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一名49岁男性,有进行性构音障碍、吞咽困难和左侧偏瘫。

[A 49-year-old man with progressive dysarthria, dysphagia, and left hemiparesis].

作者信息

Nishimiya J, Yamaji Y, Nitta T, Mori H, Yamamura A, Shirai T, Kondo T, Sato K, Mizuno Y

机构信息

Department of Neurology, National Institute for Psychiatric and Neurologic Disorders Kohnodai Hospital, Japan.

出版信息

No To Shinkei. 1995 Nov;47(11):1109-18.

PMID:7495619
Abstract

We report a 49-year-old man who presented progressive dysarthria, dysphagia, and left hemiparesis. The patient was well until June 28th of 1993 when he noted 'weakness' in his both legs; despite his weakness, he could play golf on that day. In the beginning of July, he noted difficulty in swallowing solid foods. He was admitted to the neurosurgery service of our hospital on July 15th of 1993 and a neurologic consultation was asked on July 17th. Neurologic examination at that time revealed an alert but somewhat childish man who appeared to have some difficulty in paying attention to questions. He was disoriented to time and showed difficulty in recent memory and calculation. Higher cerebral functions were intact. The optic fundi were normal; pupils were isocoric and reacted to light promptly; ocular movements were intact, however, he showed difficulty in convergence. Facial sensation and facial muscles were intact. He had no deafness. He showed slurred speech and difficulty in swallowing solid foods. The remaining cranial nerves were intact. Motor-wise, he was able to walk normally and no weakness or atrophy was noted. Mild ataxia was noted in the finger-to-nose and the heel-to-knee test on the left. Muscle stretch reflexes were normal and symmetric, however, the plantar response was extensor bilaterally. Sensation was intact and no meningeal signs were noted. General routine laboratory findings were unremarkable. CSF was under a normal pressure containing 1 cell/microliter, 68 mg/dl of protein, and 54 mg/dl of glucose. Cranial CT scan showed low density areas involving the pons, midbrain, left thalamus, and the left parietal cortex. In MRI, these areas presented low signal intensity in T1-weighted images and high signal intensity in T2-weighted in images. The brain stem appeared swollen. Gadolinium enhancement was negative. He was given a course of steroid pulse with 1 g/day of DIV methylprednisolone for three days followed by oral steroid. He showed only temporary improvement in swallowing. In the subsequent course, he showed progressive deterioration in dysarthria and dysphagia. A biopsy was performed on the left parietal lobe lesion. After biopsy, he was treated with steroid and glycerol without improvement. A course of chemotherapy with procarbazine, MCNU, and vincristine was given; he did not respond to chemotherapy. His left hemiparesis deteriorated. He developed aspiration pneumonia from dysphagia and expired on October 22, 1993. The patient was discussed in a neurologic CPC, and the chief discussant arrived at the conclusion that the patient had astrocytoma grade III involving the pons, midbrain, thalamus, and the parietal cortex.(ABSTRACT TRUNCATED AT 400 WORDS)

摘要

我们报告一名49岁男性,出现进行性构音障碍、吞咽困难和左侧偏瘫。该患者此前情况良好,直到1993年6月28日,他注意到双腿“无力”;尽管双腿无力,但当天他仍能打高尔夫球。7月初,他发现吞咽固体食物困难。1993年7月15日,他入住我院神经外科,7月17日进行了神经科会诊。当时的神经科检查发现,患者意识清醒,但略显幼稚,似乎在回答问题时有些困难。他对时间定向障碍,近期记忆和计算能力也有困难。高级脑功能正常。眼底正常;瞳孔等大,对光反应迅速;眼球运动正常,但他在集合方面有困难。面部感觉和面部肌肉正常。他没有听力障碍。他说话含糊不清,吞咽固体食物困难。其余颅神经正常。运动方面,他能正常行走,未发现无力或萎缩。左侧指鼻试验和跟膝胫试验有轻度共济失调。肌肉牵张反射正常且对称,但双侧巴宾斯基征阳性。感觉正常,未发现脑膜刺激征。一般常规实验室检查结果无异常。脑脊液压力正常,每微升含1个细胞,蛋白68毫克/分升,葡萄糖54毫克/分升。头颅CT扫描显示脑桥、中脑、左侧丘脑和左侧顶叶皮质有低密度区。在MRI中,这些区域在T1加权图像上呈低信号强度,在T2加权图像上呈高信号强度。脑干似乎肿胀。钆增强扫描为阴性。给他进行了一个疗程的类固醇冲击治疗,每天静脉注射1克甲基泼尼松龙,共三天,随后口服类固醇。他吞咽功能仅暂时改善。在随后的病程中,他的构音障碍和吞咽困难逐渐恶化。对左侧顶叶病变进行了活检。活检后,他接受了类固醇和甘油治疗,但无改善。给予了一个疗程的丙卡巴肼、甲基环己亚硝脲和长春新碱化疗;他对化疗无反应。他的左侧偏瘫恶化。他因吞咽困难并发吸入性肺炎,于1993年10月22日死亡。该病例在神经科病例讨论会上进行了讨论,主要讨论者得出结论,该患者患有累及脑桥、中脑、丘脑和顶叶皮质的III级星形细胞瘤。(摘要截短至400字)

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