儿童Rasmussen脑炎的MRI影像特点及诊断
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摘要
目的分析儿童Rasmussen脑炎的MRI表现特点,提高对该病的认识及诊断水平。方法收集9例经临床或病理证实的Rasmussen脑炎患者的MRI表现及临床资料,男5例,女4例,平均年龄7.3岁,急性发病时平均年龄4.7岁,评估患者脑内异常信号及脑萎缩改变及进展情况。结果 9例Rasmussen脑炎MRI特点:1)信号异常:皮层及皮层下T_2WI、FLAIR高信号(8/9),累及两个或以上脑叶(5/8),以额顶叶多见;DWI稍高信号(2/8),等或稍低信号(6/8);尾状核或壳核T_2WI、FLAIR高信号(5/8); MRI信号无异常(1/9),SPECT患侧半球灌注减低;2)脑萎缩(8/9):患侧局部脑回变小及脑沟增宽(7/8),尾状核及壳核萎缩(5/8),外侧裂增宽(7/8),患侧颞角扩大(8/8),患侧侧脑室体部扩大(5/8),病变晚期皮层萎缩为半球性(7/8);3)病变进展情况(3/9):3例接受随访患者MRI表现患侧半球脑皮层异常信号范围加大,萎缩程度加重。结论儿童Rasmussen脑炎MRI影像表现对该病的诊断及预后评估有较高的价值,其特点为一侧皮层或皮层下T_2WI、FLAIR高信号,大脑半球进行性萎缩改变,可累及深部灰质核团,了解此病的MRI特点,有助于提高对该病的认识水平。
Objective To analyze the characteristics of MRI manifestations of Rasmussen encephalitis in children, and to improve the understanding and diagnostic level of the disease. Methods MRI findings and clinical data were analyzed retrospectively in 9 patients with Rasmussen encephalitis confirmed by clinical or pathology, including 5 cases of male, 4 cases of female, with an average age of 7.3 years and the average onset age of 4.7 years, then, abnormal signals in the brain and brain atrophy change and progress were evaluated. Results MRI features of 9 cases of Rasmussen encephalitis included: 1) signal abnormality: T_2WI and FLAIR hyperintensity(8/9) in the cortex and subcortical layer, and two or more cerebral leaves(5/8), especially in the frontal parietal lobes; Caudate nucleus or shell nuclei high signal(5/8); no abnormality in the MRI signal(1/9); 2) the brain atrophy(8/9) : small local gyrus and widened sulci in the isolateral hemisphere(7/8), atrophy in caudate nucleus and putamen(5/8), lateral fissure broadened(7/8), temporal Angle(8/8) and lateral ventricle expanded(5/8), late cortical atrophy lesions for hemisphere(7/8); 3) progression of the lesion(3/9) : 3 patients with follow-up MRI showed an increase in abnormal signal range in the lateral hemispheric cortex and increased brain atrophy. Conclusion MRI findings demonstrated rasmussen encephalitis for children have higher value to evaluate the diagnosis and prognosis of the disease, and its characteristics including unilateral cortical or subcortical T_2 and FLAIR hyperintensity, progressive brain atrophy such as enlargement of lateral ventricle, lateral fissure and sulci, the deep gray matter nuclei may be involved. So, understanding the certain MRI characteristics can help to improve the level of diagnosis of the disease.
Objective To analyze the characteristics of MRI manifestations of Rasmussen encephalitis in children, and to improve the understanding and diagnostic level of the disease. Methods MRI findings and clinical data were analyzed retrospectively in 9 patients with Rasmussen encephalitis confirmed by clinical or pathology, including 5 cases of male, 4 cases of female, with an average age of 7.3 years and the average onset age of 4.7 years, then, abnormal signals in the brain and brain atrophy change and progress were evaluated. Results MRI features of 9 cases of Rasmussen encephalitis included: 1) signal abnormality: T_2WI and FLAIR hyperintensity(8/9) in the cortex and subcortical layer, and two or more cerebral leaves(5/8), especially in the frontal parietal lobes; Caudate nucleus or shell nuclei high signal(5/8); no abnormality in the MRI signal(1/9); 2) the brain atrophy(8/9) : small local gyrus and widened sulci in the isolateral hemisphere(7/8), atrophy in caudate nucleus and putamen(5/8), lateral fissure broadened(7/8), temporal Angle(8/8) and lateral ventricle expanded(5/8), late cortical atrophy lesions for hemisphere(7/8); 3) progression of the lesion(3/9) : 3 patients with follow-up MRI showed an increase in abnormal signal range in the lateral hemispheric cortex and increased brain atrophy. Conclusion MRI findings demonstrated rasmussen encephalitis for children have higher value to evaluate the diagnosis and prognosis of the disease, and its characteristics including unilateral cortical or subcortical T_2 and FLAIR hyperintensity, progressive brain atrophy such as enlargement of lateral ventricle, lateral fissure and sulci, the deep gray matter nuclei may be involved. So, understanding the certain MRI characteristics can help to improve the level of diagnosis of the disease.
引文
[1] Bien CG, Granata T, Antozzi C, et al. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a european consensus statement [J]. Brain, 2005, 128(3): 454-471.
[2] Press C, Wallace A, Chapman KE. The Janus-faced nature of Rasmussen's encephalitis [J]. Semin Pediatr Neurol, 2014, 21(2): 129-136.
[3] 侯丽亚,石向群,罗红波,等. Rasmussen脑炎的发病机制及病例特点(附1例报道)[J].神经损伤与功能重建,2015,10(1):75-77.
[4] Pardo CA, Vining EP, Guo L, et al. The pathology of Rasmussen syndrome: stages of cortical involvement and neuropathological studies in 45 hemispherectomies [J]. Epilepsia, 2004, 45(5): 516-526.
[5] Derry PA, Harnadek MC, McLachlan RS, et al. A longitudinal study of the effects of seizure symptoms on the Minnesota Multiphasic Personality Inventory-2 (MMPI-2) clinical interpretation [J]. J Clin Psychol, 2002,58(7): 817-826.
[6] Holec M, Nagahama Y, Kovach C, et al.Rethinking the magnetic resonance imaging findings in early rasmussen encephalitis: a case report and review of the literature [J]. Pediatr Neurol, 2016, 59(6): 85-89.
[7] Pradeep K, Sinha S, Saini J, et al. Evolution of MRI changes in Rasmussen's encephalitis [J]. Acta Neurol Scand, 2014, 130(4): 253-259.
[8] Kuki I, Matsuda K, Kubota Y, et al. Functional neuroimaging in Rasmussen syndrome [J]. Epilepsy Research, 2018, 140(2): 120-127.
[9] 费容,张雅红,于祥,等. 磁共振扩散加权成像在病毒性脑炎中的临床价值分析[J].医学影像学杂志,2013,23(11):1787-1788.
[10] 刘文源,张立波,邹明宇,等. 磁共振对 Rasmussen脑炎的诊断价值[J].中国医科大学学报, 2013,42(7):638-640.
[11] 赵殿江,朱明旺,杜铁桥,等. Rasmussen脑炎的MRI诊断[J].中华放射学杂志,2012,46(4):308-311.
[2] Press C, Wallace A, Chapman KE. The Janus-faced nature of Rasmussen's encephalitis [J]. Semin Pediatr Neurol, 2014, 21(2): 129-136.
[3] 侯丽亚,石向群,罗红波,等. Rasmussen脑炎的发病机制及病例特点(附1例报道)[J].神经损伤与功能重建,2015,10(1):75-77.
[4] Pardo CA, Vining EP, Guo L, et al. The pathology of Rasmussen syndrome: stages of cortical involvement and neuropathological studies in 45 hemispherectomies [J]. Epilepsia, 2004, 45(5): 516-526.
[5] Derry PA, Harnadek MC, McLachlan RS, et al. A longitudinal study of the effects of seizure symptoms on the Minnesota Multiphasic Personality Inventory-2 (MMPI-2) clinical interpretation [J]. J Clin Psychol, 2002,58(7): 817-826.
[6] Holec M, Nagahama Y, Kovach C, et al.Rethinking the magnetic resonance imaging findings in early rasmussen encephalitis: a case report and review of the literature [J]. Pediatr Neurol, 2016, 59(6): 85-89.
[7] Pradeep K, Sinha S, Saini J, et al. Evolution of MRI changes in Rasmussen's encephalitis [J]. Acta Neurol Scand, 2014, 130(4): 253-259.
[8] Kuki I, Matsuda K, Kubota Y, et al. Functional neuroimaging in Rasmussen syndrome [J]. Epilepsy Research, 2018, 140(2): 120-127.
[9] 费容,张雅红,于祥,等. 磁共振扩散加权成像在病毒性脑炎中的临床价值分析[J].医学影像学杂志,2013,23(11):1787-1788.
[10] 刘文源,张立波,邹明宇,等. 磁共振对 Rasmussen脑炎的诊断价值[J].中国医科大学学报, 2013,42(7):638-640.
[11] 赵殿江,朱明旺,杜铁桥,等. Rasmussen脑炎的MRI诊断[J].中华放射学杂志,2012,46(4):308-311.