Okumura A, Kidokoro H, Tsuji T, Suzuki M, Kubota T, Kato T, Komatsu M, Shono T, Hayakawa F, Shimizu T, Morishima T
Department of Pediatrics, Juntendo University School of Medicine, Tokyo, Japan.
AJNR Am J Neuroradiol. 2009 Apr;30(4):825-30. doi: 10.3174/ajnr.A1431. Epub 2009 Jan 8.
The precise clinical characteristics of acute encephalopathy with bilateral reduced diffusion are not fully understood. We compared clinical, laboratory, and neuroimaging findings according to the patterns of brain lesions among children with reduced diffusion in the bilateral hemispheres.
Nine patients were analyzed. The patterns of brain lesions were divided into diffuse lesions and central-sparing lesions. Diffuse lesions were defined as reduced diffusion in the whole cortex and/or subcortical white matter. Central-sparing lesions were defined as the lack of reduced diffusion in the areas around the bilateral Sylvian fissures. Clinical, laboratory, and neuroimaging findings were compared between groups.
Five patients showed diffuse lesions and 4 showed central-sparing lesions. Coma was significantly more common in patients with diffuse lesions, whereas a biphasic clinical course was more common in those with central-sparing lesions. Outcome was worse in patients with diffuse lesions. Maximal aspartate aminotransferase, alanine aminotransferase, and kinase levels were also significantly higher in patients with diffuse lesions. In 2 patients with diffuse lesions, diffusion-weighted images during the acute phase revealed reduced diffusion in the bilateral frontal and occipital areas, followed by diffuse lesions. No patient with central-sparing lesions showed MR imaging abnormalities during the acute phase.
Clinical manifestations in patients with diffuse lesions were severe, whereas those in patients with central-sparing lesions were relatively mild.
双侧扩散受限的急性脑病的确切临床特征尚未完全明确。我们根据双侧半球扩散受限患儿的脑损伤模式,比较了临床、实验室及神经影像学检查结果。
对9例患者进行分析。脑损伤模式分为弥漫性损伤和中央保留性损伤。弥漫性损伤定义为整个皮质和/或皮质下白质扩散受限。中央保留性损伤定义为双侧外侧裂周围区域无扩散受限。比较两组患者的临床、实验室及神经影像学检查结果。
5例患者表现为弥漫性损伤,4例表现为中央保留性损伤。弥漫性损伤患者昏迷更为常见,而中央保留性损伤患者双相临床病程更为常见。弥漫性损伤患者的预后较差。弥漫性损伤患者的最大天冬氨酸转氨酶、丙氨酸转氨酶和激酶水平也显著更高。2例弥漫性损伤患者在急性期的扩散加权成像显示双侧额叶和枕叶区域扩散受限,随后出现弥漫性损伤。中央保留性损伤患者在急性期均未出现磁共振成像异常。
弥漫性损伤患者的临床表现严重,而中央保留性损伤患者的临床表现相对较轻。