Increased asymmetric pulvinar magnetic resonance imaging signals in Creutzfeldt-Jakob disease with florid plaques following a cadaveric dura mater graft
Increased asymmetric pulvinar magnetic resonance imaging signals in Creutzfeldt-Jakob disease with florid plaques following a cadaveric dura mater graft
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DOI：
10.1111/j.1440-1789.2006.00638.x
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发表时间：
2006-02-01
期刊：
影响因子：
2.3
通讯作者：
Iwaki, T
中科院分区：
文献类型：
--
作者：
Wakisaka, Y;Santa, N;Iwaki, T
一名9岁的日本女孩在头部受伤并脑挫伤后，在手术中接受了尸体硬脑膜移植。她继续做得很好，但当她19岁时，她逐渐表现出暴力的性格，并在精神病院接受治疗。又过了6年，手术后200个月，她出现了进行性步态共济失调，随后导致她在发病后10个月内死亡。尸检显示她患有克雅氏病。该患者为硬脑膜相关性克雅氏病（dCJD）的非典型病例，具有不寻常的临床病理特征，包括迟发性肌阵挛、脑电图中缺乏周期性同步放电和广泛的花斑。然而，我们检测到的不对称增加的MRI衍生图像枕核以前没有观察到在其他非典型病例的dCJD。由于非典型dCJD病例与vCJD病例有几个共同的临床病理学特征，并且由于在一些神经病理学证实的vCJD病例中观察到枕部不对称的高信号，因此我们在非典型dCJD和vCJD之间的鉴别诊断中存在一定的困难。这是第一个不典型的dCJD病例，在MRI上显示枕高信号比所有其他基底节高信号。
A 9-year-old Japanese girl received a cadaveric dura mater graft during surgery following a head injury with brain contusion. She continued to do well, but when she became 19-years-old, she gradually showed a violent character and was treated in a psychiatric hospital. Another 6 years later, 200 months after the procedure, she developed a progressive gait ataxia, which subsequently led to her death within 10 months of onset. An autopsy showed she had CJD. This patient represents an atypical case of dura-associated CJD (dCJD) with unusual clinicopathological features including the late occurrence of myoclonus, an absence of periodic synchronous discharges in the electroencephalogram, and the presence of widespread florid plaques. However, our detection of an asymmetrical increase in the MRI-derived images of pulvinar nuclei has not been previously observed in other atypical cases of dCJD. Because atypical dCJD cases share several clinicopathological features with those of vCJD, and because asymmetrical hyperintense signals in the pulvinar have been observed in some neuropathologically confirmed vCJD cases, we had some difficulty in a differential diagnosis between atypical dCJD and vCJD. This is the first atypical dCJD case showing a pulvinar high signal compared with all other basal ganglia on MRI.