thalamic and basal ganglia regions and in the right occipital lobe. In addition, she showed isolated angitis of the central nervous system involving mainly in the small arteries located in the superficial areas of the brain and the spinal cord. This patient was interesting in that despite relatively mild leukoaraiosis in MRI, post-mortem examination revealed profound pathologic changes in the subcortical white matters. In addition, she showed the isolated angitis of the CNS. The cause and the clinical correlates of her angitis were unclear.
我们报告一例83岁患有痴呆症的女性。她在1993年12月81岁之前身体状况显然良好。当时,她接受了白内障手术。术后恢复过程顺利,然而,术后不久,她开始出现记忆力减退和行为异常。她的精神障碍呈波动病程。1995年,她的痴呆症因夜间躁动而加重。1995年6月12日她入住我们科室。她神志清醒,血压为140/100 mmHg。她存在近期记忆力减退和时间定向障碍。在运动方面,她无法独立站立。在有人扶持下行走时,她步伐小且步基宽。在指鼻试验和跟膝试验中,她动作迟缓且共济失调,然而,未发现强直或震颤。她的磁共振成像(MRI)显示双侧丘脑内侧区域、右侧枕叶、双侧脑白质以及基底节区有T2高信号病变。1995年7月3日她出院进行门诊随访。出院四天后,她对刺激的反应逐渐减弱,并于1995年7月14日出现呼吸困难。同一天她再次入院。她体温为38.5摄氏度,左肺野可闻及湿啰音。她看起来昏昏欲睡,未得到言语回应;未发现明显的运动性麻痹。血常规显示白细胞增多(14,300/ml)。通过面罩吸入61%氧气时的血气分析结果如下:pH 7.460,PCO2 39.6 mmHg,PO2 67 mmHg,HCO3- 28.5 mEq/l。入院两天后,她左臂出现抽搐并失去意识。她的脑电图显示右侧额中央区有周期性反复出现的局灶性癫痫放电。她随后的病程并发癫痫持续状态和呼吸窘迫。她于1995年7月26日死亡。在一次神经科病例讨论会(CPC)上对她的病例进行了讨论。主要讨论者得出结论,她患有多发梗死性痴呆。双侧丘脑梗死被认为在她的痴呆症中起了重要作用。尸检发现为宾斯旺格(Binswanger)型皮质下白质脑病以及丘脑、基底节区和右侧枕叶的脑梗死。此外,她还表现出主要累及脑和脊髓浅表区域小动脉的中枢神经系统孤立性血管炎。该患者的有趣之处在于,尽管MRI显示脑白质疏松相对较轻,但尸检发现皮质下白质有严重的病理改变。此外,她还表现出中枢神经系统孤立性血管炎。其血管炎的病因及临床相关因素尚不清楚。