中枢神经系统表面铁沉积症1例报道

<正>1病例介绍患者女性,74岁,主因“走路不稳、双下肢麻木4年,双上肢麻木疼痛1年”于2021年11月1日入院。患者4年前无明显诱因逐渐出现走路不稳、行走蹒跚、双下肢麻木,伴双手持物时轻微不自主抖动,言语停顿,伴头晕、情绪低落,无头部、肢体静止时抖动,无肢体僵硬、表情僵硬,无肢体无力、言语不清、口角流涎,无精神行为异常、睡眠中行为异常、记忆力减退,无嗅觉减退、便秘、二便失禁,无吞咽困难、饮水呛咳、视物成双。     

肾上腺脑白质营养不良一例

<正>患者男性,15岁,主因双眼视物不清、记忆力减退进行性加重2年,于2012年6月18日入院。患者2年前出现双眼视物不清,认为是因患者喜玩电脑游戏而出现近视,予佩戴约-300度眼镜,但视力仍下降,换为-400多度的眼镜仍视物不清,并相继出现记忆力、计算力减退,学习成绩下降,由原来的班内前十名变为倒数几名,以上两组症状呈进行性加重。6个月前逐渐表现反应迟钝,行走不稳,尤其爬坡时明显,不能上体育课,容易疲劳。于入     

脊髓小脑性共济失调6型一家系12例报道

1临床资料1先证者,男,50岁.渐进性走路不稳、饮水呛咳,说话不清8年就诊.8年前患者无明显诱因出现走路不稳,易跌倒,当时院外头颅MRI示"小脑萎缩",诊为"遗传性共济失调",经治疗(具体不详)后症状无缓解,且逐渐加重;4年前患者骑自行车困难;3年前行走不稳加重,并出现双手持物不稳、复视及饮水呛咳;1年余前上述症状进一步加重,生活不能自理.     

肝硬化、行走不稳

患者 男性，55岁。因行走不稳，进行性加重4年于2006年8月7日入院。4年前无明显诱因出现行走不稳，易摔倒，逐渐出现双手活动笨拙，记忆力减退；症状呈缓慢性展，渐至行走困难。慢性乙型肝炎病史30余年，肝硬化4年，糖尿病1年余，心房颤动1年，近几年偶有黑便。     

肝硬化、行走不稳

患者 男性，55岁。因行走不稳，进行性加重4年于2006年8月7日入院。4年前无明显诱因出现行走不稳，易摔倒，逐渐出现双手活动笨拙，记忆力减退；症状呈缓慢性展，渐至行走困难。慢性乙型肝炎病史30余年，肝硬化4年，糖尿病1年余，心房颤动1年，近几年偶有黑便。     

合并脑干、小脑损害的肝豆状核变性1例报告

报告伴脑干、小脑损害的肝豆状核变性(HLD)1例如下。1病例男,20岁。因“口齿不清3年、全身不自主抖动2年”于2007年10月19日入院。患者3年前无明显诱因出现口齿不清并逐渐加重,2年前出现全身不自主抖动,逐渐加重并出现走路不稳、流涎、失眠、记忆力下降;曾在当地人民医院行头部     

海洛因致中毒性白质脑病2例报道

1　病　例例 1,患者 ,男性 ,40岁 ,因手抖半年 ,走路不稳、吐词不清半月入院 ,患者半年前在执筷、玩牌时出现轻微手抖 ,偶尔出现饮水呛咳 ,以后手抖逐渐加重 ,书写困难 ,入院前半月出现走路不稳 ,易向右侧倾倒 ,需人扶行 ,同时出现吐词不清 ,无发热、头痛、肢体麻木和无力。既往无高血压病及糖尿病病史 ,有吸食海洛因史 3年 ,入院前半月开始戒毒。查体 :体温 36 .5℃ ,心率 70次 /分 ,呼吸 2 0次 /分 ,血压 10 5 / 75ｍｍＨｇ,神志清楚 ,精神稍差 ,吟诗样语言 ,双侧瞳孔等大等圆 ,直径 2 .5ｍｍ ,光反射灵敏 ,双眼球有不恒定的轻微水平眼震 …     

橄榄桥脑、小脑萎缩1例报告(OPCA)

患者男性,38岁,因头晕、行走不稳、言语不清6年,排尿费力一年,于1991年1月16日入院.患者于6年前,无明显诱因出现头晕、言语不清,双下肢无力,二年后,双下肢无力加重,走路不稳,以踏棉花感,性功能减退,当时侧血压为12/7kPa,在当地医院按“脑血栓”治疗,病情不见好转,走路不稳,言语不清,逐渐加重,走路时经常前倾而跌倒,近一年来,排尿费力,既往:健康,否认家族史。查体:T36℃,P96次/分,R18次/分,血压,卧位12/8.4kPa,立位9.6/5kPa,神志清,言语含糊不清,记忆力、计算力尚好,眼底正常,双软腭抬举有力,咽反射双侧减弱,余颅神经未见异常。双下肢肌力略弱、肌张力高、双膝腱反射活跃、踝震挛( )、右侧 Babinski 氏征( ),chaddok 氏征( )、左侧 Babinski 氏征( )、双下肢音叉振动觉减弱、右侧提睾反射弱。双手指鼻稳准、轮替灵活、双侧跟膝胫不稳准,龙伯氏征( )。辅助检查:腰穿脑脊液压力正常,压颈通畅、常规生化检查正常,头部 CT 扫描:鞍上池、环池扩大、小脑脑沟加深、双侧处侧裂扩大,大脑沟加深,其他血液化验均正常.     

记忆力减退 精神行为异常 四肢抽搐

正病例摘要患者男性,74岁。因记忆力减退7年、行为异常2年、四肢抽搐4 d,于2014年10月22日入院。患者系财务工作者,自7年前开始出现严重记忆力减退,如工作中不记得他人交代的事情,经反复提示亦无任何印象,需依靠便条、日记等方法协助记事;此后6个月逐渐出现重复询问同样问题,有时晨起刷牙5～6次,自觉记忆力有问题,于2007年10月     

Hallervorden—Spatz病1例报告

Hallervorden- Spatz病临床少见 ,现报告 1例如下。1　病例　男 ,61岁。因四肢运动不灵 ,伴走路不稳 10年 ,加重 1年 ,于 2 0 0 0年 1月 2 0日入院。患者自 1990年始逐渐出现四肢活动不灵、行走不稳 ,夜间较为明显。近 1年上述症状逐渐加重 ,1999年 4月在当地医院就诊 ,头颅 MRI检查发现双侧桥脑、基底节对称性短 T2 信号影 ,按“脑梗死”行活血化瘀治疗无效。近 1月言语吐字不清、语速变慢、语音单调低沉、记忆力下降、偶有吞咽困难、饮水呛咳。家族中无类似病人。查体 :神志清楚 ,表情呆板 ,慌张步态 ,构音不清 ,简易智能量表检查正常…     

[20]右下肢麻木、无力20余年,左下肢麻木、无力8年,排尿困难2年(上)

患者,女,46岁。于2012年3月21日入神经内科会诊中心病房。20余年前无明显诱因开始出现右下肢麻木及无力感,无疼痛及晨轻暮重感,症状缓慢进行性加重,于12年前右下肢不能抬起。8年前开始出现左下肢疼痛,并伴有麻木及活动不灵     

[16]进行性四肢无力9年,足尖行走4年(上)

患者男性,14岁,主因"进行性四肢无力9年,行走不能1年"于2004年8月于北京神经内科会诊中心会诊.患者9年前(5岁)出现四肢力弱,进展缓慢,并逐渐消瘦,跑步较同龄儿童慢.4年前(10岁)出现双上肢伸不直,足尖走路,低头困难.1年前(13岁)独立行走困难.既往史:足月顺产,病前发育正常.否认类似疾病家族史.     

[20]右下肢麻木、无力20余年,左下肢麻木、无力8年,排尿困难2年(下)——椎管内硬膜外脂肪增多症

会诊中心讨论分析病史及辅助检查见本刊2012年第38卷8期。会诊专家分析:魏岗之、康德瑄、杨秉贤教授:右下肢麻木、无力20余年,左下肢麻木、无力8年,排尿困难2年。神经     

Malnutrition at age 3 years and externalizing behavior problems at ages 8, 11, and 17 years

OBJECTIVE: Poor nutrition is thought to predispose to externalizing behavior problems, but to date there appear to have been no prospective longitudinal studies testing this hypothesis. This study assessed whether 1) poor nutrition at age 3 years predisposes to antisocial behavior at ages 8, 11, and 17 years, 2) such relationships are independent of psychosocial adversity, and 3) IQ mediates the relationship between nutrition and externalizing behavior problems. METHOD: The participants were drawn from a birth cohort (N=1,795) in whom signs of malnutrition were assessed at age 3 years, cognitive measures were assessed at ages 3 and 11 years, and antisocial, aggressive, and hyperactive behavior was assessed at ages 8, 11, and 17 years. RESULTS: In relation to comparison subjects (N=1,206), the children with malnutrition signs at age 3 years (N=353) were more aggressive or hyperactive at age 8 years, had more externalizing problems at age 11, and had greater conduct disorder and excessive motor activity at age 17. The results were independent of psychosocial adversity and were not moderated by gender. There was a dose-response relationship between degree of malnutrition and degree of externalizing behavior at ages 8 and 17. Low IQ mediated the link between malnutrition and externalizing behavior at ages 8 and 11. CONCLUSIONS: These results indicate that malnutrition predisposes to neurocognitive deficits, which in turn predispose to persistent externalizing behavior problems throughout childhood and adolescence. The findings suggest that reducing early malnutrition may help reduce later antisocial and aggressive behavior.     

Stroke-blind for colors, faces and locations: Partial recovery after three years

Purpose. To study and follow-up achromatopsia, prosopagnosia, and topographagnosia in a patient who suffered a bilateral stroke of the posterior cerebral arteries. Methods. Ophthalmological, neuropsychological and neuroradiological examinations were conducted over a span of 3 years to assess the amount of brain damage and look for signs of functional recovery. Results. After the onset of achromatopsia, perception of the color green re-appeared first, followed by red, yellow, and brown. Blue which had appeared entirely black was last to return. While reading and color naming have largely recovered, color discrimination after three years remains poor especially in dim lighting. Similarly, with prosopagnosia, while the patient has learn to identify people (including photographs) by individual features, his ability to perceive and recognize faces and facial expressions holistically remains severely impaired. Recognition of streets, houses, and topographical layouts also continues to be affected, while the perception of speed and distance has somewhat improved. Perimetry further suggests a mild improvement of the superior hemianopia in his visual fields. Conclusions. Although major deficits in color, face and place perception remain, some functions impaired by the stroke have returned par-alleling a partial recovery from the brain lesion as demonstrated by NMR.     

Stroke-blind for colors, faces and locations: Partial recovery after three years

Purpose. To study and follow-up achromatopsia, prosopagnosia, and topographagnosia in a patient who suffered a bilateral stroke of the posterior cerebral arteries. Methods. Ophthalmological, neuropsychological and neuroradiological examinations were conducted over a span of 3 years to assess the amount of brain damage and look for signs of functional recovery. Results. After the onset of achromatopsia, perception of the color green re-appeared first, followed by red, yellow, and brown. Blue which had appeared entirely black was last to return. While reading and color naming have largely recovered, color discrimination after three years remains poor especially in dim lighting. Similarly, with prosopagnosia, while the patient has learn to identify people (including photographs) by individual features, his ability to perceive and recognize faces and facial expressions holistically remains severely impaired. Recognition of streets, houses, and topographical layouts also continues to be affected, while the perception of speed and distance has somewhat improved. Perimetry further suggests a mild improvement of the superior hemianopia in his visual fields. Conclusions. Although major deficits in color, face and place perception remain, some functions impaired by the stroke have returned par-alleling a partial recovery from the brain lesion as demonstrated by NMR.     

反复发热 头痛12年

正病历摘要患者男性,33岁,反复发热、头痛12年,于2007年6月18日入院。患者12年前(1995年5月)无明显诱因突然出现发热、头痛,体温不详,全头部持续性钝痛,伴呕吐,校医院诊断为"感冒",予抗炎治疗(具体方案不详),治疗后症状缓