Alexander disease: diagnosis with MR imaging.
Alexander disease: diagnosis with MR imaging.
复制标题
亚历山大病：磁共振成像诊断。
DOI：
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复制
发表时间：
2001
期刊：
影响因子：
--
通讯作者：
J. Powers
中科院分区：
文献类型：
--
作者：
M. Knaap;S. Naidu;S. Breiter;S. Blaser;H. Stroink;S. Springer;Jacobus C. Begeer;Rudy van Coster;P. Barth;N. Thomas;J. Valk;J. Powers
背景和目的
到目前为止，罗森塔尔纤维在脑活检或尸检标本上的展示被认为是亚历山大病确诊的先决条件。我们对亚历山大病的假定和确诊病例的MR异常进行了多机构调查，以评估基于MR诊断的可能性。
方法
分析了3例尸检诊断为亚历山大病的患者的MR成像研究，以确定诊断的MR标准。然后将这些标准应用于217名不明原因的白质脑病儿童。
结果
定义了5个MR成像标准：广泛的大脑白色物质改变，额叶占优势，T1加权图像上高信号和T2加权图像上低信号的脑室周围边缘，基底节和丘脑异常，脑干异常，以及特定的灰色和白色物质结构的对比增强。基于MR成像的诊断必须满足五项标准中的四项。在对217例患者的MR研究的回顾性分析中，发现19例符合这些标准。在这些患者中未发现其他实质性新发MR异常。在19例患者中，有4例获得了随后的组织学证实。临床病理学在有和无组织学证实的患者中是相同的，并且与MR异常相关。MR异常与已知的亚历山大病的组织病理学结果非常一致。
结论
定义的标准是足够的亚历山大病的体内磁共振成像诊断，只有在非典型的情况下，脑活检仍然是必要的明确诊断。
BACKGROUND AND PURPOSE
To date, the demonstration of Rosenthal fibers on brain biopsy or autopsy specimens is considered a prerequisite for a definitive diagnosis of Alexander disease. We initiated a multiinstitutional survey of MR abnormalities in both presumed and confirmed cases of Alexander disease to assess the possibility of an MR-based diagnosis.
METHODS
MR imaging studies in three patients with an autopsy-based diagnosis of Alexander disease were analyzed to define MR criteria for the diagnosis. These criteria were then applied to 217 children with leukoencephalopathy of unknown origin.
RESULTS
Five MR imaging criteria were defined: extensive cerebral white matter changes with frontal predominance, a periventricular rim with high signal on T1-weighted images and low signal on T2-weighted images, abnormalities of basal ganglia and thalami, brain stem abnormalities, and contrast enhancement of particular gray and white matter structures. Four of the five criteria had to be met for an MR imaging-based diagnosis. In a retrospective analysis of the MR studies of the 217 patients, 19 were found who fulfilled these criteria. No other essentially new MR abnormalities were found in these patients. In four of the 19 patients, subsequent histologic confirmation was obtained. The clinical symptomatology was the same in the patients with and without histologic confirmation and correlated well with the MR abnormalities. MR abnormalities were in close agreement with the known histopathologic findings of Alexander disease.
CONCLUSION
The defined criteria are sufficient for an in vivo MR imaging diagnosis of Alexander disease; only in atypical cases is a brain biopsy still necessary for a definitive diagnosis.
影响因子：
4.2
作者：
McNaught, KSP;Olanow, CW
通讯作者：
Olanow, CW
Alpha B-晶状体蛋白和 27-kd 热休克蛋白受中枢神经系统应激条件的调节，并在 Rosenthal 纤维中积累。
DOI：
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发表时间：
1993
期刊：
The American journal of pathology
影响因子：
--
作者：
Iwaki,T;Iwaki,A;Tateishi,J;Sakaki,Y;Goldman,JE
通讯作者：
Goldman,JE