Dubovsky E C, Booth T N, Vezina G, Samango-Sprouse C A, Palmer K M, Brasseux C O
Department of Diagnostic Imaging and Radiology, Children's National Medical Center, Washington, DC 20010-2970, USA.
AJNR Am J Neuroradiol. 2001 Jan;22(1):190-5.
Many pediatric patients with neurofibromatosis type 1 (NF-1) have an apparent increased thickness of the corpus callosum (CC) on sagittal T1-weighted images compared with patients not affected by NF-1. In this study, we compared the surface area of the CC in children with NF-1 with that of healthy pediatric control subjects to determine if this was another common intracranial manifestation of NF-1.
Midsagittal T1-weighted MR images of 43 consecutive children with NF-1 and 43 age- and gender-matched healthy control subjects were reviewed retrospectively. The surface area of the CC and the midsagittal intracranial skull surface (MISS) area were measured five times each on all midsagittal images. A mean CC to mean midline intracranial surface area ratio (CC/MISS) was calculated for each.
There is a statistically significant increase in the mean CC surface area in pediatric patients with NF-1 (680 mm2 +/- 98, range 509-974 mm2) compared with control subjects (573 mm2 +/- 83, range 404-797 mm2). The mean MISS is significantly increased in patients with NF-1 (16568 mm2 +/- 1161, range 14107-19394 mm2 vs 15402 mm2 +/- 1133, range 12951-17905 mm2 for control subjects). CC/MISS was also significantly increased in the patients with NF-1 relative to the control subjects (.0410 +/- .0043, range .0330-.0530 vs .0372 +/- .0043, range .0270-.0470 for control subjects).
A larger midsagittal surface area of the CC is another intracranial manifestation of NF-1 that can be demonstrated by sagittal MR imaging. The etiology is unclear, but could be related to abnormal neurofibromin and Ras protein activity. Potential clinical relevance is discussed herein.
与未患1型神经纤维瘤病（NF-1）的患者相比，许多患NF-1的儿科患者在矢状面T1加权图像上胼胝体（CC）厚度明显增加。在本研究中，我们比较了患NF-1儿童与健康儿科对照受试者的CC表面积，以确定这是否为NF-1另一种常见的颅内表现。
回顾性分析43例连续的患NF-1儿童和43例年龄及性别匹配的健康对照受试者的矢状面T1加权磁共振图像。在所有矢状面图像上，对CC表面积和矢状面颅内颅骨表面（MISS）面积各测量5次。计算每个受试者的平均CC与平均中线颅内表面积比值（CC/MISS）。
与对照受试者（573 mm²±83，范围404 - 797 mm²）相比，患NF-1的儿科患者平均CC表面积有统计学显著增加（680 mm²±98，范围509 - 974 mm²）。NF-1患者的平均MISS也显著增加（16568 mm²±1161，范围14107 - 19394 mm²，对照受试者为15402 mm²±1133，范围12951 - 17905 mm²）。相对于对照受试者，NF-1患者的CC/MISS也显著增加（0.0410±0.0043，范围0.0330 - 0.0530，对照受试者为0.0372±0.0043，范围0.0270 - 0.0470）。
CC矢状面表面积增大是NF-1的另一种颅内表现，可通过矢状面磁共振成像显示。病因尚不清楚，但可能与神经纤维瘤蛋白和Ras蛋白活性异常有关。本文讨论了其潜在的临床意义。
