Idiopathic intracranial hypertension (pseudotumor cerebri): recognition, treatment, and ongoing management.

Idiopathic intracranial hypertension (pseudotumor cerebri): recognition, treatment, and ongoing management.
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DOI:
10.1007/s11940-012-0207-4
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发表时间:
2013-02
影响因子:
2
通讯作者:
Wall, Michael
Wall, Michael
中科院分区:
医学3区
文献类型:
--
作者:
Thurtell, Matthew J.;Wall, Michael

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特发性颅内高压(IIH,假性脑瘤)是一种不明原因的颅内压升高综合征,主要发生在育龄期肥胖女性。这是一种排除性诊断,因此,在做出诊断之前,必须通过病史、影像学和脑脊液检查来寻找颅内压升高的其他原因。IIH产生颅内压升高的症状和体征,包括视神经乳头水肿。如果不治疗,视乳头水肿可导致进行性不可逆视力丧失和视神经萎缩。治疗方法取决于症状和视力丧失的严重程度和时间进程,由正式的视野测试确定。治疗的主要目标是缓解症状,包括头痛和保护视力。应鼓励所有超重IIH患者参加体重管理计划,目标是减轻5%至10%的体重,同时沿着低盐饮食。当出现轻度视力丧失时,应开始乙酰唑胺治疗。当乙酰唑胺单药治疗不足或耐受性差时,可以添加或替代其他药物治疗。当视力丧失更严重或进展迅速时,可能需要手术干预,如视神经鞘开窗术或脑脊液分流术,以防止进一步的不可逆视力丧失。干预措施的选择取决于症状和视力丧失的相对严重程度以及当地的专业知识。目前,横静脉窦支架置入术的作用尚不清楚。虽然没有循证数据来指导治疗,但有一项正在进行的随机双盲安慰剂对照治疗试验,研究饮食和乙酰唑胺治疗IIH。
Idiopathic intracranial hypertension (IIH, pseudotumor cerebri) is a syndrome of elevated intracranial pressure of unknown cause that occurs predominantly in obese women of childbearing age. It is a diagnosis of exclusion and, therefore, other causes of increased intracranial pressure must be sought with history, imaging, and cerebrospinal fluid examination before the diagnosis can be made. IIH produces symptoms and signs of increased intracranial pressure, including papilledema. If untreated, papilledema can cause progressive irreversible visual loss and optic atrophy. The treatment approach depends on the severity and time course of symptoms and visual loss, as determined by formal visual field testing. The main goals of treatment are alleviation of symptoms, including headache, and preservation of vision. All overweight IIH patients should be encouraged to enter a weight-management program with a goal of 5% to 10% weight loss, along with a low-salt diet. When there is mild visual loss, medical treatment with acetazolamide should be initiated. Other medical treatments can be added or substituted when acetazolamide is insufficient as monotherapy or poorly tolerated. When visual loss is more severe or rapidly progressive, surgical interventions, such as optic nerve sheath fenestration or cerebrospinal fluid shunting, may be required to prevent further irreversible visual loss. The choice of intervention depends on the relative severity of symptoms and visual loss, as well as local expertise. At present, the role of transverse venous sinus stenting remains unclear. Although there are no evidence-based data to guide therapy, there is an ongoing randomized double-blind placebo-controlled treatment trial, investigating diet and acetazolamide therapy for IIH.
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