Department of Ophthalmology and Visual Science, Yale School of Medicine, New Haven, Connecticut.
Division of Radiology at the Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Pediatr Neurol. 2019 Oct;99:31-39. doi: 10.1016/j.pediatrneurol.2019.04.010. Epub 2019 May 17.
Revised diagnostic criteria for pseudotumor cerebri syndrome require three of four neuroimaging findings in the absence of papilledema. We examined the sensitivity and specificity of three or more of four of these magnetic resonance imaging (MRI) findings for pseudotumor cerebri syndrome in children.
As part of clinical care, patients in whom there was suspicion for pseudotumor cerebri syndrome underwent neurological and fundoscopic examinations, lumbar puncture, MRI, or magnetic resonance venogram. For this retrospective study, we used this information to classify 119 subjects into definite (n = 66) or probable pseudotumor cerebri syndrome (n = 12), elevated opening pressure without papilledema (n = 23), or controls who had normal opening pressure without papilledema (n = 24). A neuroradiologist, unaware of the clinical findings or original MRI report, reviewed MRIs for pituitary gland flattening, flattening of the posterior sclera, optic nerve sheath distention, and transverse venous sinus stenosis.
The presence of three or more MRI findings has a sensitivity of 62% (95% confidence interval: 47% to 75%) and a specificity of 95% (95% confidence interval: 77% to 100%), compared with controls. Two of three (transverse venous sinus stenosis, pituitary gland flattening, flattening of the posterior sclera) had a similar sensitivity and specificity. Transverse venous sinus stenosis alone had a slightly higher sensitivity (74%, 95% confidence interval: 60% to 85%) and specificity (100%, 95% confidence interval: 80% to 100%).
In children, three of four of the proposed neuroimaging criteria and transverse venous sinus stenosis alone have a moderate sensitivity and robust specificity for pseudotumor cerebri syndrome. MRIs should be reviewed for these criteria, and their presence should raise suspicion for pseudotumor cerebri syndrome in children, particularly if the presence of papilledema is uncertain.
修订后的假性脑瘤综合征诊断标准要求在无视乳头水肿的情况下，四项神经影像学检查中有三项符合。我们检查了四项磁共振成像（MRI）检查中三项或更多项检查对儿童假性脑瘤综合征的敏感性和特异性。
作为临床护理的一部分，怀疑患有假性脑瘤综合征的患者接受了神经学和眼底检查、腰椎穿刺、MRI 或磁共振静脉造影。在这项回顾性研究中，我们使用这些信息将 119 名受试者分为明确（n=66）或可能的假性脑瘤综合征（n=12）、眼压升高但无视乳头水肿（n=23）或对照组，即眼压正常且无视乳头水肿（n=24）。一位神经放射科医生，在不知道临床发现或原始 MRI 报告的情况下，对垂体腺变平、后巩膜变平、视神经鞘扩张和横窦狭窄的 MRI 进行了复查。
与对照组相比，存在三项或更多项 MRI 检查结果的敏感性为 62%（95%置信区间：47%至 75%），特异性为 95%（95%置信区间：77%至 100%）。三项中的两项（横窦狭窄、垂体腺变平、后巩膜变平）具有相似的敏感性和特异性。单独横窦狭窄的敏感性略高（74%，95%置信区间：60%至 85%），特异性（100%，95%置信区间：80%至 100%）较高。
在儿童中，四项神经影像学标准中的三项和单独的横窦狭窄对假性脑瘤综合征具有中等敏感性和较强特异性。应审查 MRI 以寻找这些标准，如果视乳头水肿的存在不确定，这些标准的存在应引起对儿童假性脑瘤综合征的怀疑。
