特发性颅内压增高

一旦确诊颅内高压，超重患者应开始减重计划，并消除或治疗其他致病因素（例如，停用四环素类药物、维甲酸或过量的维生素 A，重新开始皮质类固醇治疗并更缓慢的减量，开始低钠饮食，以及建议轻微限制液体摄入）。[8]NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee; Wall M, McDermott MP, Kieburtz KD, et al. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA. 2014;311:1641-1651.http://jama.jamanetwork.com/article.aspx?articleid=1861803http://www.ncbi.nlm.nih.gov/pubmed/24756514?tool=bestpractice.com[10]Johnson LN, Krohel GB, Madsen RW, et al. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri). Ophthalmology. 1998 Dec;105(12):2313-7.http://www.ncbi.nlm.nih.gov/pubmed/9855165?tool=bestpractice.com[57]Newborg B. Pseudotumor cerebri treated by rice reduction diet. Arch Intern Med. 1974 May;133(5):802-7.http://www.ncbi.nlm.nih.gov/pubmed/4821775?tool=bestpractice.com[58]Sinclair AJ, Burdon MA, Nightingale PG, et al. Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension: prospective cohort study. BMJ. 2010 Jul 7;341:c2701.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2898925/http://www.ncbi.nlm.nih.gov/pubmed/20610512?tool=bestpractice.com证据 C视神经乳头水肿缓解：低质量证据表明，给予低能量饮食，限制液体和钠时，视神经乳头水肿会有所好转。[57]Newborg B. Pseudotumor cerebri treated by rice reduction diet. Arch Intern Med. 1974 May;133(5):802-7.http://www.ncbi.nlm.nih.gov/pubmed/4821775?tool=bestpractice.com低质量的观察性（队列）研究或者受试者＜200名且方法学存在缺陷的随机对照临床试验（RCT）。

建议患者使总体重减少 5%~10%，并保持减轻的体重。 饮食疗法效果不明显的特发性颅内高压 (IIH) 和病态肥胖 (BMI＞40) 患者，应考虑进行肥胖症手术（已被证明是有效的）。[60]Amaral JF, Tsiaris W, Morgan T, et al. Reversal of benign intracranial hypertension by surgically induced weight loss. Arch Surg. 1987 Aug;122(8):946-9.http://www.ncbi.nlm.nih.gov/pubmed/3632342?tool=bestpractice.com[61]Sugerman HJ, Felton WL 3rd, Sismanis A, et al. Gastric surgery for pseudotumor cerebri associated with severe obesity. Ann Surg. 1999 May;229(5):634-40.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1420807/http://www.ncbi.nlm.nih.gov/pubmed/10235521?tool=bestpractice.com

有许多与 IIH 相关的疾病和医源性病因；如可能，应消除或降低其存在的可能性。[1]Wall M. Idiopathic intracranial hypertension. Neurol Clin. 2010;28:593-617.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2908600/http://www.ncbi.nlm.nih.gov/pubmed/20637991?tool=bestpractice.com

针对经选择患者组全部患者的治疗推荐

特发性颅内高压治疗试验 (IIHTT) 的研究结果为轻度视力丧失的特发性颅内高压 (IIH) 患者提供了循证治疗策略。[8]NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee; Wall M, McDermott MP, Kieburtz KD, et al. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA. 2014;311:1641-1651.http://jama.jamanetwork.com/article.aspx?articleid=1861803http://www.ncbi.nlm.nih.gov/pubmed/24756514?tool=bestpractice.com 在 IIHTT 研究中，与安慰剂加饮食治疗相比，乙酰唑胺加饮食治疗能显著改善视野功能、视乳头水肿等级、生活质量指标和脑脊液 (CSF) 压力升高。

据专家共识，建议治疗 IIH 轻度视力丧失的患者时，应使用乙酰唑胺的最大耐受剂量。[63]Lubow M, Kuhr L. Pseudotumor cerebri: comments on practical management. In: Glaser JS, Smith JL, eds. Neuro-ophthalmology, Vol. IX. St Louis, MO: CV Mosby; 1976:199-206.[64]Tomsak RL, Niffenegger AS, Remler BF. Treatment of pseudotumor cerebri with Diamox (acetazolamide). J Clin Neuroophthalmol. 1988;8:93-8. 因为唯一可用的数据显示，直到乙酰唑胺用量达到每日 3000-4000 mg 时，脑脊液生成量才会减少，[114]Gucer G, Vierenstein L. Long-term intracranial pressure recording in management of pseudotumor cerebri. J Neurosurg. 1978 Aug;49(2):256-63.http://www.ncbi.nlm.nih.gov/pubmed/671078?tool=bestpractice.com 所以专家建议使用最大耐受剂量，但起始剂量应较低。磺胺过敏者可以安全地使用乙酰唑胺。[65]Lee AG, Anderson R, Kardon RH, Wall M. Presumed "sulfa allergy" in patients with intracranial hypertension treated with acetazolamide or furosemide: cross-reactivity, myth or reality? Am J Ophthalmol. 2004 Jul;138(1):114-8.http://www.ncbi.nlm.nih.gov/pubmed/15234289?tool=bestpractice.com

前三个月后，孕妇与其他患者的治疗方法相同。[9]Digre KB, Corbett JJ. Pseudotumor cerebri in men. Arch Neurol. 1988;45:866-872.http://www.ncbi.nlm.nih.gov/pubmed/3395260?tool=bestpractice.com 因可能发生神经管缺陷，前三个月内应避免使用乙酰唑胺。 罹患 IIH 的孕妇其自然流产率不会增加。[101]Tang RA, Dorotheo EU, Schiffman JS, et al. Medical and surgical management of idiopathic intracranial hypertension in pregnancy. Curr Neurol Neurosci Rep. 2004 Sep;4(5):398-409.http://www.ncbi.nlm.nih.gov/pubmed/15324607?tool=bestpractice.com

可将乙酰唑胺治疗改为呋塞米或托吡酯治疗，这两种药物均可作为二线治疗药。[38]Corbett JJ. The 1982 Silversides lecture: problems in the diagnosis and treatment of pseudotumor cerebri. Can J Neurol Sci. 1983 Nov;10(4):221-9.http://www.ncbi.nlm.nih.gov/pubmed/6652584?tool=bestpractice.com[72]Pollay M, Fullenwider C, Roberts PA, et al. Effect of mannitol and furosemide on blood-brain osmotic gradient and intracranial pressure. J Neurosurg. 1983 Dec;59(6):945-50.http://www.ncbi.nlm.nih.gov/pubmed/6415245?tool=bestpractice.com[73]Roberts PA, Pollay M, Engles C, et al. Effect on intracranial pressure of furosemide combined with varying doses and administration rates of mannitol. J Neurosurg. 1987 Mar;66(3):440-6.http://www.ncbi.nlm.nih.gov/pubmed/3102698?tool=bestpractice.com[74]Vogh BP, Langham MR Jr. The effect of furosemide and bumetanide on cerebrospinal fluid formation. Brain Res. 1981 Sep 21;221(1):171-83.http://www.ncbi.nlm.nih.gov/pubmed/6791768?tool=bestpractice.com 对于呋塞米，其剂量可逐渐增加，直到达到期望的效果。建议密切监测血清中钾的水平。难治性疾病可同时使用乙酰唑胺和呋塞米，但同样需要密切监测血钾水平。[76]Schoeman JF. Childhood pseudotumor cerebri: clinical and intracranial pressure response to acetazolamide and furosemide treatment in a case series. J Child Neurol. 1994 Apr;9(2):130-4.http://www.ncbi.nlm.nih.gov/pubmed/8006361?tool=bestpractice.com[77]Schoeman J, Donald P, van Zyl L, et al. Tuberculous hydrocephalus: comparison of different treatments with regard to ICP, ventricular size and clinical outcome. Dev Med Child Neurol. 1991 May;33(5):396-405.http://www.ncbi.nlm.nih.gov/pubmed/2065826?tool=bestpractice.com

托吡酯的疗效与乙酰唑胺类似。[78]Celebisoy N, Gokcay F, Sirin H, et al. Treatment of idiopathic intracranial hypertension: topiramate vs acetazolamide, an open-label study. Acta Neurol Scand. 2007 Nov;116(5):322-7.http://www.ncbi.nlm.nih.gov/pubmed/17922725?tool=bestpractice.com 它对预防偏头痛有效，还能抑制碳酸酐酶。

针对经选择患者组全部患者的治疗推荐

即使服用了药物减轻脑脊液 (CSF) 压力，特发性颅内压增高症 (IIH) 患者的头痛症状仍经常是一个主要的治疗问题。

虽然三环抗抑郁药（例如阿米替林）能有效治疗与 IIH 有关的头痛，但可能会导致体重增加的不良反应；因此，建议低剂量使用。 该药还会导致尿潴留、口干和镇静，但低剂量使用时，这几乎不是显著的问题。

非甾体抗炎药 (NSAID) 也可用于治疗头痛，特别是萘普生，但应强烈建议患者每周只使用 2 天以防止头痛反弹。

有偏头痛、镇痛药反弹性或咖啡因反弹性头痛病史的患者可能需要静脉注射双氢麦角胺，并联合使用胃复安来治疗这些恼人的头痛症状。 为最大程度降低神经系统反应和其他不良反应的风险，甲氧氯普胺最多使用 5 天。[79]European Medicines Agency. European Medicines Agency recommends changes to the use of metoclopramide. July 2013 [internet publication].http://www.ema.europa.eu/ema/index.jsp?curl=pages/news_and_events/news/2013/07/news_detail_001854.jsp

多种不同的分流手术已被用于治疗顽固性头痛。 专家共识是，立体定向脑室腹膜分流术如果可用，则是最佳选择。[80]Eggenberger ER, Miller NR, Vitale S. Lumboperitoneal shunt for the treatment of pseudotumor cerebri. Neurology. 1996 Jun;46(6):1524-30.http://www.ncbi.nlm.nih.gov/pubmed/8649541?tool=bestpractice.com[81]Rosenberg M, Smith C, Beck R, et al. The efficacy of shunting procedures in pseudotumor cerebri (abstract). Neurology. 1989;39(suppl 1):S209.[82]Johnston I, Besser M, Morgan MK. Cerebrospinal fluid diversion in the treatment of benign intracranial hypertension. J Neurosurg. 1988 Aug;69(2):195-202.http://www.ncbi.nlm.nih.gov/pubmed/3392566?tool=bestpractice.com[83]Burgett RA, Purvin VA, Kawasaki A. Lumboperitoneal shunting for pseudotumor cerebri. Neurology. 1997 Sep;49(3):734-9.http://www.ncbi.nlm.nih.gov/pubmed/9305333?tool=bestpractice.com证据 C症状消退：低质量证据表明，脑脊液 (CSF) 分流术能改善症状，尤其是当患者的主要问题是剧烈头痛时。[80]Eggenberger ER, Miller NR, Vitale S. Lumboperitoneal shunt for the treatment of pseudotumor cerebri. Neurology. 1996 Jun;46(6):1524-30.http://www.ncbi.nlm.nih.gov/pubmed/8649541?tool=bestpractice.com[81]Rosenberg M, Smith C, Beck R, et al. The efficacy of shunting procedures in pseudotumor cerebri (abstract). Neurology. 1989;39(suppl 1):S209.[82]Johnston I, Besser M, Morgan MK. Cerebrospinal fluid diversion in the treatment of benign intracranial hypertension. J Neurosurg. 1988 Aug;69(2):195-202.http://www.ncbi.nlm.nih.gov/pubmed/3392566?tool=bestpractice.com[83]Burgett RA, Purvin VA, Kawasaki A. Lumboperitoneal shunting for pseudotumor cerebri. Neurology. 1997 Sep;49(3):734-9.http://www.ncbi.nlm.nih.gov/pubmed/9305333?tool=bestpractice.com低质量的观察性（队列）研究或者受试者＜200名且方法学存在缺陷的随机对照临床试验（RCT）。

虽然分流是一个可行的治疗方法，尤其是对于以严重头痛为主要问题的患者，但分流也只能缓解约半数患者的长期头痛症状。[84]McGirt MJ, Woodworth G, Thomas G, et al. Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache: predictors of treatment response and an analysis of long-term outcomes. J Neurosurg. 2004 Oct;101(4):627-32.http://www.ncbi.nlm.nih.gov/pubmed/15481717?tool=bestpractice.com 重要的是不要忽视这些患者的镇痛药或咖啡因反弹性头痛。

孕妇可在局麻下完成手术，因此不需要延迟手术。

最常见的并发症是分流闭塞和颅内低压。 分流障碍可伴发严重视力丧失（视野平均偏差 ＞ 5 dB）。

如果在全面的内科治疗后，患者仍出现视力缺失，则可进行视神经鞘减压术或脑脊液 (CSF) 分流术等外科治疗。大型的病例研究已证明了这两种技术的有效性。[85]Goh KY, Schatz NJ, Glaser JS. Optic nerve sheath fenestration for pseudotumor cerebri. J Neuroophthalmol. 1997 Jun;17(2):86-91.http://www.ncbi.nlm.nih.gov/pubmed/9176777?tool=bestpractice.com[86]Acheson JF, Green WT, Sanders MD. Optic nerve sheath decompression for the treatment of visual failure in chronic raised intracranial pressure. J Neurol Neurosurg Psychiatry. 1994 Nov;57(11):1426-9.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1073203/pdf/jnnpsyc00041-0124.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/7964827?tool=bestpractice.com[87]Kelman SE, Heaps R, Wolf A, et al. Optic nerve decompression surgery improves visual function in patients with pseudotumor cerebri. Neurosurgery. 1992 Mar;30(3):391-5.http://www.ncbi.nlm.nih.gov/pubmed/1620303?tool=bestpractice.com[88]Sergott RC, Savino PJ, Bosley TM. Modified optic nerve sheath decompression provides long-term visual improvement for pseudotumor cerebri. Arch Ophthalmol. 1988 Oct;106(10):1384-90.http://www.ncbi.nlm.nih.gov/pubmed/3178549?tool=bestpractice.com[89]Corbett JJ, Nerad JA, Tse DT, et al. Results of optic nerve sheath fenestration for pseudotumor cerebri: the lateral orbitotomy approach. Arch Ophthalmol. 1988 Oct;106(10):1391-7.http://www.ncbi.nlm.nih.gov/pubmed/3273487?tool=bestpractice.com[90]Plotnik JL, Kosmorsky GS. Operative complications of optic nerve sheath decompression. Ophthalmology. 1993 May;100(5):683-90.http://www.ncbi.nlm.nih.gov/pubmed/8493011?tool=bestpractice.com[91]Brourman ND, Spoor TC, Ramocki JM. Optic nerve sheath decompression for pseudotumor cerebri. Arch Ophthalmol. 1988 Oct;106(10):1378-83.http://www.ncbi.nlm.nih.gov/pubmed/3178548?tool=bestpractice.com[92]Rizzo JF 3rd, Lessell S. Choroidal infarction after optic nerve sheath fenestration. Ophthalmology. 1994 Sep;101(9):1622-6.http://www.ncbi.nlm.nih.gov/pubmed/8090466?tool=bestpractice.com[93]Spoor TC, McHenry JG. Long-term effectiveness of optic nerve sheath decompression for pseudotumor cerebri. Arch Ophthalmol. 1993 May;111(5):632-5.http://www.ncbi.nlm.nih.gov/pubmed/8489443?tool=bestpractice.com[94]Fonseca PL, Rigamonti D, Miller NR, et al. Visual outcomes of surgical intervention for pseudotumour cerebri: optic nerve sheath fenestration versus cerebrospinal fluid diversion. Br J Ophthalmol. 2014 Oct;98(10):1360-3.http://www.ncbi.nlm.nih.gov/pubmed/24820047?tool=bestpractice.com[95]Huang LC, Winter TW, Herro AM, et al. Ventriculoperitoneal shunt as a treatment of visual loss in idiopathic intracranial hypertension. J Neuroophthalmol. 2014 Sep;34(3):223-8.http://www.ncbi.nlm.nih.gov/pubmed/24637911?tool=bestpractice.com[96]Niotakis G, Grigoratos D, Chandler C, et al. CSF diversion in refractory idiopathic intracranial hypertension: single-centre experience and review of efficacy. Childs Nerv Syst. 2013 Feb;29(2):263-7.http://www.ncbi.nlm.nih.gov/pubmed/22918619?tool=bestpractice.com[97]Rizzo JL, Lam KV, Wall M, et al. Perimetry, retinal nerve fiber layer thickness and papilledema grade after cerebrospinal fluid shunting in patients with idiopathic intracranial hypertension. J Neuroophthalmol. 2015 Mar;35(1):22-5.http://www.ncbi.nlm.nih.gov/pubmed/25295682?tool=bestpractice.com证据 B术后视敏度：中等质量证据表明，尽管围手术期可能会出现视力丧失，视神经纤维髓鞘开窗术能改善视敏度或视野。[85]Goh KY, Schatz NJ, Glaser JS. Optic nerve sheath fenestration for pseudotumor cerebri. J Neuroophthalmol. 1997 Jun;17(2):86-91.http://www.ncbi.nlm.nih.gov/pubmed/9176777?tool=bestpractice.com[86]Acheson JF, Green WT, Sanders MD. Optic nerve sheath decompression for the treatment of visual failure in chronic raised intracranial pressure. J Neurol Neurosurg Psychiatry. 1994 Nov;57(11):1426-9.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1073203/pdf/jnnpsyc00041-0124.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/7964827?tool=bestpractice.com[87]Kelman SE, Heaps R, Wolf A, et al. Optic nerve decompression surgery improves visual function in patients with pseudotumor cerebri. Neurosurgery. 1992 Mar;30(3):391-5.http://www.ncbi.nlm.nih.gov/pubmed/1620303?tool=bestpractice.com[88]Sergott RC, Savino PJ, Bosley TM. Modified optic nerve sheath decompression provides long-term visual improvement for pseudotumor cerebri. Arch Ophthalmol. 1988 Oct;106(10):1384-90.http://www.ncbi.nlm.nih.gov/pubmed/3178549?tool=bestpractice.com[89]Corbett JJ, Nerad JA, Tse DT, et al. Results of optic nerve sheath fenestration for pseudotumor cerebri: the lateral orbitotomy approach. Arch Ophthalmol. 1988 Oct;106(10):1391-7.http://www.ncbi.nlm.nih.gov/pubmed/3273487?tool=bestpractice.com[90]Plotnik JL, Kosmorsky GS. Operative complications of optic nerve sheath decompression. Ophthalmology. 1993 May;100(5):683-90.http://www.ncbi.nlm.nih.gov/pubmed/8493011?tool=bestpractice.com[91]Brourman ND, Spoor TC, Ramocki JM. Optic nerve sheath decompression for pseudotumor cerebri. Arch Ophthalmol. 1988 Oct;106(10):1378-83.http://www.ncbi.nlm.nih.gov/pubmed/3178548?tool=bestpractice.com[92]Rizzo JF 3rd, Lessell S. Choroidal infarction after optic nerve sheath fenestration. Ophthalmology. 1994 Sep;101(9):1622-6.http://www.ncbi.nlm.nih.gov/pubmed/8090466?tool=bestpractice.com[93]Spoor TC, McHenry JG. Long-term effectiveness of optic nerve sheath decompression for pseudotumor cerebri. Arch Ophthalmol. 1993 May;111(5):632-5.http://www.ncbi.nlm.nih.gov/pubmed/8489443?tool=bestpractice.com受试者＜200名的随机对照临床试验（RCT）、受试者＞200名且方法学存在缺陷的随机对照临床试验（RCT）、方法学存在缺陷的系统评价或者高质量的观察性（队列）研究。

视神经纤维髓鞘开窗术的临床过程有待进一步研究，因为 meta 分析的回顾性病例表明，术后约 10% 患者仍会出现视力丧失；[98]Wall M, Johnson CA, Kutzko KE, et al. Long- and short-term variability of automated perimetry results in patients with optic neuritis and healthy subjects. Arch Ophthalmol. 1998 Jan;116(1):53-61.http://www.ncbi.nlm.nih.gov/pubmed/9445208?tool=bestpractice.com 与脑脊液分流术后视力丧失的发生率相似。[1]Wall M. Idiopathic intracranial hypertension. Neurol Clin. 2010;28:593-617.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2908600/http://www.ncbi.nlm.nih.gov/pubmed/20637991?tool=bestpractice.com 需重新进行开窗术的晚期恶化并不常见。 对孕妇的手术可在局麻下完成，因此手术不需要推迟。

视神经纤维髓鞘开窗术通常会产生立竿见影的效果，几个月内视力就会有所改善。 通常会对视力较差的眼睛进行手术，术后，未经手术的眼睛视力也会恢复，视神经乳头水肿会减轻；非手术眼的改善可能持续数月甚至数年。 并发症包括围手术期视力缺失、眼球运动障碍和强直性瞳孔。

第六颅神经麻痹而导致复视时，可使用棱镜。

针对经选择患者组全部患者的治疗推荐

非甾体抗炎药 (NSAID) 可用于颈部疼痛。

应限制非甾体抗炎药的使用，如果伴随头痛症状，每周只能用 2 天。

虽然脉冲同步耳鸣通常对减肥和药物治疗有反应，但在出现颅内压增高相关性横静脉窦狭窄时，可考虑进行横窦支架置入术。