Ikeda J, Tsuyumu M, Takada Y, Seida M, Inaba Y
No Shinkei Geka. 1986 May;14(6):771-5.
A case of Paget's disease in an elderly female who had a favourable evolution following ventriculoperitoneal (V-P) shunt is reported. On May 28, 1983, a 52-year-old female was transferred and admitted to us from the Dept. of Neurology because of headache in the occipital region and ataxic gait. On admission, neurological examinations revealed remarkably increased tendon reflexes, ataxic gait, and mild dementia. Headache was also observed, but urinary incontinence was not present. Skull X-ray showed "cotton wool appearance", which was characteristic of Paget's disease. On chemical analysis of blood and urine, serum Al-P and urinary OH-proline level were elevated, which established a diagnosis of Paget's disease. Triventricular dilatation was found on CT scan, and neck tomography showed basilar impression. After admission, the patient was treated with calcitonin, but it was interrupted because of side effects such as nausea and vomiting. Then she gradually took a turn for the worse, particularly dementia became severer. On July 25, 1983, V-P shunt was performed. After operation, "soft landing maneuver" was employed, namely the intraventricular pressure was checked and was gradually lowered with external CSF drainage system for 7 days. Thereafter the patient's head was elevated gradually from supine to sitting position through 7 days. Her hospital course after operation was that of gradual improvement. The purpose of this maneuver was to prevent sudden change of intraventricular pressure that causes aggravation of basilar impression and sudden respiratory arrest.
报告了一例患有佩吉特氏病的老年女性,其在接受脑室腹腔(V-P)分流术后病情向好发展。1983年5月28日,一名52岁女性因枕部头痛和共济失调步态从神经科转至我院并入院。入院时,神经检查发现腱反射明显增强、共济失调步态和轻度痴呆。也观察到头痛,但无尿失禁。颅骨X线显示“棉絮状外观”,这是佩吉特氏病的特征。血液和尿液化学分析显示血清碱性磷酸酶(Al-P)和尿羟脯氨酸水平升高,从而确诊为佩吉特氏病。CT扫描发现三脑室扩张,颈部断层扫描显示颅底陷入。入院后,患者接受降钙素治疗,但因恶心、呕吐等副作用而中断。随后她的病情逐渐恶化,尤其是痴呆变得更加严重。1983年7月25日,进行了V-P分流术。术后采用“软着陆操作”,即检查脑室内压力,并通过外部脑脊液引流系统在7天内逐渐降低压力。此后,患者的头部在7天内从仰卧位逐渐抬高至坐位。术后她的住院病程呈逐渐改善趋势。此操作的目的是防止脑室内压力突然变化导致颅底陷入加重和突然呼吸骤停。