脑积水脑室-腹腔分流术后帕金森综合征3例临床分析

2018-05-18 李胜德 王琳 关鸿志 中国现代神经疾病杂志

男性,41岁,主因头痛2年余,饮水呛咳、吞咽困难、构音不清1月余,于2004年10月18日入院。

例1

【一般资料】

男性,41岁,

【主诉】

主因头痛2年余,饮水呛咳、吞咽困难、构音不清1月余,于2004年10月18日入院。

【现病史】

患者2年前无明显诱因出现缓慢加重的全头部胀痛,发作初期呈间断性,逐渐进展为持续性;外院头部CT显示梗阻性脑积水,遂行右侧侧脑室-腹腔分流术,术后头痛症状完全缓解。2个月前再次出现头痛,性质同前;外院头部CT显示梗阻性脑积水,再次行左侧侧脑室-右心房分流术,术后头痛症状完全缓解。1月余前逐渐出现流涎、声音低沉、饮水呛咳、吞咽困难、言语不清等症状;外院头部MRI未见脑室系统扩张。为求进一步诊断与治疗,至我院就诊。

【体格检查】

神志清楚,言语不清、声音低沉,反应迟钝,表情减少,眼球上视受限,颈项强直,四肢肌力正常、肌张力增高,肢体深浅感觉正常,双上肢伴随动作减少、双下肢腱反射活跃,左侧病理征阳性、右侧可疑阳性。

【辅助检查】

实验室检查血清学指标未见明显异常;腰椎穿刺脑脊液检查压力110mmH2O(1mmH2O=9.81×10-3kPa,80~180mmH2O),常规、生化、细胞学和免疫学指标均于正常水平。肌电图检查神经传导速度(NCV)和瞬目反射未见异常。入院后1周头痛症状加重,尿失禁伴全身颤抖,无法站立和行走,继而出现嗜睡。复查CT显示第三脑室和双侧侧脑室再次扩张,遂行左侧侧脑室-腹腔分流术,术后复查CT显示扩张的侧脑室明显缩小,但临床症状无明显改善。

【初步诊断

临床诊断为帕金森综合征(脑室-腹腔分流术后),

【治疗】

予以左旋多巴200mg/次(3次/d)+苄丝肼50mg/次(3次/d)以及金刚烷胺100mg/次(3次/d)口服,连续治疗10d后临床症状明显改善,可自主进流食,在轻微辅助下站立和行走。患者共住院22d,出院后失访。

例2

【一般资料】

女性,35岁,

【主诉】

因反复恶心、呕吐15个月,烦躁3个月,缄默、肢体僵直2个月,于2015年2月26日入院。

【现病史】

患者17年前颅脑创伤致脑积水于外院行右侧侧脑室-腹腔分流术,术后正常生活16年。15个月前开始反复出现恶心、呕吐,时为喷射状呕吐,呕吐物为胃内容物,外院予甘露醇治疗(具体剂量不详)后症状缓解,头部CT和(或)MRI显示侧脑室扩大,未予特殊处理。2个月前出现颈部前屈僵硬,言语减少,动作迟缓,外院诊断为抑郁症、自身免疫性脑炎,予舒必利对症治疗(具体剂量不详),治疗10d后出现吞咽困难、流涎、面无表情、肢体僵直、少动、无法行走,遂停药,症状仍持续加重。为求进一步诊断与治疗,至我院就诊。

【体格检查】

神志清楚,缄默,可配合闭眼、张口等简单指令,动作幅度小、动作缓慢,四肢肌力正常、肌张力增高,可见缓慢自主活动,肢体深浅感觉正常,四肢腱反射活跃,双侧病理征阳性,颈项强直,无疼痛回避,共济运动和姿势步态检查不配合。

【辅助检查】

实验室检查数次腰椎穿刺脑脊液压力200~260mmH2O,常规、生化、细胞学和免疫学指标均于正常值范围。头部CT和(或)MRI显示侧脑室呈扩大-缩小波动性变化(图1)。


图1  头部影像学检查所见。1a横断面CT(2013年11月5日)显示双侧侧脑室明显缩小;1b横断面CT(2013年12月24日)显示双侧侧脑室扩大;1c横断面FLAIR成像(2014年1月13日)显示双侧侧脑室扩大;1d横断面T1WI(2014年5月20日)显示双侧侧脑室扩大;1e横断面CT(2014年11月27日)显示双侧侧脑室明显缩小;1f横断面T1WI(2014年12月17日)显示双侧侧脑室较图1e扩大;1g横断面T1WI(2015年1月21日)显示双侧侧脑室较图1f扩大;1h横断面T1WI(2015年3月5日)显示双侧侧脑室较图1g缩小

【初步诊断】

临床诊断为帕金森综合征(脑室-腹腔分流术后)

【治疗】

予以左旋多巴400mg/次(3次/d)+苄丝肼100mg/次(3次/d)口服,症状逐渐缓解,可自主言语、遵嘱活动肢体、自主进食。患者住院25d,出院后10个月随访,药物剂量减至左旋多巴100mg/次(3次/d)+苄丝肼25mg/次(3次/d),临床症状持续改善,可自主行走、器械锻炼;出院后19个月随访时,继续服用左旋多巴+苄丝肼(药物剂量维持不变),运动功能基本恢复,无头痛、恶心、呕吐和肢体僵直等症状。

例3

【一般资料】

女性,18岁,

【主诉】

主因头痛4年,言语减少、动作迟缓1个月,于2015年12月23日入院。

【现病史】

患者4年前车祸伤后出现头部胀痛、视物模糊、全身乏力,偶有恶心、呕吐,呈非喷射状呕吐;外院头部CT显示脑积水,由于患者临床症状不明显,未予特殊处理。症状进行性加重,6个月前症状明显加重,外院头部CT显示梗阻性脑积水,行右侧侧脑室-腹腔分流术,术后症状缓解。1个月前出现反复头痛、言语减少、全身颤抖、动作迟缓,外院诊断为抑郁症,予奥氮平2mg后全身颤抖加重、言语不能、无法进食、行走不能,无意识障碍,停药后症状仍进行性加重。为求进一步诊断与治疗,至我院门就诊。

【体格检查】

神志清楚,不能对答,双眼上视眼震,颈项强直,四肢肌力检查不能配合、肌张力增高,可见缓慢自主运动,共济运动和感觉系统检查不能配合,病理征阴性。

【辅助检查】

头部CT显示侧脑室呈扩大-缩小波动性变化(图2)。


图2  头部横断面CT检查所见。2a2014年5月2日显示双侧侧脑室扩大;2b2015年6月19日显示双侧侧脑室明显扩大;2c2015年6月24日显示双侧侧脑室明显缩小;2d2015年10月8日显示脑室-腹腔分流术后双侧侧脑室明显扩大

【初步诊断】

临床诊断为帕金森综合征(脑室-腹腔分流术后),

【治疗】

予以左旋多巴100mg/次(3次/d)+苄丝肼25mg/次(3次/d)口服,连续治疗1个月后可自主进食,肢体活动增多。患者2个月后随访时,停用左旋多巴,可于辅助下行走,言语自如;3个月后随访时,能够生活自理。

原始出处:

李胜德,王琳,关鸿志, 等.脑积水脑室-腹腔分流术后帕金森综合征三例临床分析[J].中国现代神经疾病杂志,2017,(2):127-132. DOI:10.3969/j.issn.1672-6731.2017.02.008.

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    2018-05-25 杨柳青青

    签到学习了--

    0

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    2018-05-20 tastas
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    2018-05-18 杨柳青青

    签到学习了--

    0

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