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12例累及胼胝体的急性播散性脑脊髓炎患者的临床特征及神经影像学表现

[Clinical features and neuroimaging findings of 12 patients with acute disseminated encephalomyelitis involved in corpus callosum].

作者信息

Liu Jian-guo, Qiao Wen-ying, Dong Qin-wen, Zhang Hai-ling, Zheng Kui-hong, Qian Hai-rong, Qi Xiao-kun

机构信息

Department of Neurology, Navy General Hospital of PLA, Beijing 100048, China.

出版信息

Zhonghua Yi Xue Za Zhi. 2012 Nov 20;92(43):3036-41.

Abstract

OBJECTIVE

To summarize the clinical features and neuroimaging findings of the patients with acute disseminated encephalomyelitis (ADEM) involved in corpus callosum (CC) so as to distinguish it from other diseases.

METHODS

A total of 12 ADEM patients with the involvement of CC during the period of 2010-2012 were recruited. There were 9 males and 3 females with a mean age of 31±14 years (range: 10-54). Their clinical and neuroimaging features were retrospectively reviewed and all data analyzed by SPSS 18.0.

RESULTS

(1) All of them had an acute or subacute onset. Two patients had a history of vaccination and 5 suffered upper respiratory tract infection or diarrhea. (2) The presenting symptoms included fever (n=5), headache (n=4), unsteady gait (n=2), urinary retention (n=1), indifference (n=1) and delirium (n=1). (3) The main clinical symptoms included memory loss (n=9), delirium (n=5), somnolence (n=4), urinary retention (n=9), paraplegia (n=4) and unsteady gait (n=5). (4) The examinations of cerebrospinal fluid (CSF) revealed increased intracranial pressure (n=4), leucocytosis (n=3) and increased protein (n=7) of 7 cases. All oligoclonal bands were negative. (5) The lesions were involved in bilateral CCs in 12 patients. Among them, splenium was the most commonly affected (n=9), secondly stem (n=5) and lastly genu (n=4). For 6 patients, the intracranial lesions were all in their CCs. And among them, 2 cases were involved in spinal cord. Except for CC, there were other focal lesions in brain stem and cerebellum (n=4) and spinal cord (n=6). (6) On magnetic resonance imaging (MRI), all cases showed long T2 signal intensity with blurred images. And among them, 2 cases' lesions in brain were discerned only by diffuse weighing imaging (DWI) or T2 fast fluid-attenuated inversion recovery (T2FLAIR) instead of T2-weighted. The lesions of CCs showed on gadolinium-enhanced MRI were significantly enhanced and the shapes were sheet-like (4/6). Spinal cord lesions was found in 6 cases and most spinal cord lesions were discontinuous. And the number of spinal cord segments with lesions was from 4 to 8. The shapes of lesions of spinal cord showed on enhanced MRI were like thin line. (7) Most of them were misdiagnosed as viral encephalitis (n=5), tuberculous meningoencephalitis (n=1) and brain neoplasms (n=2). And another case was admitted into urology surgery ward due to urinary retention.

CONCLUSION

There are three key points about the characteristics of the ADEM patients with CC lesions: (1) They may have an adult male preponderance. The distinctive symptoms include fever, headache, delirium, somnolence, memory loss, unsteady gait and urination disorders, etc.. (2) The number of lesions on brain MRI can be multiple or single, especially the lesions of CC (mostly in splenium). On MRI, all cases showed long T2 signal intensity with blurred images so that DWI and T2 FLAIR may have a higher efficiency of detecting the lesions. In particular, multiple lesions may be all enhanced or not enhanced at equal pace on enhanced MRI. (3) In ADEM patients with CC lesions, many indices of CSF chemical examination, such as increased intracranial pressure, leucocytosis, increased protein, low sugar and low chloride, indicate the presence of intracranial infective diseases. Therefore they are most likely to be misdiagnosed as viral encephalitis or tuberculous meningoencephalitis. However, CC is not the predilection site for viral encephalitis since CC belongs to white matter but not gray matter. So ADEM should be a more appropriate diagnosis for these cases.

摘要

目的

总结累及胼胝体(CC)的急性播散性脑脊髓炎(ADEM)患者的临床特征及神经影像学表现,以与其他疾病相鉴别。

方法

选取2010年至2012年期间12例累及CC的ADEM患者。其中男性9例,女性3例,平均年龄31±14岁(范围:10 - 54岁)。回顾性分析其临床及神经影像学特征,并采用SPSS 18.0对所有数据进行分析。

结果

(1)所有患者均急性或亚急性起病。2例有疫苗接种史,5例有上呼吸道感染或腹泻史。(2)首发症状包括发热(5例)、头痛(4例)、步态不稳(2例)、尿潴留(1例)、淡漠(1例)和谵妄(1例)。(3)主要临床症状包括记忆力减退(9例)、谵妄(5例)、嗜睡(4例)、尿潴留(9例)、截瘫(4例)和步态不稳(5例)。(4)脑脊液(CSF)检查显示7例患者颅内压升高(4例)、白细胞增多(3例)及蛋白升高(7例)。所有寡克隆带均为阴性。(5)12例患者双侧CC均有病变。其中,压部最常受累(9例),其次是体部(5例),膝部最少受累(4例)。6例患者颅内病变均局限于CC。其中2例累及脊髓。除CC外,脑干和小脑(4例)及脊髓(6例)还有其他局灶性病变。(6)在磁共振成像(MRI)上,所有病例均表现为长T2信号强度,图像模糊。其中2例脑内病变仅在弥散加权成像(DWI)或T2快速液体衰减反转恢复序列(T2FLAIR)上显示,而非T2加权像。CC病变在钆增强MRI上显示明显强化,形态为片状(4/6)。6例发现脊髓病变,多数脊髓病变不连续。病变累及的脊髓节段数为4至8个。增强MRI上脊髓病变形态呈细线状。(7)多数患者被误诊为病毒性脑炎(5例)、结核性脑膜脑炎(1例)和脑肿瘤(2例)。另有1例因尿潴留入住泌尿外科病房。

结论

累及CC病变的ADEM患者特征有三个关键点:(1)可能以成年男性居多。独特症状包括发热、头痛、谵妄、嗜睡、记忆力减退、步态不稳及排尿障碍等。(2)脑MRI上病变数量可为多发或单发,尤其是CC病变(多在压部)。MRI上所有病例均表现为长T2信号强度,图像模糊,因此DWI和T2 FLAIR对病变的检出效率可能更高。特别是在增强MRI上,多发病变可能全部强化或强化程度不一。(3)在累及CC病变的ADEM患者中,CSF化学检查的许多指标,如颅内压升高、白细胞增多、蛋白升高、低糖和低氯,提示存在颅内感染性疾病。因此他们最易被误诊为病毒性脑炎或结核性脑膜脑炎。然而,CC并非病毒性脑炎的好发部位,因为CC属于白质而非灰质。所以对于这些病例,ADEM应是更合适的诊断。

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