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Abstract：
Idiopathic intracranial hypertension (IIH) is a neurological disease characterized by symptoms and signs of increased intracranial pressure (ICP) of unknown cause. Most attention has been given to the role of cerebrospinal fluid (CSF) disturbance and intracranial venous hypertension caused by sinus vein stenosis. We previously proposed that key pathophysiological processes take place within the brain at the glia-neuro-vascular interface. However, the relative importance of the proposed mechanisms in IIH disease remains unknown. Modern treatment regimens aim to reduce intracranial CSF and venous pressures, but a substantial proportion of patients experience lasting complaints. In 2010, the first author established a database for the prospective collection of information from individuals being assessed for IIH. The database incorporates clinical, imaging, physiological, and biological data, and information about treatment/outcome. This study retrieved information from the database, asking the following research questions: In IIH subjects responding to shunt surgery, what is the occurrence of signs of CSF disturbance, sinus vein stenosis, intracranial hypertension, and microscopic evidence of structural abnormalities at the glia-neuro-vascular interface? Secondarily, do semi-quantitative measures of abnormal ultrastructure at the glia-neurovascular differ between subjects with definite IIH and non-IIH (reference) subjects? The study included 13 patients with IIH who fulfilled the diagnostic criteria and who improved following shunt surgery, i.e., patients with definite IIH. Comparisons were done regarding magnetic resonance imaging (MRI) findings, pulsatile and static ICP scores, and immune-histochemistry microscopy. Among these 13 IIH subjects, 6/13 (46%) of patients presented with magnetic resonance imaging (MRI) signs of CSF disturbance (empty sella and/or distended perioptic subarachnoid spaces), 0/13 (0%) of patients with IIH had MRI signs of sinus vein stenosis, 13/13 (100%) of patients with IIH presented with abnormal preoperative pulsatile ICP [overnight mean ICP wave amplitude (MWA) above thresholds], 3/13 (23%) patients showed abnormal static ICP (overnight mean ICP above threshold), and 12/13 (92%) of patients with IIH showed abnormal structural changes at the glia-neuro-vascular interface. Comparisons of semi-quantitative structural variables between IIH and aged- and gender-matched reference (REF) subjects showed IIH abnormalities in glial cells, neurons, and capillaries. The present data suggest a key role of disease processes affecting the glia-neuro-vascular interface.
摘要：
特发性颅内高压（IIH）是一种神经系统疾病，其特征是原因不明的颅内压（ICP）升高的症状和体征。最关注的是由窦静脉狭窄引起的脑脊液（CSF）紊乱和颅内静脉高压的作用。我们先前提出，关键的病理生理过程发生在大脑中的神经胶质-神经血管界面。然而,所提出的机制在IIH疾病中的相对重要性仍然未知.现代治疗方案旨在降低颅内CSF和静脉压，但是很大一部分患者经历了持久的抱怨。2010年，第一作者建立了一个数据库，用于从正在评估IIH的个人那里收集信息。该数据库包含临床，成像,生理,和生物数据，以及有关治疗/结果的信息。这项研究从数据库中检索信息，问以下研究问题：在IIH受试者对分流手术的反应中，脑脊液紊乱的迹象是什么，窦静脉狭窄,颅内高压,神经胶质-神经-血管界面结构异常的微观证据？其次，神经胶质血管异常超微结构的半定量测量在明确IIH和非IIH（参考）受试者之间是否存在差异？该研究包括13名符合诊断标准且在分流手术后有所改善的IIH患者，即，明确IIH的患者。对磁共振成像（MRI）的发现进行了比较，脉动和静态ICP评分，和免疫组织化学显微镜。在这13名IIH受试者中，6/13（46％）的患者出现CSF紊乱的磁共振成像（MRI）征象（空蝶鞍和/或扩张的视神经周围蛛网膜下腔），0/13（0％）的IIH患者有MRI征象的窦静脉狭窄，13/13（100％）的IIH患者出现异常的术前脉动ICP[夜间平均ICP波振幅（MWA）高于阈值]，3/13（23％）患者表现出异常的静态ICP（夜间平均ICP高于阈值），和12/13（92％）的IIH患者在神经胶质-神经-血管界面显示异常结构变化。IIH与年龄和性别匹配的参考（REF）受试者之间的半定量结构变量的比较显示神经胶质细胞中的IIH异常，神经元，和毛细血管。目前的数据表明疾病过程影响神经胶质-神经-血管界面的关键作用。
