Version May 2024
INTRODUCTION — Idiopathic intracranial hypertension (IIH) is also called pseudotumor cerebri. It is a disorder defined by clinical criteria that include symptoms and signs isolated to those produced by increased intracranial pressure (ICP; eg, headache, papilledema, double vision, transient visual obscurations, and vision loss), elevated ICP with normal cerebrospinal fluid (CSF) composition, and no other cause of intracranial hypertension evident on neuroimaging or other evaluations [1].
While once called "benign" intracranial hypertension to distinguish it from secondary intracranial hypertension produced by a neoplasm, it is not a benign disorder. Many patients suffer from intractable, disabling headaches, and there is a risk of severe, permanent vision loss in approximately 5 to 15 percent of patients.
Recommendations for the treatment of IIH are limited by only a few randomized controlled trials [2,3]. In addition, the natural history of IIH is variable, with some patients resolving within months, while in others, the condition is chronic or recurrent.
This topic will discuss the prognosis and treatment of IIH. The epidemiology, pathogenesis, clinical features, and diagnosis of this disorder are discussed separately. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Epidemiology and pathogenesis" and "Idiopathic intracranial hypertension (pseudotumor cerebri): Clinical features and diagnosis".)
TREATMENT GOALS — The treatment of patients with IIH has two major goals: the alleviation of symptoms (usually headache) and the preservation of vision.
IDENTIFYING PATIENTS AT RISK FOR VISION LOSS
Risk factors — Studies have attempted to identify patients at risk of severe, permanent vision loss. Features that appear to identify such patients include:
●Severe papilledema – IIH patients with higher-grade papilledema, Frisén grades 3 to 5 (table 1 and picture 1A-F), have poor visual outcomes if not treated aggressively [4,5]. Absence of papilledema appears to identify patients at low risk of vision loss [5]. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Clinical features and diagnosis", section on 'Papilledema'.)
●Vision loss and visual symptoms – Significant vision loss (ie, vision loss that is noticeable to the patient) at presentation suggests a higher risk, while transient visual obscurations likely suggest an intermediate risk [4-8].
In addition, patients who develop such symptoms despite treatment are at risk and may be appropriate for more aggressive interventions. (See 'Interventions for severe or refractory disease' below.)
Other risk factors more variably identified in studies include male sex, systemic hypertension, anemia, younger age or onset in puberty, more severe obesity or recent weight gain, and higher opening pressure on lumbar puncture [6,9-14].
The presence or absence of findings on magnetic resonance imaging does not appear to predict visual outcomes [15].
Fulminant disease — A subset of individuals with IIH have a more malignant or fulminant course with rapid development of vision loss within a few weeks of symptom onset [16-18]. This is generally apparent at presentation. Such patients present with a rapid progression of symptoms, severe papilledema (grade 3 or worse), substantial visual field and/or visual acuity loss, and/or more than 30 transient visual obscurations per month [4].
In these patients, more aggressive interventions to limit the severity of permanent vision loss are considered at the outset. Urgent temporizing measures may be employed as well. In one series, 13 of 79 patients presented with severe vision loss (visual field mean deviation of -7 decibels [dB] or worse); over half of such patients required surgical intervention [8]. (See 'Interventions for severe or refractory disease' below and 'Urgent temporizing measures' below.)
MONITORING DURING TREATMENT — Patients require regular ophthalmology follow-up visits until they stabilize. Follow-up visit intervals are individualized based on the severity, duration, and response to treatment of the clinical manifestations, but initially should be at least monthly in patients with moderate symptoms. Patients with chronic low-grade papilledema (grade 1) seldom worsen quickly and can be followed less frequently [5].
Each office visit should include a best corrected visual acuity, formal visual field testing, dilated fundus examination with optic disc photographs, and often optical coherence tomography (OCT) of the optic nerve, retinal nerve fiber layer, and macular ganglion cell layer [6]. Worsening vision is an indication for intensifying treatment.
INITIAL TREATMENT FOR MOST PATIENTS — Some patients with normal vision and minimal symptoms require no treatment other than monitoring; most others will require risk factor modification and medications (algorithm 1).
Address risk factors and comorbid conditions — Any potential agents that might cause or worsen IIH (eg, tetracycline derivatives) should be discontinued. However, this intervention alone may not be sufficient to manage IIH. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Epidemiology and pathogenesis", section on 'Medications'.)
Similarly, patients should be questioned regarding symptoms of sleep apnea; diagnostic polysomnography and treatment of sleep apnea should follow where appropriate. Since during apneic periods cerebrospinal fluid (CSF) pressure increases [19], obstructive sleep apnea appears to be an aggravating factor in patients with IIH [20]. (See "Clinical presentation and diagnosis of obstructive sleep apnea in adults".)
Weight loss — A low-sodium weight reduction program, ideally in conjunction with a nutritionist or dietician, is recommended for all patients with obesity and IIH. An approach using motivational interviewing may be especially helpful. Weight loss appears to alleviate symptoms and signs in many but not all patients [3]. Because weight loss is difficult for many to achieve and also takes time to achieve, other treatments for IIH are required at the same time when there is visual loss present.
However, studies documenting benefit are limited to observations in patients who generally receive treatment with other interventions for IIH. These studies typically report that weight loss is associated with reductions in intracranial pressure (ICP) and/or papilledema [5,21-25]. As an example, in the IIH treatment trial (IIHTT), all patients were recommended a low-sodium weight reduction diet and lifestyle management, which were associated with a 6 percent weight loss and mean CSF pressure reduction of 50 mm water [5]. A smaller number of other studies report improvement in either symptoms or visual fields [21,23]; however, no study has shown an association with vision outcomes.
Medically supervised weight loss programs or surgically induced weight reduction (eg, gastric banding or gastric bypass procedures) may be necessary in patients with severe obesity. Case series of patients with IIH undergoing gastric surgery report improvement of IIH symptoms and signs including papilledema, headache, tinnitus, and CSF pressure [26-30]. These findings were observed over one to three years after surgery and were associated with mean weight loss of 45 to 58 kg. (See "Bariatric surgery for management of obesity: Indications and preoperative preparation".)
Weight gain may be a risk factor for recurrence of IIH. (See 'Recurrence and long-term monitoring' below.)
Weight loss also has other health benefits. (See "Obesity in adults: Overview of management", section on 'Importance of weight loss'.)
Carbonic anhydrase inhibitors
Acetazolamide — For initial treatment of patients with IIH who have vision loss and/or vision symptoms, we suggest the carbonic anhydrase inhibitor acetazolamide (algorithm 1).
Carbonic anhydrase inhibitors are believed to reduce the rate of CSF production and have been associated with modestly improved outcomes in patients with IIH [5,31,32].
In a randomized trial of 165 patients with IIH and mild visual loss (the IIHTT), treatment with acetazolamide was associated with modest improvements in a perimetric measurement of global visual field loss, along with improvements in papilledema grade, CSF pressure, and vision-related quality of life at six months [5]. More patients in the placebo group had poor visual outcome compared with those in the acetazolamide treatment group (six versus one).
Other observational evidence supports the use of acetazolamide. Lowered CSF pressure with acetazolamide has been documented in patients with IIH who underwent continuous ICP recordings [33]. Case series also suggest that in patients who can tolerate it, acetazolamide is successful in managing symptoms and stabilizing vision in 47 to 67 percent of patients [34-36]. In addition, in a long-term follow-up study of 54 patients, the average treatment duration for acetazolamide was 14 months [37]. While recurrent episodes of IIH occurred in 38 percent over a mean of 6.2 years of follow-up, no recurrences occurred in the setting of ongoing acetazolamide treatment.
●Dosing and administration – In adult patients, we usually start with 500 mg twice per day and advance the dose as required up to 2 to 4 grams per day, as tolerated by the patient. The titration can be stopped if the desired outcome is reached. While many patients cannot tolerate more than 2 grams per day, 40 percent of subjects in the IIHTT reached the dose of 4 grams per day and tolerated it well, that is, it did not interfere with activities of daily living. In young children, the recommended starting dose is 25 mg/kg per day with a maximum dose of 100 mg/kg or 2 grams per day [34]. The extended-release formulation (Diamox sequels) may be better tolerated by patients who are intolerant of immediate-release acetazolamide but may be more expensive.
●Potential contraindications – Although a sulfa allergy is reported to be a relative contraindication to acetazolamide use, there is little clinical or pharmacologic basis for this recommendation. Nonetheless, it remains a concern for many clinicians. A true cross-reaction between sulfonamide antimicrobials and the sulfa moiety in acetazolamide and furosemide is unlikely [38]. Before prescribing acetazolamide to patients who report a sulfa allergy, we discuss the risks and benefits of treatment considering the nature of the adverse reaction composing the reported sulfa allergy.
•For patients who have had major adverse reactions (eg, Stevens-Johnson syndrome, anaphylaxis), we usually consider that the risk precludes the use of acetazolamide. Allergy consultation or other desensitization protocols may be considered in these patients, but we have not employed these. (See "Sulfonamide allergy in HIV-uninfected patients".)
•However, if the previous sulfa-related reaction was minor, we usually proceed with acetazolamide treatment. We treated 27 patients who had a reported sulfa allergy (excluding those that reported a severe adverse reaction) with acetazolamide; only two patients suffered urticaria, and there were no severe allergic cross-reactions to sulfa [38]. Another case series documents uneventful administration of acetazolamide to three patients with reported sulfa allergy [39].
Pregnancy, particularly the first 20 weeks, is often considered a relative contraindication to the use of acetazolamide, although limited information on safety is available. Teratogenic effects have been reported with high doses in animals, and a single case of a teratoma was seen in humans [40,41]. If we consider that the benefit of acetazolamide use outweighs potential risks, we do use it in pregnant patients after discussion with the patient and the obstetrician and after obtaining informed consent. Treatment options are somewhat limited in pregnancy as caloric restriction and the use of other diuretics are also relatively contraindicated during pregnancy [42-44].
●Adverse effects – Medication side effects of acetazolamide include digital and oral paresthesias, anorexia, malaise, metallic taste, fatigue, nausea, vomiting, electrolyte changes, mild metabolic acidosis, and kidney stones. These are usually dose related. While many patients in the IIHTT experienced side effects of acetazolamide, quality-of-life measures were still higher in patients who received acetazolamide [5]. The IIHTT found monitoring of electrolytes during acetazolamide treatment is not necessary if acetazolamide is the only diuretic used.
For patients who are unable to tolerate acetazolamide, furosemide is an alternative.
Topiramate and other agents — Topiramate is an antiseizure medication that inhibits carbonic anhydrase activity. Its efficacy in the treatment of migraine headaches and its association with weight loss are features that make it an attractive potential therapeutic option in IIH. Case reports and one small unblinded study suggest that topiramate appears to have a similar efficacy to acetazolamide with regard to visual field improvement and symptom relief [45-49].
Other carbonic anhydrase inhibitors, such as methazolamide (Neptazane), can also be used in acetazolamide-intolerant patients. We occasionally have had apparent success with these agents.
Adjunctive treatments
Loop diuretics — For patients with persistent or worsening visual symptoms despite maximizing treatment with acetazolamide, we suggest the addition of furosemide (20 to 40 mg per day for adults and 1 to 2 mg/kg per day in children) (algorithm 1). [34]. The evidence supporting its use in IIH is anecdotal. In one report of eight children treated with combined therapy of acetazolamide and furosemide, all had a rapid clinical response with resolution of papilledema, reduction in the mean CSF pressure after the first week of treatment, and normalization of CSF pressure within six weeks of starting therapy [50]. In our experience, furosemide can be a useful agent, preferably as an adjunct to acetazolamide and sometimes as an alternative to acetazolamide.
The same considerations discussed above regarding sulfa allergy and acetazolamide also apply to furosemide. We, after discussion of risk and benefits with patients, use furosemide in patients whose prior reactions have not included Stevens-Johnson syndrome, anaphylaxis, or other severe reactions. Among 21 such patients treated with furosemide, no allergic reactions were noted [38].
Headache prophylaxis — Patients with IIH can continue to have headaches despite improvement in papilledema and visual function. Medications used in the prophylactic treatment of migraine headaches are often used for headache management in IIH if other treatments described above are not effective in this regard [51]. Many patients with headaches and IIH have migraine or another headache syndrome that is not directly related to increased ICP. These syndromes should be identified early and treated, as they may persist following otherwise successful IIH treatment. The choice of agent is influenced by the propensity of some of these medications (eg, valproate, tricyclic antidepressants) to produce weight gain; however, weight gain can be mitigated by use of low doses and careful monitoring of weight. (See "Preventive treatment of episodic migraine in adults".)
Analgesic overuse or rebound headaches may be common in patients with IIH [51-53]. Efforts should be made to avoid and treat this condition. (See "Medication overuse headache: Etiology, clinical features, and diagnosis" and "Medication overuse headache: Treatment and prognosis".)
URGENT TEMPORIZING MEASURES — Rare patients present with or develop rapidly progressive vision loss (ie, fulminant IIH). (See 'Fulminant disease' above.)
Treatment is urgent to preserve vision:
●Medical therapy – Acetazolamide should be initiated promptly and can be rapidly titrated up to 4 grams per day divided into two doses. (See 'Acetazolamide' above.)
●Consultation for intervention – We refer such patients urgently for one of the interventions listed below. (See 'Interventions for severe or refractory disease' below.)
In addition, short-term temporizing measures can be employed in such patients until surgery can be performed [54]:
●Glucocorticoids – In the setting of acute visual loss, a short course of intravenous glucocorticoids may be useful as a temporizing measure prior to surgical intervention in fulminant IIH.
One case series describes successful use of methylprednisolone (250 mg four times a day for five days followed by an oral taper) in conjunction with acetazolamide in four patients with IIH and severe, acute visual loss [17]. We do not use glucocorticoids long term, as their use is complicated by rebound increased intracranial pressure (ICP). (See 'Treatments with a limited role' below.)
●Serial lumbar punctures – Serial lumbar punctures or lumbar drainage can be a useful temporizing measure as a prelude to surgery [43,44,55]. However, these are not useful for long-term management of IIH in most patients. (See 'Treatments with a limited role' below.)
INTERVENTIONS FOR SEVERE OR REFRACTORY DISEASE
Indications for intervention — Patients with IIH who appear to benefit from surgical intervention include those who fail, are intolerant to, or are noncompliant with maximum medical therapy (algorithm 1). They have intractable headache and/or progressive visual loss. Such patients are the minority, less than 10 percent in one center, of patients treated for IIH [56]. Potential indications for surgical therapy include [57]:
●Worsening visual field defect despite medical therapy
●Presence of visual acuity loss attributed to papilledema (ie, not due to serous detachment, macular edema, hemorrhage, or choroidal folds)
While deteriorating vision (as suggested by an abnormal visual acuity or worsening visual field deficits) is a universally accepted indication for surgical intervention, other potential considerations need to be balanced against the inherent risks of the surgical procedures and the uncertainties regarding their efficacy. Intractable headache may be an indication for surgical treatment, but most chronic headaches in IIH are from migraine, medication overuse, caffeine overuse, or other non-intracranial pressure (ICP)-related headaches.
Choice of procedure — The two main surgical procedures in IIH are optic nerve sheath fenestration (ONSF) and cerebrospinal fluid (CSF) shunting procedures. Cerebral venous sinus stenting is an alternative intervention for IIH. These treatments have not been compared in a single population. Indirect comparisons of their efficacy in case series are challenged by different indications considered for intervention and different outcome measures used [56].
Clinicians should choose the surgical procedure based on local expertise. While several papers discuss the relative benefits of ONSF versus CSF shunting, an individualized approach based upon availability of surgeons and procedures and clinician preference is recommended [56,58,59]. The overall rate of visual improvement seems to be equivalent across all of the surgical treatment modalities, and there is insufficient evidence to recommend or reject any one surgical modality over another at this time [59,60].
Some patients require both shunting and ONSF.
Optic nerve sheath fenestration — ONSF appears to be an effective procedure in patients who have progressive vision loss despite medical therapy. It is usually performed using a medial orbital approach. The optic nerve sheath is identified and a window is cut in this sheath, allowing CSF egress into the orbit. ONSF can stabilize or improve visual loss due to papilledema in IIH [3,61-72]. In general, ONSF is an outpatient procedure, although it typically involves general anesthesia. The recovery time is typically same day.
●Efficacy – In one of the largest published case series summarizing 158 operations in 86 patients, visual acuity stabilized or improved in 94 percent, while visual fields stabilized or improved in 88 percent [72]. These results are consistent with aggregated data from other smaller case series [56,61-65,68-71,73]. Patients with a less severe and a shorter duration of visual abnormality have better visual outcomes after ONSF [74,75].
Preservation of vision is the primary goal of ONSF. While some patients experience headache relief after ONSF, many do not [61,72]. ONSF has been associated with improvement in patients with deteriorating vision loss despite a working shunt [66,68,72]. ONSF also appears to be safe and effective in children [67,76].
●Adverse effects – Complications of ONSF occur in as much as 40 to 45 percent of patients [70,72,77,78]. Most but not all of these are transient and nondisabling. The more common complications include:
•Temporary diplopia (due to injury to extraocular muscle, nerve, or blood supply) in 29 to 35 percent [62,70,72].
•Efferent pupillary dysfunction from ciliary ganglion damage can be seen after ONSF in up to 11 percent [62,70,72,75].
•Vision loss in up to 11 percent [70]. This is usually transient but can be catastrophic and permanent in 1.5 to 2.6 percent [68,70]. Vision loss can result from vascular complications (central retinal or branch artery occlusion, choroidal infarction), trauma (eg, operative traction), infectious optic neuritis, orbital hematoma, hemorrhage into the optic nerve sheath causing compressive hematoma, and other operative events [65,72,75,77-79].
●Relapse – ONSF may fail after initial benefit, requiring repeat surgery in 7 to 32 percent of eyes, depending in part on the duration of follow-up; failure may occur within months or after several years [64,69,72]. Repeat surgery appears to have a similar rate of benefit to the original [74].
Shunting — CSF shunting procedures include ventriculoperitoneal shunt (VPS) or lumboperitoneal shunt (LPS). At most centers, VPS is performed preferentially due to a higher complication rate with LPS.
●Efficacy – Uncontrolled observations report that shunt placement can relieve headache, diplopia, papilledema, and visual loss in patients with IIH [3,56,80-85]. However, reported outcomes are mixed; some report that 95 to 100 percent achieve stabilization or remission of visual problems [56,73,80-83], while others note that vision continued to worsen in approximately 10 percent. While headache relief appeared to occur in almost all patients soon after shunting, in one case series, the benefit was not universally sustained, with nearly half of patients having recurrent severe headaches within three years of surgery, despite a working shunt [86].
●Complications – Shunt failure requiring revision is the most common complication of LPS and occurs in approximately half of patients, with a few patients requiring multiple (10 to 38) shunt revisions [80-84,86]. In rare cases, shunt failure is accompanied by visual loss that can be rapid and severe [87]. Shunt failure in VPS appears less common, occurring in 6 of 32 (19 percent) in one series; however, reported rates vary considerably, in part depending on the duration of follow-up [88].
Other complications of shunting include shunt infection, abdominal pain, and overdrainage causing low pressure [80-84,89,90]. Rare complications include cerebellar tonsillar herniation and syringomyelia, subdural and subarachnoid hemorrhage, and bowel perforation [89,91,92].
More recently developed technologies using advanced imaging as well as endoscopic operative techniques have improved the ability of surgeons to place catheters in the ventricles of patients with IIH who do not have ventricular enlargement. Case series suggested that ventriculoperitoneal catheters may be less prone to shunt obstruction than LPS [86,93-95]. However, the risks for low pressure, infections, and other complications were similar to LPS, and requirements for shunt revision remained substantial at 40 to 60 percent.
Venous sinus stenting — Venous sinus stenting is a relatively new and somewhat controversial treatment option for IIH. Its use results from the observation that many patients with IIH have apparent stenoses of the transverse venous sinus or other cerebral veins, although whether this is a primary or secondary phenomenon is uncertain. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Epidemiology and pathogenesis", section on 'Intracranial venous hypertension'.)
A number of case series describe some centers' experience with venous stenting in patients with IIH who had apparent venous sinus obstruction on cerebral venography [96-99]. In a 2013 literature review that summarizes data on 143 patients treated with venous stenting, 88 percent of patients reported improvement of headache, and 87 percent had improved visual symptoms [100]. Subsequent studies report similar results [99,101]. However, among the individual series, the effects on vision were not consistently documented and follow-up was often limited. While the procedure was technically successful in 99 percent, 6 percent of patients had complications, including three patients with subdural hematomas requiring surgical decompression. Also, two patients were treated with thrombolytic therapy when an intraluminal thrombus was noted to develop postoperatively, despite anticoagulation treatment [97]. Other reported complications include retroperitoneal hemorrhage and contrast reactions; recurrent stenosis has also been reported [99,101].
We and others feel that further documentation of clinical benefit from venous stenting is required before this becomes a routine part of IIH treatment, but at some centers it has become a first-line consideration for IIH [3,102].
TREATMENTS WITH A LIMITED ROLE
●Glucocorticoids – While used in rare situations as a temporizing measure prior to surgical intervention, we and others avoid using glucocorticoids for long-term management of IIH [103] for the following reasons [57]:
•Glucocorticoids can cause weight gain that might worsen IIH.
•Steroid withdrawal can cause severe rebound intracranial hypertension associated with marked visual loss.
•There are significant systemic side effects from chronic glucocorticoids. (See "Major adverse effects of systemic glucocorticoids".)
●Serial lumbar punctures and lumbar drain – Although serial lumbar punctures have been advocated for the treatment of IIH, we suggest against this treatment for the following reasons [104]:
•Cerebrospinal fluid (CSF) reforms within six hours unless there is a CSF leak, making any treatment benefit of short-term duration only.
•Lumbar punctures are uncomfortable for most patients and painful for many.
•Lumbar punctures can produce complications (eg, low-pressure headaches, CSF leak, CSF infection, intraspinal epidermoid tumors).
•In patients with obesity, lumbar punctures are often technically difficult.
However, as an exception, serial lumbar punctures or a lumbar drain can be used in patients who are pregnant who wish to avoid medical therapy [43,44,55].
●Indomethacin – Some reports suggest that indomethacin may have efficacy in the treatment of secondary intracranial hypertension (eg, traumatic brain injury, hepatic encephalopathy), presumably by causing cerebral vasoconstriction and reducing cerebral blood flow [105]. In one report of seven patients with IIH, intravenous administration of 50 mg indomethacin was associated with prompt reduction in CSF pressure, and long-term treatment with 75 mg daily produced symptom relief and improvement in visual fields and papilledema grade [106]. Further study of this treatment is needed before it can be recommended for use in IIH.
●Iron – Iron supplementation in IIH patients with iron deficiency anemia appeared to be efficacious in a case series of six patients, and other reported cases suggest that treating anemia appears to be useful in some patients with IIH [107].
PROGNOSIS
Clinical course and outcomes — No large prospective series describe the natural history of IIH. A protracted course lasting months to years appears to be common [6,11,40,108,109]. In most patients, symptoms worsen slowly if untreated.
With treatment, there is usually gradual improvement and/or stabilization. As symptoms stabilize, acetazolamide can be tapered and withdrawn. Some patients have persistent papilledema, elevated intracranial pressure (ICP) as documented on lumbar puncture, and mild residual visual field deficits.
Permanent disabling vision loss is the major morbidity associated with IIH but is uncommon. While an early, hospital-based study found that 24 percent of 57 patients developed blindness or severe visual impairment [9], subsequent studies that have been outpatient based have found a lower rate of severe visual loss of 6 to 14 percent [7,10,110].
Risk factors for vision loss are described above, but also include noncompliance with medication. (See 'Risk factors' above.)
Recurrence and long-term monitoring — A recurrence of symptoms may occur in 8 to 38 percent of patients after recovery from an episode of IIH or after a prolonged period of stability [9,37,111,112]. Weight gain is a common but not universal antecedent to recurrent IIH [111,113]. The time interval may be many years [112].
Thus, patients should be followed for several years after recovery (algorithm 1):
●For patients with Frisén grade 2 papilledema or higher, we examine them every few to several months depending on the amount of visual loss and duration of stability.
●For patients with chronic Frisén grade 1 edema, we examine yearly or biyearly, as the risk of visual loss is low as long as they do not have an increase in their IIH symptoms.
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)
●Basics topics (see "Patient education: Idiopathic intracranial hypertension (pseudotumor cerebri) (The Basics)")
SUMMARY AND RECOMMENDATIONS
●Treatment goals – The major morbidity associated with idiopathic intracranial hypertension (IIH) is vision loss. As much as 15 percent of individuals with IIH may be at risk of severe, permanent vision loss. Persistent headaches are also a source of disability and decreased quality of life. (See 'Treatment goals' above.)
●Monitoring – Patients with IIH require regular follow-up visits with serial ophthalmology examinations until they stabilize. (See 'Monitoring during treatment' above.)
●Initial treatment – The approach to treatment is summarized in the algorithm (algorithm 1).
Agents that might cause or worsen IIH (eg, tetracycline derivatives) should be discontinued, and treatment provided as indicated for any potential comorbid conditions (eg, sleep apnea and anemia). (See 'Address risk factors and comorbid conditions' above.)
Patients with obesity and IIH should be counseled and/or referred to make lifestyle changes to increase fitness and promote weight loss of 6 to 10 percent of body weight. (See 'Weight loss' above and "Obesity in adults: Overview of management".)
For initial treatment of patients with IIH, we suggest treatment with the carbonic anhydrase inhibitor acetazolamide (Grade 2B). In one clinical trial, acetazolamide was associated with improvement of visual field function, papilledema grade, cerebrospinal fluid (CSF) pressure reduction, and vision-related quality-of-life measure at six months. Topiramate and other carbonic anhydrase inhibitors are alternatives to acetazolamide; experience with these agents is more limited. (See 'Carbonic anhydrase inhibitors' above.)
●Adjunctive treatments for persistent symptoms – Furosemide may provide an additional benefit to acetazolamide in patients who experience continuing symptoms on acetazolamide. (See 'Loop diuretics' above.)
Traditional headache therapies can be employed in symptomatic IIH patients. Analgesic rebound headache in particular is a common complication in IIH and should be prevented and treated as appropriate. (See 'Headache prophylaxis' above.)
●Fulminant disease – Rare patients present with rapid development of vision loss within a few weeks of symptom onset. (See 'Fulminant disease' above.)
Such patients require urgent initiation of acetazolamide and referral for surgical intervention. (See 'Interventions for severe or refractory disease' above.)
In such patients, we also suggest initiating a temporizing measure (eg, lumbar drain) until surgery is performed (Grade 2C). (See 'Urgent temporizing measures' above.)
●Refractory or worsening disease – For unusual patients with IIH and progressing visual loss despite medical therapy, we recommend surgical intervention with optic nerve sheath fenestration (ONSF) and/or a CSF shunting procedure (Grade 1B). The choice of surgical procedure is individualized based upon available expertise and patient preference. Patients who do not respond to one treatment may respond to the other. Neither intervention is a panacea; serious complications may occur. Failure and symptom recurrence are common. (See 'Interventions for severe or refractory disease' above.)
●Prognosis and follow-up – Most patients with IIH improve or stabilize with medical treatment over the course of several months. Recurrences may occur even after several years, necessitating ongoing monitoring. (See 'Prognosis' above.)
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