Cranial magnetic resonance imaging of Wolfram (DIDMOAD) syndrome.
Cranial magnetic resonance imaging of Wolfram (DIDMOAD) syndrome.
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DOI：
10.1111/j.1440-1673.2005.01420.x
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发表时间：
2005-04-01
期刊：
影响因子：
--
通讯作者：
Sermez, Y
中科院分区：
文献类型：
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作者：
Pakdemirli, E;Karabulut, N;Sermez, Y
Wolfram 综合征是一种罕见的神经退行性疾病，其特征为尿崩症、糖尿病、视神经萎缩和耳聋 (DIDMOAD)。还观察到中枢神经系统、泌尿道和内分泌腺的广泛异常。我们报告了一位患有 Wolfram 综合征的 32 岁女性患者的头颅 MRI 结果。除了神经垂体正常高信号缺失、视觉通路、脑干、小脑和大脑皮层萎缩等经典特征外，我们还观察到与颞叶和顶枕叶脑室周围白质视辐射相关的质子密度和T2加权图像双侧高信号，这可能在病理上反映神经胶质增生。
Wolfram syndrome is a rare neurodegenerative disorder characterized by diabetes insipidus, diabetes mellitus, optic atrophy and deafness (DIDMOAD). A wide spectrum of abnormalities of the central nervous system, urinary tract and endocrine glands is also observed. We report cranial MRI findings in a 32-year-old female patient with Wolfram syndrome. In addition to the classical features, including absence of the normal high signal of the neurohypophysis, atrophy of visual pathways, the brainstem, cerebellum and cerebral cortex, we observed bilateral hyperintensity on proton density- and T2- weighted images related to the optic radiations in the periventricular white matter of the temporal and parieto-occipital lobes, which may reflect gliosis pathologically.