Department of Ophthalmology, Emory University, Atlanta, Georgia, USA.
Am J Ophthalmol. 2011 Aug;152(2):163-9. doi: 10.1016/j.ajo.2011.03.020. Epub 2011 Jun 21.
To provide an update on various features of idiopathic intracranial hypertension.
Perspective.
Selected articles on the epidemiology, clinical and imaging features, natural history, pathophysiology, and treatment of idiopathic intracranial hypertension were reviewed and interpreted in the context of the authors' clinical and research experience.
Idiopathic intracranial hypertension primarily is a disease of obese women of childbearing age, but it can affect patients of any weight, sex, and age. Although a relatively rare disorder, idiopathic intracranial hypertension's associated costs in the United States entail hundreds of millions of dollars. Even after treatment, headaches frequently are persistent and may require the continued involvement of a neurologist. Quality-of-life reductions and depression are common among idiopathic intracranial hypertension patients. However, visual dysfunction, especially visual field abnormalities, represents the major morbidity of this disorder, and serial automated perimetry remains the primary method of patient monitoring. Patients who are men, black, very obese, or anemic are at higher risk of visual loss. Vitamin A metabolism, adipose tissue as an actively secreting endocrine tissue, and cerebral venous abnormalities are areas of active study regarding the pathophysiology of idiopathic intracranial hypertension. Treatment studies show that lumbar puncture is a valuable treatment (in addition to its crucial diagnostic role), and that weight management is critical. However, open questions remain regarding the efficacy of acetazolamide, cerebrospinal fluid diversion procedures, and cerebral venous stenting.
Many questions remain unanswered about idiopathic intracranial hypertension. Ongoing studies, especially an ongoing National Institutes of Health-funded clinical trial of acetazolamide, should provide more insight into this important, yet poorly understood, syndrome of isolated intracranial hypertension.
介绍特发性颅内高压的各种特征。
观点。
在作者的临床和研究经验的背景下,回顾和解释了有关特发性颅内高压的流行病学、临床和影像学特征、自然史、病理生理学和治疗的选定文章。
特发性颅内高压主要是肥胖育龄妇女的疾病,但它可以影响任何体重、性别和年龄的患者。尽管是一种相对罕见的疾病,但特发性颅内高压在美国的相关费用涉及数亿美元。即使经过治疗,头痛仍然经常持续,并可能需要神经科医生的持续参与。特发性颅内高压患者的生活质量下降和抑郁很常见。然而,视觉功能障碍,特别是视野异常,是这种疾病的主要发病率,并且连续自动视野检查仍然是患者监测的主要方法。男性、黑人、非常肥胖或贫血的患者发生视力丧失的风险更高。维生素 A 代谢、脂肪组织作为活跃的内分泌组织以及脑静脉异常是特发性颅内高压病理生理学的活跃研究领域。治疗研究表明,腰椎穿刺是一种有价值的治疗方法(除了其至关重要的诊断作用外),体重管理至关重要。然而,关于乙酰唑胺、脑脊液分流术和脑静脉支架的疗效仍存在悬而未决的问题。
关于特发性颅内高压仍有许多问题未得到解答。正在进行的研究,特别是美国国立卫生研究院资助的乙酰唑胺临床试验,应该为这种重要但尚未被充分理解的孤立性颅内高压综合征提供更多的见解。