Wakerley B R, Tan M H, Ting E Y
The Oxford Headache Centre, Department of Neurology, John Radcliffe Hospital, UK
Department of Ophthalmology, Royal Perth Hospital, Australia.
Cephalalgia. 2015 Mar;35(3):248-61. doi: 10.1177/0333102414534329. Epub 2014 May 20.
Idiopathic intracranial hypertension or pseudotumour cerebri is primarily a disorder of young obese women characterised by symptoms and signs associated with raised intracranial pressure in the absence of a space-occupying lesion or other identifiable cause.
The overall incidence of idiopathic intracranial hypertension is approximately two per 100,000, but is considerably higher among obese individuals and, given the global obesity epidemic, is likely to rise further. The pathophysiology of this condition is poorly understood, but most theories focus on the presence of intracranial venous hypertension and/or increased cerebrospinal fluid outflow resistance and how this relates to obesity. A lack of randomised clinical trials has resulted in unsatisfactory treatment guidelines and although weight loss is important, especially when used in conjunction with drugs that reduce cerebrospinal fluid production, resistant cases remain difficult to manage and patients invariably undergo neurosurgical shunting procedures. The use of transverse cerebral sinus stenting remains contentious and long-term benefits are yet to be determined.
An understanding of the clinical features, diagnostic work-up and therapeutic options available for patients with idiopathic intracranial hypertension is important both for neurologists and ophthalmologists as visual loss maybe permanent if untreated.
特发性颅内高压或假性脑瘤主要是年轻肥胖女性的一种疾病，其特征是在没有占位性病变或其他可识别病因的情况下，出现与颅内压升高相关的症状和体征。
特发性颅内高压的总体发病率约为每10万人中有2例，但在肥胖个体中发病率要高得多，鉴于全球肥胖流行，发病率可能会进一步上升。这种疾病的病理生理学尚不清楚，但大多数理论集中在颅内静脉高压和/或脑脊液流出阻力增加的存在以及这与肥胖的关系上。缺乏随机临床试验导致治疗指南不尽人意，尽管减肥很重要，尤其是与减少脑脊液生成的药物联合使用时，但难治性病例仍然难以管理，患者总是要接受神经外科分流手术。横窦支架置入术的应用仍存在争议，其长期益处尚未确定。
对于神经科医生和眼科医生来说，了解特发性颅内高压患者的临床特征、诊断检查和可用的治疗选择非常重要，因为如果不治疗，视力丧失可能是永久性的。
