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1.
Acute Confusional Migraine May Be a Presenting Feature of CADASIL   总被引:1,自引:0,他引:1  
Objective.— Characterize the phenomenon of acute confusional migraine (ACM) among Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) patients and emphasize the possibility of CADASIL in adults with ACM.
Background.— ACM, well described in children, has rarely been reported in adults. Although 30-60% of CADASIL patients have migraine, acute confusional state during migraine has not been described. We describe 7 patients with ACM that complicated up to 50% of the migraine episodes.
Design/Methods.— Detailed neurologic evaluation was performed in 20 CADASIL patients; International Classification of Headache Disorders 2nd edition criteria were used to diagnose migraine.
Results.— The mean age was 51 years. Fourteen patients reported headache and 11 met the criteria for migraine (mean age of onset 25). Seven patients experienced concomitant confusion, within 3 years of migraine onset. Confusion occurred either abruptly or insidiously, at the onset of aura or headache, lasting for 2-48 hours, and ending abruptly. These episodes were stereotypic, characterized by disorientation with agitation, and retrograde amnesia for the episodes. Patients reported disorientation to time and place, inability to recognize friends and relatives, difficulty with finding directions home, fear of getting lost, inability to analyze traffic lights or tell time. Patients reliably predicted the episodes and felt the need to seek a safe place for protection. Severity of the episodes progressed, but a striking improvement occurred after the first stroke.
Conclusion.— ACM may be a presenting feature and important clue, enabling CADASIL to be recognized up to a decade or earlier than at present. Therefore, a brain MRI and/or testing for Notch3 mutations should be considered in adult patients with ACM.  相似文献
2.
例1,女,47岁,三年来反复发作头昏,上下肢无力和走路不稳,以"脑梗塞"4次住院治疗.1个月前患者出现起床困难,持续数分钟后好转,伴有构音障碍,吞咽困难,饮水呛咳.否认高血压,高血脂,心脏病,糖尿病.查体:血压90/60 mmHg,构音障碍,定向力完整,近记忆力差,理解力正常,计算差.Romberg征(-).四肢腱反射活跃,左侧为著,双侧踝阵挛( ).四肢病理征( ).家族中有多人发病.  相似文献
3.
目的:研究CADASIL的影像学表现。材料与方法:报告1家系7人中4人患病,介绍了先证者的5年间颅脑CT、MRI表现。结果:颅脑MRI见脑内多发性腔梗灶和脑白质变性,颞叶前部和外囊部病变明显,且随年龄增加而加重,同时伴有脑萎缩。结论:MRI结合临床表现和家族史可诊断本病。  相似文献
4.
CADASIL病     
CADASIL(伴皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病)的研究是近年来证实遗传因素参与脑血管病发病的重要进展之一,其主要临床特点为家族遗传方式起病、中年发病、逐渐进展的缺血性脑卒中样病程、假性球麻痹、进行性血管性痴果、先兆症状的偏头痛发作和精神症状,脑内广泛多发的白质灶、明确的MRI白质异常信号以及病理学明确的小动脉病变是本病的基本特征,分子遗传学研究发现19号染色体Notch3基因突变与本病有关。  相似文献
5.
Mutations in the NOTCH3 gene cause cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Exons 3 and 4 are mutation hotspots. Migraine is a clinical hallmark of CADASIL. The objective of this study was to investigate whether genetic variants in exons 3 and 4 of the NOTCH3 gene are associated with migraine. Exons 3 and 4 of the NOTCH3 were analysed for mutations and polymorphisms by direct DNA sequencing in 97 migraineurs and the same number of control individuals. No mutations in exons 3 and 4 of the NOTCH3 gene were found in 97 patients with migraine. However, association analysis revealed significant association of the single nucleotide polymorphism (SNP) rs1043994 with migraine.  相似文献
6.
目的:探讨皮层下梗死伴白质脑病的常染色体显性遗传性脑动脉病(CADASIL)的磁共振表现.材料与方法:回顾性分析有临床症状的一家系三兄弟患者资料,分析其MRI信号改变、发病部位和预后.结果:3名CADSSIL患者具有或轻或重神经缺失,MRI表现均异常.第一,脑室旁周围白质、脑干、基底节区和丘脑出现多发腔隙性梗塞;第二,脑白质病出现在皮层下区域,具有对称并相互融合的趋势,其中以颞叶多见.第三,同时伴有脑萎缩.结论:结合MRI表现(皮层下弥漫对称脱髓鞘和腔隙性脑梗塞)和家族史可诊断CADASIL此病.  相似文献
7.
目的:分析CADASIL的临床及影像学表现。方法:回顾性分析我院基因检测诊断为CADASIL的患者11例,分析其临床及影像特点。结果:患者平均发病年龄为46岁,无血管疾病危险因素10例,MRI病变基本呈双侧对称分布,7例出现对称的颞极白质病变,8例白质病变累及外囊,6例出现胼胝体梗死,7例出现脑干梗死。结论:中青年发病,有阳性家族史,无脑血管疾病危险因素的脑缺血性卒中患者,结合典型影像特点,排除其它白质病变及血管病变,辅助基因检测及皮肤、周围血管活检结果可诊断CADASIL。  相似文献
8.
Different types of migraine have been reported in 20–40% of patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). We describe a novel migrainous manifestation of CADASIL consisting in status migrainosus and persistent aura without infarction. The symptoms resolved after i.v. treatment with lorazepam and mannitol.  相似文献
9.
It is remarkable that migraine is a prominent part of the phenotype of several genetic vasculopathies, including cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL), retinal vasculopathy with cerebral leukodystrophy (RVCL) and hereditary infantile hemiparessis, retinal arteriolar tortuosity and leukoencephalopahty (HIHRATL). The mechanisms by which these genetic vasculopathies give rise to migraine are still unclear. Common genetic susceptibility, increased susceptibility to cortical spreading depression (CSD) and vascular endothelial dysfunction are among the possible explanations. The relation between migraine and acquired vasculopathies such as ischaemic stroke and coronary heart disease has long been established, further supporting a role of the (cerebral) blood vessels in migraine. This review focuses on genetic and acquired vasculopathies associated with migraine. We speculate how genetic and acquired vascular mechanisms might be involved in migraine.  相似文献
10.
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