Materiały źródłowe

• #1 Pseudotumor cerebri: An update on treatment options

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278127/

  The aim was to identify Pseudotumor cerebri treatment options and assess their efficacy. […] Treatment options range from observation to surgical intervention. Weight loss and medical treatment may be utilized in cases without vision loss or in combination with surgical treatment. Cerebrospinal fluid shunting procedures and/or optic nerve sheath decompression is indicated for severe vision loss or headache unresponsive to medical management. The recent use of endovascular stenting of transverse sinus stenoses has also demonstrated benefit in patients with pseudotumor cerebri. […] While each treatment form may be successful individually, a multimodal approach is typically utilized with treatments selected on a case-by-case basis. […] Treatment of PTC ranges from observation to emergent surgery. A team approach amongst the patient’s neurologist, ophthalmologist, primary care physician, and neurosurgeon is essential.

• #1 Idiopathic intracranial hypertension: consensus guidelines on management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/89/10/1088

  The aim was to capture interdisciplinary expertise from a large group of clinicians, reflecting practice from across the UK and further, to inform subsequent development of a national consensus guidance for optimal management of idiopathic intracranial hypertension (IIH). […] Over 20 questions were constructed: one based on the diagnostic principles for optimal investigation of papilloedema and 21 for the management of IIH. Three main principles were identified: (1) to treat the underlying disease; (2) to protect the vision; and (3) to minimise the headache morbidity. Statements presented provide insight to uncertainties in IIH where research opportunities exist. […] For optimal management of patients with IIH, there must be clear communication between clinicians for seamless joint care between the various specialties. Weight loss reduces ICP and has been shown to be effective in improving papilloedema and headaches.28 The main principles of management of IIH are: (1) to treat the underlying disease; (2) to protect the vision; (3) to minimise the headache morbidity.

• #1 Idiopathic Intracranial Hypertension (IIH) Treatment & Management: Approach Considerations, Pharmacologic Therapy, Optic Nerve Sheath Fenestration, CSF Diversion, and Venous Sinus Stenting

  https://emedicine.medscape.com/article/1214410-treatment

  Management guidelines are based on symptoms and extent of visual impairment at presentation. If there is no immediate threat to vision, medical therapy is recommended. In the event of an immediate threat to visual function, a temporary CSF draining procedure (ie, placement of a lumbar drain) is immediately performed, and a definitive surgical plan is made; either a ventriculoperitoneal shunt or optic nerve sheath fenestration. […] The primary treatment objectives for patients with idiopathic intracranial hypertension (IIH) are to preserve optic nerve function, manage elevated intracranial pressure (ICP), and alleviate symptoms. […] The initial treatment typically involves administering acetazolamide, dosed according to the patient’s symptoms, tolerance, and visual function. If progressive visual field loss occurs despite maximal medical therapy, urgent surgical intervention may be necessary.

• #1 Idiopathic intracranial hypertension: consensus guidelines on management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/89/10/1088

  Weight loss is the only disease-modifying therapy in typical IIH.28 […] Where there is evidence of declining visual function, the acute management to preserve vision is surgical. […] The preferred surgical procedure is neurosurgical CSF diversion (see 5. What other surgical procedures are performed for visual loss IIH?). […] Acetazolamide could be prescribed for those with IIH symptoms. […] The current Cochrane review on IIH management reported on the use of acetazolamide, a carbonic anhydrase inhibitor, in IIH. It concluded: the two included RCTs showed modest benefits for acetazolamide for some outcomes, there is insufficient evidence to recommend or reject the efficacy of this intervention, or any other treatments currently available, for treating people with IIH. […] Serial lumbar punctures are not recommended for management of IIH. […] CSF diversion is generally not recommended as a treatment for headache alone in IIH.

• #1 Idiopathic intracranial hypertension (pseudotumor cerebri): recognition, treatment, and ongoing management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23136035/

  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. […] 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 5-10 % 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.

• #1 Intracranial Hypertension or Pseudotumor Cerebri: The Basics – Migraine Canada

  https://migrainecanada.org/intracranial-hypertension-or-pseudotumor-cerebri-the-basics/

  Idiopathic Intracranial Hypertension (IIH), also known as pseudotumor cerebri, is a condition where the pressure inside the skull increases without an obvious cause. […] Losing weight, even as little as 10% of body weight, can significantly improve symptoms and protect vision. Weight loss is challenging, but crucial for managing IIH. […] In severe cases where vision is at risk, surgical shunting may be considered. This involves placing a tube to drain fluid from the brain ventricles to the abdomen (ventriculo-peritoneal shunt) or from the spine to the abdomen (lumbo-peritoneal shunt). These procedures carry high risks and should only be performed by experts. […] Physicians may recommend treatments like topiramate and Botox for headache control in these patients. […] Acetazolamide is a diuretic that can improve vision but may not be as effective for headaches. Side effects include nausea, tingling in extremities, and fatigue.

• #1 Idiopathic Intracranial Hypertension (IIH) Treatment & Management: Approach Considerations, Pharmacologic Therapy, Optic Nerve Sheath Fenestration, CSF Diversion, and Venous Sinus Stenting

  https://emedicine.medscape.com/article/1214410-treatment

  Reduction of body weight by 5-10% was found to be effective with resulting improvement of papilledema and visual fields. […] In a literature review of bariatric surgery in 62 obese patients with IIH, Fridley et al found that 52 (92%) experienced resolution of the presenting symptoms. […] The authors called for prospective controlled studies to confirm the effectiveness of this surgical approach for patients with IIH in long-term follow-up.

• #1 Pseudotumor cerebri: An update on treatment options

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278127/

  The goals of treatment in an individual with PTC are twofold: Preservation of vision, and reduction of symptoms (usually headache). […] As obesity and/or recent weight gain are the main identifiable PTC risk factors in most patients, a defined weight loss program should be initiated regardless of disease severity. […] Medical treatment is indicated in the setting of good vision when a patient’s primary symptom is headache. Carbonic anhydrase inhibitors (CAI) are the treatment of choice, although no prospective data confirm their effectiveness. […] Initial surgical treatment of PTC is indicated when there is severe optic neuropathy, either acute or rapidly progressive, or medical treatment failure. CSF diversion (ventriculoperitoneal [VP], ventriculoatrial [VA], or lumboperitoneal [LP] shunting) and optic nerve sheath decompression (ONSD) are most commonly performed, with subtemporal decompression reserved for extreme cases.

• #1 Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment – UpToDate

  https://www.uptodate.com/contents/idiopathic-intracranial-hypertension-pseudotumor-cerebri-prognosis-and-treatment

  Idiopathic intracranial hypertension (IIH) was formerly called pseudotumor cerebri. […] Recommendations for the treatment of IIH are limited by only a few randomized controlled trials. […] The treatment of patients with IIH has two major goals: the alleviation of symptoms (usually headache) and the preservation of vision. […] For initial treatment of patients with IIH, we suggest treatment with the carbonic anhydrase inhibitor acetazolamide. […] Acetazolamide should be initiated promptly and can be rapidly titrated up to 4 grams per day divided into two doses. […] For patients with persistent or worsening visual symptoms despite maximizing treatment with acetazolamide, we suggest the addition of furosemide. […] Patients with IIH can continue to have headaches despite improvement in papilledema and visual function.

• #1 European Headache Federation guideline on idiopathic intracranial hypertension | The Journal of Headache and Pain | Full Text

  https://thejournalofheadacheandpain.biomedcentral.com/articles/10.1186/s10194-018-0919-2

  […] […] There is a clear association between IIH and weight with over 90-95% of patients being obese. Additionally, IIH is reported in the context of gaining 5-15% of body weight. Weight loss is the only established disease modifying therapy in IIH. Consequently, patients should be sensitively counselled about the importance of weight loss. However, the amount of weight loss required is not well established. Additionally, the optimal method of weight loss is uncertain. Dietary strategies are notoriously difficult to achieve and maintain in the long term. The role of bariatric surgery is being increasingly suggested as a lasting therapy to induce IIH remission. […] […] […] Therapeutic agents currently used in IIH aim to reduce ICP through reduction in CSF secretion. There are few therapeutic options and the recent Cochrane review reported: the two included randomised controlled trials showed modest benefits for acetazolamide for some outcomes, there is insufficient evidence to recommend or reject the efficacy of this intervention, or any other treatments currently available, for treating people with IIH. The IIHTT is the largest RCT to date and reports improvement in visual field function in patients with mild visual loss when treated with acetazolamide. Benefits were most marked in those with the most marked papilloedema. However high doses of acetazolamide were used (greater than 40% of patients were treated with 4 g of acetazolamide daily) and this may not be tolerable. Previous studies have demonstrated that 48% of patients discontinue acetazolamide when doses of just 1500 mg are utilised. Side effects include paraesthesia, dysgeusia, vomiting and diarrhoea as well as malaise, fatigue and depression. Acetazolamide is the only therapeutic that has been evaluated in RCT and is regarded as the first line therapy for IIH. However, not all clinicians prescribe acetazolamide for IIH due to the limitations of the evidence base highlighted by the 2015 Cochrane review in conjunction with the potential side effect profile.

• #1 Idiopathic Intracranial Hypertension

  https://practicalneurology.com/diseases-diagnoses/headache-pain/idiopathic-intracranial-hypertension/31655/

  Topiramate is an evidence-based medication for the preventive treatment of migraine that is a weak inhibitor of carbonic anhydrase and often used in the treatment of IIH. […] Furosemide, a loop diuretic, also inhibits carbonic anhydrase. […] Octreotide, a somatostatin analogue, has been shown to reduce ICP and improve headache. […] Obesity is associated with IIH and individuals with IIH who have a body mass index (BMI) greater than 40 kg/m2 are at higher risk for vision loss, making weight loss critical in the treatment of IIH. […] Surgical intervention should be considered for individuals who are at immediate risk of vision loss and have medically refractory disease. […] There are 3 main surgical options for IIH treatment. These include CSF-diverting procedures: LP or ventriculoperitoneal (VP) shunting, optic nerve sheath fenestration (ONSF), and venous sinus stenting.

• #1 Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment – UpToDate

  https://www.uptodate.com/contents/idiopathic-intracranial-hypertension-pseudotumor-cerebri-prognosis-and-treatment

  Rare patients present with or develop rapidly progressive vision loss (ie, fulminant IIH). […] Patients with IIH who appear to benefit from surgical intervention include those who fail, are intolerant to, or are noncompliant with maximum medical therapy. […] The two main surgical procedures in IIH are optic nerve sheath fenestration (ONSF) and cerebrospinal fluid (CSF) shunting procedures. […] ONSF appears to be an effective procedure in patients who have progressive vision loss despite medical therapy. […] CSF shunting procedures include ventriculoperitoneal shunt (VPS) or lumboperitoneal shunt (LPS). […] Venous sinus stenting is a relatively new and somewhat controversial treatment option for IIH. […] 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.

• #1 Idiopathic Intracranial Hypertension (IIH) Treatment & Management: Approach Considerations, Pharmacologic Therapy, Optic Nerve Sheath Fenestration, CSF Diversion, and Venous Sinus Stenting

  https://emedicine.medscape.com/article/1214410-treatment

  Pharmacologic management generally includes carbonic anhydrase inhibitors such as acetazolamide or topiramate. Acetazolamide decreases cerebrospinal fluid production, whereas topiramate promotes weight loss, potentially reducing intracranial pressure and alleviating headaches. […] In patients with stable visual function but inadequate headache relief with first-line pressure-lowering drugs, primary headache prophylaxis should be considered. Patients with IIH may experience headaches that have many of the features of migraine. […] Corticosteroids may rarely be used as a supplement to acetazolamide to hasten recovery in patients who present with severe papilledema. […] Glucagon-like peptide-1 receptor agonists (GLP-1-RA) have shown promise as an effective treatment for IIH. […] CSF diversion procedures are highly effective in lowering ICP. In most facilities they are the procedures of choice for treating patients with IIH who do not respond to maximum medical treatment.

• #1 Pseudotumor cerebri (idiopathic intracranial hypertension) – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pseudotumor-cerebri/diagnosis-treatment/drc-20354036

  The goal of pseudotumor cerebri treatment is to improve your symptoms and keep your eyesight from worsening. […] If you’re obese, your doctor might recommend a low-sodium weight-loss diet to help improve your symptoms. You might work with a dietitian to help with your weight-loss goals. Some people benefit from weight-loss programs or gastric surgery. […] One of the first drugs usually tried is acetazolamide, a glaucoma drug. This medication might reduce the production of cerebrospinal fluid and reduce symptoms. […] If your vision worsens, surgery to reduce the pressure around your optic nerve or to decrease the intracranial pressure might be necessary. […] In this procedure, a surgeon cuts a window into the membrane that surrounds the optic nerve to allow excess cerebrospinal fluid to escape.

• #1 Pseudotumor cerebri: An update on treatment options

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278127/

  Cerebrospinal fluid shunting is the most widely performed surgical treatment for PTC, and it is useful in the treatment of papilledema, headache, and visual loss. […] Optic nerve sheath decompression effectively treats patients with papilledema and severe visual loss but does not improve headache in most patients. […] While in some cases, the stenosis is a consequence of elevated ICP, several studies have demonstrated that a pressure gradient may persist across the stenotic segment even after ICP is normalized by lumbar or cervical puncture. […] In such cases, the stenosis may be the cause of ICP elevation, and endovascular stenting may be pursued if medical management is inadequate to relieve headache or papilledema. […] Treatment of PTC must be tailored to the patient’s presenting symptoms, vision, and comorbidities. A multidisciplinary team and often multiple treatment forms are necessary.

• #1 Pseudotumor cerebri (idiopathic intracranial hypertension) – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pseudotumor-cerebri/diagnosis-treatment/drc-20354036

  In another type of surgery, your doctor inserts a long, thin tube (shunt) into your brain or lower spine to help drain excess cerebrospinal fluid. […] This relatively new procedure is rarely used. It involves placing a stent in one of the larger veins in the head to increase the blood’s ability to flow. More study is needed to determine the benefits and risks of this procedure.

• #1 European Headache Federation guideline on idiopathic intracranial hypertension | The Journal of Headache and Pain | Full Text

  https://thejournalofheadacheandpain.biomedcentral.com/articles/10.1186/s10194-018-0919-2

  Surgical management is essential for IIH patients with rapidly declining visual function. The evidence base for choice of surgical technique is lacking and practice varies internationally and with surgeon preference. CSF diversion procedures including ventriculo-peritoneal, lumbo-peritoneal, and less frequently ventriculo-atrial shunting may be utilised. Ventriculo-peritoneal shunts are preferred due to lower revision rates compared to lumbo-peritoneal shunts (1.8 versus 4.3 revisions per patient respectively). Ventriculo-peritoneal shunts are typically placed using neuro-navigation and adjustable valves (anti-gravity or anti-siphon devices) that can reduce the risk of low pressure headaches. However, ventriculo-peritoneal shunt insertion leads to a temporary driving restriction in some countries such as the United Kingdom. Lumbo-peritoneal shunting may be considered but should be avoided in those with low lying cerebellar tonsils due to the risk of post-operative cerebellar tonsillar descent. Shunt revision is common with 51% requiring revision and multiple revisions required in 30%. Complications can occur including abdominal pain, shunt obstruction, migration and infection, low pressure headaches and subdural haematoma. An alternative to shunting is optic nerve sheath fenestration (ONSF) which is more cost effective in some health care systems than CSF shunting. But, this procedure also has a 26% revision rate, due to closing over of the fenestration, with an ensuring rise in ICP and consequent potential for further visual deterioration. Headache improvement after ONSF is variable (one third to one-half have no headache response).

• #1 Idiopathic intracranial hypertension | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/idiopathic-intracranial-hypertension-1?lang=us

  Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a syndrome with signs and symptoms of increased intracranial pressure but where a causative mass or hydrocephalus is not identified. […] First-line treatment options include weight loss in patients with a BMI 30kg/m2, with weight loss of ~15% possibly being curative, and carbonic anhydrase inhibitors such as acetazolamide and topiramate. […] Invasive treatment options, usually reserved for refractory cases, include venous sinus stenting for transverse sinus stenosis, which is typically reserved for severe cases with a trans-stenotic gradient of 8 mmHg and has been increasingly shown to be effective. […] Venous sinus stenting has been reported to improve headache in ~80% of cases, tinnitus in ~95%, papilledema in ~90%, and visual symptoms in ~90%, with a reduction in opening pressure by ~15 cmH2O. […] Other invasive options include internal jugular venous decompression, bariatric surgery as a surgical weight loss strategy, optic nerve sheath fenestration (only if vision is acutely threatened in fulminant idiopathic intracranial hypertension), and serial CSF letting or CSF shunting.

• #1 Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) – EyeWiki

  https://eyewiki.org/Pseudotumor_Cerebri_(Idiopathic_Intracranial_Hypertension)

  Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), is a disorder characterized by increased intracranial pressure (ICP) of unknown cause that predominantly affects obese women of childbearing age. […] Variable treatment options are available, but there are no formal guidelines with regards to therapeutic approach. […] The goal of treatment is to alleviate symptoms of ICP and preserve vision. Although diagnostic lumbar puncture may provide symptom relief the latter is often transient and requires combination with further long-term therapy. […] All obese patients should be encouraged to lose modest amount of weight. Among patients who are obese or overweight, weight loss of about 5-10% has been found to improve symptoms and signs. […] Medical therapy is usually considered among patients with mild to moderate disease.

• #1 Idiopathic intracranial hypertension doc – Bobby Jones CSF

  https://bobbyjonescsf.org/physician-information/idiopathic-intracranial-hypertension/

  Repeated lumbar punctures with drainage of up to 30 to 40 cc of spinal fluid can improve IIH symptoms temporarily, sometimes for a month or more. Occasionally, the condition has been brought under remission following one lumbar puncture. Serial lumbar punctures are a useful approach in pregnancy by reducing symptoms without the need for additional medications or other more invasive treatments. […] If the pressure is 25 mmH2O or greater, and a Chiari I malformation is not present, a spinal fluid shunt can be considered. These shunts drain spinal fluid into the abdominal cavity and reduce intracranial and spinal pressure. […] If the body mass index is very high (BMI of 35kg/m2 or more) spinal fluid shunts fail so often that they are not advised. The current recommendation when the BMI is 35kg/m2 or greater is to consider bariatric surgery.

• #1 Idiopathic Intracranial Hypertension

  https://practicalneurology.com/diseases-diagnoses/headache-pain/idiopathic-intracranial-hypertension/31655/

  Despite improving visual outcomes by reducing ICP, many people with IIH continue to have disabling headache even after ICP returns to normal. […] Treatment includes antiseizure medications, antidepressants, antihypertensives, onabotulinumtoxinA and, more recently, antibodies to calcitonin gene-related peptide (CGRP) or its receptor. […] Ultimately, treatment of headache in IIH is based on the headache phenotype, which is typically migrainous, and data on specific therapies for headache in IIH are limited.

• #1 Idiopathic Intracranial Hypertension – Neurologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/neurologic-disorders/headache/idiopathic-intracranial-hypertension

  Definitive treatment includes optic nerve sheath fenestration, shunting, and venous sinus stenting. […] Frequent ophthalmologic assessment (including quantitative visual fields) is required to monitor response to treatment; testing visual acuity is not sensitive enough to warn of impending vision loss. […] If vision deteriorates despite treatment, one of the following may be indicated: […] Optic nerve sheath fenestration […] Shunting (lumboperitoneal or ventriculoperitoneal) […] Endovascular venous stenting. […] Bariatric surgery with sustained weight loss may cure the disorder in patients who have obesity and were otherwise unable to lose weight (eg, with use of GLP-1 receptor agonists).

• #1 Idiopathic intracranial hypertension: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000351.htm

  Treatment is aimed at the cause of IIH. The main goal of treatments is to preserve vision and reduce the severity of headaches. […] A lumbar puncture (spinal tap) can help relieve pressure in the brain and prevent vision problems. Repeat lumbar punctures are helpful for pregnant women in order to delay surgery until after delivery. […] Other treatments may include: fluid or salt restriction, medicines such as corticosteroids, acetazolamide, furosemide, and topiramate, shunting procedures to relieve pressure from spinal fluid buildup, surgery to relieve pressure on the optic nerve, weight loss, treatment of the underlying disease, such as vitamin A overdose. […] People will need to have their vision closely monitored. There can be vision loss, which is sometimes permanent. Follow-up MRI or CT scans may be done to rule out problems such as tumors or hydrocephalus (buildup of fluid inside the skull). […] In some cases, the pressure inside the brain remains high for many years. Symptoms can return in some people. A small number of people have symptoms that slowly get worse and lead to blindness.

• #1 Idiopathic Intracranial Hypertension (Pseudotumor cerebri): From One Medical Student to Another

  http://webeye.ophth.uiowa.edu/eyeforum/tutorials/IIH-med-student/index.htm

  Following the results of the IIHTT, first-line treatment of IIH includes weight loss including bariatric surgery if indicated and acetazolamide, a carbonic anhydrase inhibitor in the maximally tolerated dosage of up to 4 grams per day. […] If a patient fails medical treatment and continues to have progressive visual loss, surgery with either optic nerve sheath fenestration or CSF shunting can be performed. […] Treatment can help to stabilize decreases in vision, and in many cases partial or complete recovery of vision is possible. Long-term follow-up studies have shown that treatment leads to visual field improvement in most patients (60%) and bilateral blindness is a less common complication (10%). […] First-line treatment includes weight loss and acetazolamide to help reverse or stabilize visual field damage. Different interventions can be considered if the patients with severe, vision threatening papilledema or in patients that are refractory to more conservative therapies. Long-term follow-up and monitoring are required for all patients with IIH.

• #1 DigitalCommons@PCOM – Research Day: A holistic approach to idiopathic intracranial hypertension treatment and management

  https://digitalcommons.pcom.edu/research_day/research_day_GA_2024/researchGA2024/55/

  A holistic approach to idiopathic intracranial hypertension treatment and management […] Effective IIH management requires a comprehensive care team to ensure that the patient feels supported in all aspects of health management. This approach should have a PCP serving as the center of a coordinated care model encompassing various resources (e.g., mental health professionals, dietitians, osteopathic manipulative treatment.) Emphasizing the importance of a holistic strategy that addresses both mental and physical aspects of the disease can improve positive outcomes in patient care and disease management. Until further research sheds light on the underlying pathophysiology of IIH, the focus should shift towards establishing holistic treatment protocols and developing multifaceted care teams to promote the best outcomes for patients. This approach will empower patients and allow them to foster a sense of control in the face of this challenging condition.

• #1 Current Overview of Idiopathic Intracranial Hypertension | 2021, Volume 27 – Issue 3 | Turkish Journal of Neurology

  https://www.tjn.org.tr/full-text/157/eng

  Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), is an increment in the intracranial pressure without identifiable causal factors. […] IIH treatment should be arranged considering the severity of headache and papilledema, presence of vision loss, response to medical treatment, and comorbid conditions, with individual planning for each patient. […] In patients where weight loss and acetazolamide are insufficient for symptoms resolution, other medical and surgical treatment options should be evaluated rapidly. […] The treatment options, including weight loss, medical treatment, and surgical interventions, should be evaluated individually for each patient, according to the presence, degree, and rate of development of vision loss, severity of headache and papilledema, response to medical treatment, comorbid conditions, and tolerability of treatment.

• #2 Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment – UpToDate

  https://www.uptodate.com/contents/idiopathic-intracranial-hypertension-pseudotumor-cerebri-prognosis-and-treatment

  Idiopathic intracranial hypertension (IIH) was formerly called pseudotumor cerebri. […] Recommendations for the treatment of IIH are limited by only a few randomized controlled trials. […] The treatment of patients with IIH has two major goals: the alleviation of symptoms (usually headache) and the preservation of vision. […] For initial treatment of patients with IIH, we suggest treatment with the carbonic anhydrase inhibitor acetazolamide. […] Acetazolamide should be initiated promptly and can be rapidly titrated up to 4 grams per day divided into two doses. […] For patients with persistent or worsening visual symptoms despite maximizing treatment with acetazolamide, we suggest the addition of furosemide. […] Patients with IIH can continue to have headaches despite improvement in papilledema and visual function.

• #2 Pseudotumor cerebri: An update on treatment options

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278127/

  The aim was to identify Pseudotumor cerebri treatment options and assess their efficacy. […] Treatment options range from observation to surgical intervention. Weight loss and medical treatment may be utilized in cases without vision loss or in combination with surgical treatment. Cerebrospinal fluid shunting procedures and/or optic nerve sheath decompression is indicated for severe vision loss or headache unresponsive to medical management. The recent use of endovascular stenting of transverse sinus stenoses has also demonstrated benefit in patients with pseudotumor cerebri. […] While each treatment form may be successful individually, a multimodal approach is typically utilized with treatments selected on a case-by-case basis. […] Treatment of PTC ranges from observation to emergent surgery. A team approach amongst the patient’s neurologist, ophthalmologist, primary care physician, and neurosurgeon is essential.

• #2 Idiopathic Intracranial Hypertension, Pseudotumor cerebri – EyeRounds.org – Ophthalmology – The University of Iowa

  http://eyerounds.org/cases/99-Pseudotumor-Cerebri.htm

  A variety of treatments for IIH have been proposed and there is much anecdotal evidence for these treatments. There is no evidence-based data currently available but an NIH sponsored clinical treatment trial is beginning in 2009. Patients with no visual loss or mild visual loss are treated medically. […] Weight loss is an important part of treatment and is always recommended. Most patients improve after losing 5-10% of their total body weight. It appears that in addition to weight reduction, sodium restriction is also a useful adjunct. We recommend that patients lose about one pound a week for several months and then maintain the weight loss. The mechanism of the beneficial effect of weight loss on this disease is unclear but there are parallels with the beneficial effect of weight loss and sodium restriction for treatment of essential hypertension.

• #2 Idiopathic intracranial hypertension (pseudotumor cerebri): recognition, treatment, and ongoing management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23136035/

  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. […] 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 5-10 % 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.

• #2 Idiopathic intracranial hypertension: from concise history to current management | The Egyptian Journal of Neurology, Psychiatry and Neurosurgery | Full Text

  https://ejnpn.springeropen.com/articles/10.1186/s41983-023-00730-7

  Bariatric surgery was also excellent for decreasing intracranial pressure than weight management in a randomized clinical trial conducted by Mollan and colleagues in 2021. […] The clinical course of IIH is not always monophasic, although it can relapse. […] The management of IIH, consisting of conservative (control body weight), medical treatment, and surgical treatment, aims to reduce the symptoms and maintain visual function.

• #2 Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) – EyeWiki

  https://eyewiki.org/Pseudotumor_Cerebri_(Idiopathic_Intracranial_Hypertension)

  Among the options available, acetazolamide, a carbonic anhydrase inhibitors, is believed to reduce the rate of CSF production and is the first-line medical treatment for IIH. […] Surgical management should be the option of choice among patients with refractory headaches or more severe/ rapidly progressive visual field loss when all other options have failed to prevent progressive visual loss. […] The two most used procedures are CSF diversion via shunt and optic nerve sheath fenestration. […] Optic nerve sheath fenestration is the preferred surgical procedure for papilledema with associated severe vision loss but no or minimal ICP symptoms (such as headache). […] CSF shunting produces rapid reduction in ICP and is therefore most beneficial among patients with vision loss and symptoms of raised ICP.

• #2 Intracranial Hypertension or Pseudotumor Cerebri: The Basics – Migraine Canada

  https://migrainecanada.org/intracranial-hypertension-or-pseudotumor-cerebri-the-basics/

  Idiopathic Intracranial Hypertension (IIH), also known as pseudotumor cerebri, is a condition where the pressure inside the skull increases without an obvious cause. […] Losing weight, even as little as 10% of body weight, can significantly improve symptoms and protect vision. Weight loss is challenging, but crucial for managing IIH. […] In severe cases where vision is at risk, surgical shunting may be considered. This involves placing a tube to drain fluid from the brain ventricles to the abdomen (ventriculo-peritoneal shunt) or from the spine to the abdomen (lumbo-peritoneal shunt). These procedures carry high risks and should only be performed by experts. […] Physicians may recommend treatments like topiramate and Botox for headache control in these patients. […] Acetazolamide is a diuretic that can improve vision but may not be as effective for headaches. Side effects include nausea, tingling in extremities, and fatigue.

• #2 Idiopathic Intracranial Hypertension, Pseudotumor cerebri – EyeRounds.org – Ophthalmology – The University of Iowa

  http://eyerounds.org/cases/99-Pseudotumor-Cerebri.htm

  For patients requiring medical therapy, acetazolamide (Diamox) is the most commonly used medication. It is relatively safe but nearly all patients experience the side effect of paresthesias, or numbness and tingling in the extremities. Patients also report an alteration in taste such that carbonated soft drinks taste metallic. Less commonly, kidney stones can occur. Another diuretic commonly used that appears to be effective in some patients is furosemide (Lasix). Topiramate (Topamax) is used for migraine prophylaxis and has been found to be effective in the treatment of headaches associated with IIH. […] Intractable headache or progression of visual loss despite maximal medical treatment requires surgical therapy. The primary surgical options are optic nerve sheath fenestration or a CSF diversion procedure (shunt). Optic nerve sheath fenestration involves the creation of a window in the sheath-like covering of the optic nerve. This creates an outlet for escape of cerebrospinal fluid and alleviates the direct pressure on the optic nerve. Later, the area around the nerve within the nerve sheath scars down further protecting the optic disc from damage.

• #2 Idiopathic Intracranial Hypertension (IIH) Treatment & Management: Approach Considerations, Pharmacologic Therapy, Optic Nerve Sheath Fenestration, CSF Diversion, and Venous Sinus Stenting

  https://emedicine.medscape.com/article/1214410-treatment

  Pharmacologic management generally includes carbonic anhydrase inhibitors such as acetazolamide or topiramate. Acetazolamide decreases cerebrospinal fluid production, whereas topiramate promotes weight loss, potentially reducing intracranial pressure and alleviating headaches. […] In patients with stable visual function but inadequate headache relief with first-line pressure-lowering drugs, primary headache prophylaxis should be considered. Patients with IIH may experience headaches that have many of the features of migraine. […] Corticosteroids may rarely be used as a supplement to acetazolamide to hasten recovery in patients who present with severe papilledema. […] Glucagon-like peptide-1 receptor agonists (GLP-1-RA) have shown promise as an effective treatment for IIH. […] CSF diversion procedures are highly effective in lowering ICP. In most facilities they are the procedures of choice for treating patients with IIH who do not respond to maximum medical treatment.

• #2 Digoxin as a Treatment for Patients With Idiopathic Intracranial Hypertension

  https://www.degruyterbrill.com/document/doi/10.7556/jaoa.2014.039/html?lang=en&srsltid=AfmBOopV3gX9DLHayJ1tFrv45wDA4ouBXSLeZiEhlevPBsHcIkDNXMnB

  Neblett et al suggested that digoxin can be beneficial to patients with IIH. We discussed this option with the patient and with cardiologists and cardiology staff. The patient agreed to try digoxin. After 6 months of treatment at a dosage of 0.125 mg daily (in tablet form), her headaches disappeared and her vision stabilized. […] There are multiple efficacious treatment options for patients with IIH. However, when a patient presents with a complicated medical history, treatment becomes more challenging. […] Few cases of IIH managed with digoxin have been reported, to our knowledge. Digoxin acts on the ouabain-sensitive Na+/K+ ATPase receptors in the choroid plexus, thereby decreasing CSF production. […] In patients with IIH and complicated medical conditions, physicians should consider digoxin. Even though the mechanism of action is not well understood, it clearly has benefits in this group of patients.

• #2 Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment – UpToDate

  https://www.uptodate.com/contents/idiopathic-intracranial-hypertension-pseudotumor-cerebri-prognosis-and-treatment

  Rare patients present with or develop rapidly progressive vision loss (ie, fulminant IIH). […] Patients with IIH who appear to benefit from surgical intervention include those who fail, are intolerant to, or are noncompliant with maximum medical therapy. […] The two main surgical procedures in IIH are optic nerve sheath fenestration (ONSF) and cerebrospinal fluid (CSF) shunting procedures. […] ONSF appears to be an effective procedure in patients who have progressive vision loss despite medical therapy. […] CSF shunting procedures include ventriculoperitoneal shunt (VPS) or lumboperitoneal shunt (LPS). […] Venous sinus stenting is a relatively new and somewhat controversial treatment option for IIH. […] 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.

• #2 European Headache Federation guideline on idiopathic intracranial hypertension | The Journal of Headache and Pain | Full Text

  https://thejournalofheadacheandpain.biomedcentral.com/articles/10.1186/s10194-018-0919-2

  Surgical management is essential for IIH patients with rapidly declining visual function. The evidence base for choice of surgical technique is lacking and practice varies internationally and with surgeon preference. CSF diversion procedures including ventriculo-peritoneal, lumbo-peritoneal, and less frequently ventriculo-atrial shunting may be utilised. Ventriculo-peritoneal shunts are preferred due to lower revision rates compared to lumbo-peritoneal shunts (1.8 versus 4.3 revisions per patient respectively). Ventriculo-peritoneal shunts are typically placed using neuro-navigation and adjustable valves (anti-gravity or anti-siphon devices) that can reduce the risk of low pressure headaches. However, ventriculo-peritoneal shunt insertion leads to a temporary driving restriction in some countries such as the United Kingdom. Lumbo-peritoneal shunting may be considered but should be avoided in those with low lying cerebellar tonsils due to the risk of post-operative cerebellar tonsillar descent. Shunt revision is common with 51% requiring revision and multiple revisions required in 30%. Complications can occur including abdominal pain, shunt obstruction, migration and infection, low pressure headaches and subdural haematoma. An alternative to shunting is optic nerve sheath fenestration (ONSF) which is more cost effective in some health care systems than CSF shunting. But, this procedure also has a 26% revision rate, due to closing over of the fenestration, with an ensuring rise in ICP and consequent potential for further visual deterioration. Headache improvement after ONSF is variable (one third to one-half have no headache response).

• #2 Pseudotumor cerebri: An update on treatment options

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278127/

  Cerebrospinal fluid shunting is the most widely performed surgical treatment for PTC, and it is useful in the treatment of papilledema, headache, and visual loss. […] Optic nerve sheath decompression effectively treats patients with papilledema and severe visual loss but does not improve headache in most patients. […] While in some cases, the stenosis is a consequence of elevated ICP, several studies have demonstrated that a pressure gradient may persist across the stenotic segment even after ICP is normalized by lumbar or cervical puncture. […] In such cases, the stenosis may be the cause of ICP elevation, and endovascular stenting may be pursued if medical management is inadequate to relieve headache or papilledema. […] Treatment of PTC must be tailored to the patient’s presenting symptoms, vision, and comorbidities. A multidisciplinary team and often multiple treatment forms are necessary.

• #2 Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment – UpToDate

  https://www.uptodate.com/contents/idiopathic-intracranial-hypertension-pseudotumor-cerebri-prognosis-and-treatment/print

  Patients with IIH who appear to benefit from surgical intervention include those who fail, are intolerant to, or are noncompliant with maximum medical therapy. […] The two main surgical procedures in IIH are optic nerve sheath fenestration (ONSF) and cerebrospinal fluid (CSF) shunting procedures. […] ONSF appears to be an effective procedure in patients who have progressive vision loss despite medical therapy. […] CSF shunting procedures include ventriculoperitoneal shunt (VPS) or lumboperitoneal shunt (LPS). […] Venous sinus stenting is a relatively new and somewhat controversial treatment option for IIH. […] 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.

• #2 Idiopathic intracranial hypertension | Neurosurgery Inselspital Bern

  https://neurochirurgie.insel.ch/en/diseases-specialities/liquor-disorders/idiopathic-intracranial-hypertension

  In case of reduced visual acuity, which, however, progresses without rapid deterioration, a therapeutic lumbar puncture is performed in addition to the measures mentioned above. […] Only in cases where repeated lumbar punctures cannot be stopped due to persistent or increasing symptoms, a permanent cerebrospinal fluid diversion must be surgically implemented. […] In case of rapid deterioration of visual acuity or the visual field, prompt action is required. […] If conservative treatment does not bring immediate improvement in symptoms, a surgical approach must be considered. […] In 2002, Higgins et al. published a new procedure: the first catheter-based intervention in the form of sinus stenting in a patient with bilateral distal transverse sinus stenoses that were not treatable with conventional methods.

• #2 Idiopathic intracranial hypertension | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/idiopathic-intracranial-hypertension-1?lang=us

  Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a syndrome with signs and symptoms of increased intracranial pressure but where a causative mass or hydrocephalus is not identified. […] First-line treatment options include weight loss in patients with a BMI 30kg/m2, with weight loss of ~15% possibly being curative, and carbonic anhydrase inhibitors such as acetazolamide and topiramate. […] Invasive treatment options, usually reserved for refractory cases, include venous sinus stenting for transverse sinus stenosis, which is typically reserved for severe cases with a trans-stenotic gradient of 8 mmHg and has been increasingly shown to be effective. […] Venous sinus stenting has been reported to improve headache in ~80% of cases, tinnitus in ~95%, papilledema in ~90%, and visual symptoms in ~90%, with a reduction in opening pressure by ~15 cmH2O. […] Other invasive options include internal jugular venous decompression, bariatric surgery as a surgical weight loss strategy, optic nerve sheath fenestration (only if vision is acutely threatened in fulminant idiopathic intracranial hypertension), and serial CSF letting or CSF shunting.

• #2 Idiopathic intracranial hypertension | Neurosurgery Inselspital Bern

  https://neurochirurgie.insel.ch/en/diseases-specialities/liquor-disorders/idiopathic-intracranial-hypertension

  However, this method is not considered a standard procedure for IIH and is only applied in very carefully selected patients, exclusively in specialized centers such as Inselspital. […] As there are various treatment options for IIH, the measures and guidelines must be individually tailored to the patient and discussed in detail with them.

• #2 Idiopathic intracranial hypertension: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000351.htm

  Treatment is aimed at the cause of IIH. The main goal of treatments is to preserve vision and reduce the severity of headaches. […] A lumbar puncture (spinal tap) can help relieve pressure in the brain and prevent vision problems. Repeat lumbar punctures are helpful for pregnant women in order to delay surgery until after delivery. […] Other treatments may include: fluid or salt restriction, medicines such as corticosteroids, acetazolamide, furosemide, and topiramate, shunting procedures to relieve pressure from spinal fluid buildup, surgery to relieve pressure on the optic nerve, weight loss, treatment of the underlying disease, such as vitamin A overdose. […] People will need to have their vision closely monitored. There can be vision loss, which is sometimes permanent. Follow-up MRI or CT scans may be done to rule out problems such as tumors or hydrocephalus (buildup of fluid inside the skull). […] In some cases, the pressure inside the brain remains high for many years. Symptoms can return in some people. A small number of people have symptoms that slowly get worse and lead to blindness.

• #2 Idiopathic intracranial hypertension | Neurosurgery Inselspital Bern

  https://neurochirurgie.insel.ch/en/diseases-specialities/liquor-disorders/idiopathic-intracranial-hypertension

  Idiopathic intracranial hypertension (IIH), formerly also known as pseudotumor cerebri, is a rare and often unrecognized disorder. […] Thanks to modern neurosurgery and neuroradiology, various treatment options are now available for this rare condition. […] The treatment options for IIH are applied in a stepwise escalation, depending on the severity of the symptoms. […] Depending on the nature of the symptoms at the initial diagnosis, an individualized treatment strategy must be determined and adjusted according to the course of the disease. […] The Association of the Scientific Medical Societies in Germany (AWMF) recommends a stepwise approach: […] For mild symptoms (headaches without visual disturbances), conservative treatment is primarily recommended. […] Additionally, the medication acetazolamide, a carbonic anhydrase inhibitor, can be taken to reduce cerebrospinal fluid production.

• #2 Idiopathic Intracranial Hypertension (IIH) Treatment & Management: Approach Considerations, Pharmacologic Therapy, Optic Nerve Sheath Fenestration, CSF Diversion, and Venous Sinus Stenting

  https://emedicine.medscape.com/article/1214410-treatment

  Management guidelines are based on symptoms and extent of visual impairment at presentation. If there is no immediate threat to vision, medical therapy is recommended. In the event of an immediate threat to visual function, a temporary CSF draining procedure (ie, placement of a lumbar drain) is immediately performed, and a definitive surgical plan is made; either a ventriculoperitoneal shunt or optic nerve sheath fenestration. […] The primary treatment objectives for patients with idiopathic intracranial hypertension (IIH) are to preserve optic nerve function, manage elevated intracranial pressure (ICP), and alleviate symptoms. […] The initial treatment typically involves administering acetazolamide, dosed according to the patient’s symptoms, tolerance, and visual function. If progressive visual field loss occurs despite maximal medical therapy, urgent surgical intervention may be necessary.

• #2 Idiopathic Intracranial Hypertension – Neurologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/neurologic-disorders/headache/idiopathic-intracranial-hypertension

  Definitive treatment includes optic nerve sheath fenestration, shunting, and venous sinus stenting. […] Frequent ophthalmologic assessment (including quantitative visual fields) is required to monitor response to treatment; testing visual acuity is not sensitive enough to warn of impending vision loss. […] If vision deteriorates despite treatment, one of the following may be indicated: […] Optic nerve sheath fenestration […] Shunting (lumboperitoneal or ventriculoperitoneal) […] Endovascular venous stenting. […] Bariatric surgery with sustained weight loss may cure the disorder in patients who have obesity and were otherwise unable to lose weight (eg, with use of GLP-1 receptor agonists).

• #2 Intracranial Hypertension (Pseudotumor Cerebri): Diagnosis & Treatment | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/intracranial-hypertension-pseudotumor-cerebri

  If treated, the outcome is good. If not treated, permanent blindness can occur. Unfortunately, up to one in 10 people with intracranial hypertension have some vision loss. Treatment typically lasts six to 12 months. With treatment, in most cases, this condition goes away. However, increased pressure can return months or even years later. You can reduce this risk by helping your child maintain a healthy weight. It is important to have regular eye exams to check for vision loss even after the intracranial hypertension gets better.

• #2 Idiopathic intracranial hypertension | MedLink Neurology

  https://www.medlink.com/articles/idiopathic-intracranial-hypertension

  Idiopathic intracranial hypertension is a disorder of increased intracranial pressure that is most commonly seen in obese women of childbearing age. […] The Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) provides evidence-based therapy for patients who have mild visual loss. […] A team approach to management is ideal, and a team leader (generally a neurologist or neuro-ophthalmologist) is critical. […] Treatment options include weight loss, medication (primarily acetazolamide), and surgery (optic nerve sheath fenestration, shunting, venous sinus stenting). […] The Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) was a randomized controlled clinical trial of acetazolamide and placebo in addition to a weight reduction diet. This study provides evidence-based guidance for patients presenting with mild vision loss.

• #2 Idiopathic intracranial hypertension – Wikipedia

  https://en.wikipedia.org/wiki/Idiopathic_intracranial_hypertension

  Treatment includes a healthy diet, salt restriction, and exercise. […] The medication acetazolamide may also be used along with the above measures. […] A small percentage of people may require surgery to relieve the pressure. […] The primary goal in treatment of IIH is the prevention of visual loss and blindness, as well as symptom control. […] IIH is treated mainly through the reduction of CSF pressure and IIH may resolve after initial treatment, may go into spontaneous remission (although it can still relapse at a later stage), or may continue chronically. […] There are three main treatment approaches: weight loss, different medications and surgical interventions. […] The first step in symptom control is drainage of cerebrospinal fluid by lumbar puncture. […] The best-studied medical treatment for intracranial hypertension is acetazolamide (Diamox), which acts by inhibiting the enzyme carbonic anhydrase, and it reduces CSF production by six to 57 percent.

• #3 Idiopathic Intracranial Hypertension, Pseudotumor cerebri – EyeRounds.org – Ophthalmology – The University of Iowa

  http://eyerounds.org/cases/99-Pseudotumor-Cerebri.htm

  A variety of treatments for IIH have been proposed and there is much anecdotal evidence for these treatments. There is no evidence-based data currently available but an NIH sponsored clinical treatment trial is beginning in 2009. Patients with no visual loss or mild visual loss are treated medically. […] Weight loss is an important part of treatment and is always recommended. Most patients improve after losing 5-10% of their total body weight. It appears that in addition to weight reduction, sodium restriction is also a useful adjunct. We recommend that patients lose about one pound a week for several months and then maintain the weight loss. The mechanism of the beneficial effect of weight loss on this disease is unclear but there are parallels with the beneficial effect of weight loss and sodium restriction for treatment of essential hypertension.

• #3 A High-Pressure Situation: Idiopathic Intracranial Hypertension Diagnosis and Treatments – Ophthalmology Advisor

  https://www.ophthalmologyadvisor.com/features/pseudotumor-cerebri-iih-diagnosis-protocol-and-treatment-options/

  The goals of treatment with IIH are to limit visual morbidity from persistent optic nerve edema and to relieve symptoms, which is most commonly headache. […] Weight loss and treatment with carbonic anhydrase inhibitors (CAI) such as acetazolamide are the mainstays of managing IIH. […] The Idiopathic Intracranial Hypertension Treatment Trial was a multicenter, double-blind, randomized, placebo-controlled study of acetazolamide in 165 IIH participants with mild visual loss that found subjects taking acetazolamide along with a low-sodium diet program had significantly better visual outcomes than those taking placebo along with the diet. […] An important consideration that should be discussed with IIH patients is the potential risk of acetazolamide for pregnancy. […] For patients who cannot be treated with acetazolamide due to side effect intolerance, methazolamide and topiramate are potential alternative options.

• #3 Idiopathic Intracranial Hypertension, Pseudotumor cerebri – EyeRounds.org – Ophthalmology – The University of Iowa

  http://eyerounds.org/cases/99-Pseudotumor-Cerebri.htm

  For patients requiring medical therapy, acetazolamide (Diamox) is the most commonly used medication. It is relatively safe but nearly all patients experience the side effect of paresthesias, or numbness and tingling in the extremities. Patients also report an alteration in taste such that carbonated soft drinks taste metallic. Less commonly, kidney stones can occur. Another diuretic commonly used that appears to be effective in some patients is furosemide (Lasix). Topiramate (Topamax) is used for migraine prophylaxis and has been found to be effective in the treatment of headaches associated with IIH. […] Intractable headache or progression of visual loss despite maximal medical treatment requires surgical therapy. The primary surgical options are optic nerve sheath fenestration or a CSF diversion procedure (shunt). Optic nerve sheath fenestration involves the creation of a window in the sheath-like covering of the optic nerve. This creates an outlet for escape of cerebrospinal fluid and alleviates the direct pressure on the optic nerve. Later, the area around the nerve within the nerve sheath scars down further protecting the optic disc from damage.

• #3 Digoxin as a Treatment for Patients With Idiopathic Intracranial Hypertension

  https://www.degruyterbrill.com/document/doi/10.7556/jaoa.2014.039/html?lang=en&srsltid=AfmBOopV3gX9DLHayJ1tFrv45wDA4ouBXSLeZiEhlevPBsHcIkDNXMnB

  Neblett et al suggested that digoxin can be beneficial to patients with IIH. We discussed this option with the patient and with cardiologists and cardiology staff. The patient agreed to try digoxin. After 6 months of treatment at a dosage of 0.125 mg daily (in tablet form), her headaches disappeared and her vision stabilized. […] There are multiple efficacious treatment options for patients with IIH. However, when a patient presents with a complicated medical history, treatment becomes more challenging. […] Few cases of IIH managed with digoxin have been reported, to our knowledge. Digoxin acts on the ouabain-sensitive Na+/K+ ATPase receptors in the choroid plexus, thereby decreasing CSF production. […] In patients with IIH and complicated medical conditions, physicians should consider digoxin. Even though the mechanism of action is not well understood, it clearly has benefits in this group of patients.

• #3 Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) – EyeWiki

  https://eyewiki.org/Pseudotumor_Cerebri_(Idiopathic_Intracranial_Hypertension)

  Among the options available, acetazolamide, a carbonic anhydrase inhibitors, is believed to reduce the rate of CSF production and is the first-line medical treatment for IIH. […] Surgical management should be the option of choice among patients with refractory headaches or more severe/ rapidly progressive visual field loss when all other options have failed to prevent progressive visual loss. […] The two most used procedures are CSF diversion via shunt and optic nerve sheath fenestration. […] Optic nerve sheath fenestration is the preferred surgical procedure for papilledema with associated severe vision loss but no or minimal ICP symptoms (such as headache). […] CSF shunting produces rapid reduction in ICP and is therefore most beneficial among patients with vision loss and symptoms of raised ICP.

• #3 Idiopathic Intracranial Hypertension, Pseudotumor cerebri – EyeRounds.org – Ophthalmology – The University of Iowa

  http://eyerounds.org/cases/99-Pseudotumor-Cerebri.htm

  Placement of a shunt (lumboperitoneal or ventriculoperitoneal) is another surgical treatment option. The device diverts cerebrospinal fluid to the abdomen or into the jugular vein (ventriculojugular). Both types of surgeries have their own risks and benefits and unfortunately about 50% of shunts fail at some point. […] Other medications that can be used are topiramate and furosemide. With severe visual loss, surgery can be done immediately or after a short medical trial. Steroids can be used to lower pressure while the patient is awaiting a CSF shunting procedure.

• #3 Idiopathic intracranial hypertension | Neurosurgery Inselspital Bern

  https://neurochirurgie.insel.ch/en/diseases-specialities/liquor-disorders/idiopathic-intracranial-hypertension

  In case of reduced visual acuity, which, however, progresses without rapid deterioration, a therapeutic lumbar puncture is performed in addition to the measures mentioned above. […] Only in cases where repeated lumbar punctures cannot be stopped due to persistent or increasing symptoms, a permanent cerebrospinal fluid diversion must be surgically implemented. […] In case of rapid deterioration of visual acuity or the visual field, prompt action is required. […] If conservative treatment does not bring immediate improvement in symptoms, a surgical approach must be considered. […] In 2002, Higgins et al. published a new procedure: the first catheter-based intervention in the form of sinus stenting in a patient with bilateral distal transverse sinus stenoses that were not treatable with conventional methods.

• #3 Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment – UpToDate

  https://www.uptodate.com/contents/idiopathic-intracranial-hypertension-pseudotumor-cerebri-prognosis-and-treatment

  Rare patients present with or develop rapidly progressive vision loss (ie, fulminant IIH). […] Patients with IIH who appear to benefit from surgical intervention include those who fail, are intolerant to, or are noncompliant with maximum medical therapy. […] The two main surgical procedures in IIH are optic nerve sheath fenestration (ONSF) and cerebrospinal fluid (CSF) shunting procedures. […] ONSF appears to be an effective procedure in patients who have progressive vision loss despite medical therapy. […] CSF shunting procedures include ventriculoperitoneal shunt (VPS) or lumboperitoneal shunt (LPS). […] Venous sinus stenting is a relatively new and somewhat controversial treatment option for IIH. […] 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.

• #3 Idiopathic Intracranial Hypertension

  https://practicalneurology.com/diseases-diagnoses/headache-pain/idiopathic-intracranial-hypertension/31655/

  Despite improving visual outcomes by reducing ICP, many people with IIH continue to have disabling headache even after ICP returns to normal. […] Treatment includes antiseizure medications, antidepressants, antihypertensives, onabotulinumtoxinA and, more recently, antibodies to calcitonin gene-related peptide (CGRP) or its receptor. […] Ultimately, treatment of headache in IIH is based on the headache phenotype, which is typically migrainous, and data on specific therapies for headache in IIH are limited.

• #3 Intracranial Hypertension (Pseudotumor Cerebri): Diagnosis & Treatment | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/intracranial-hypertension-pseudotumor-cerebri

  If treated, the outcome is good. If not treated, permanent blindness can occur. Unfortunately, up to one in 10 people with intracranial hypertension have some vision loss. Treatment typically lasts six to 12 months. With treatment, in most cases, this condition goes away. However, increased pressure can return months or even years later. You can reduce this risk by helping your child maintain a healthy weight. It is important to have regular eye exams to check for vision loss even after the intracranial hypertension gets better.

• #3 Idiopathic intracranial hypertension | MedLink Neurology

  https://www.medlink.com/articles/idiopathic-intracranial-hypertension

  Idiopathic intracranial hypertension is a disorder of increased intracranial pressure that is most commonly seen in obese women of childbearing age. […] The Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) provides evidence-based therapy for patients who have mild visual loss. […] A team approach to management is ideal, and a team leader (generally a neurologist or neuro-ophthalmologist) is critical. […] Treatment options include weight loss, medication (primarily acetazolamide), and surgery (optic nerve sheath fenestration, shunting, venous sinus stenting). […] The Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) was a randomized controlled clinical trial of acetazolamide and placebo in addition to a weight reduction diet. This study provides evidence-based guidance for patients presenting with mild vision loss.

• #3 Pseudotumor cerebri: An update on treatment options

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278127/

  The aim was to identify Pseudotumor cerebri treatment options and assess their efficacy. […] Treatment options range from observation to surgical intervention. Weight loss and medical treatment may be utilized in cases without vision loss or in combination with surgical treatment. Cerebrospinal fluid shunting procedures and/or optic nerve sheath decompression is indicated for severe vision loss or headache unresponsive to medical management. The recent use of endovascular stenting of transverse sinus stenoses has also demonstrated benefit in patients with pseudotumor cerebri. […] While each treatment form may be successful individually, a multimodal approach is typically utilized with treatments selected on a case-by-case basis. […] Treatment of PTC ranges from observation to emergent surgery. A team approach amongst the patient’s neurologist, ophthalmologist, primary care physician, and neurosurgeon is essential.

• #4 European Headache Federation guideline on idiopathic intracranial hypertension | The Journal of Headache and Pain | Full Text

  https://thejournalofheadacheandpain.biomedcentral.com/articles/10.1186/s10194-018-0919-2

  […] […] There is a clear association between IIH and weight with over 90-95% of patients being obese. Additionally, IIH is reported in the context of gaining 5-15% of body weight. Weight loss is the only established disease modifying therapy in IIH. Consequently, patients should be sensitively counselled about the importance of weight loss. However, the amount of weight loss required is not well established. Additionally, the optimal method of weight loss is uncertain. Dietary strategies are notoriously difficult to achieve and maintain in the long term. The role of bariatric surgery is being increasingly suggested as a lasting therapy to induce IIH remission. […] […] […] Therapeutic agents currently used in IIH aim to reduce ICP through reduction in CSF secretion. There are few therapeutic options and the recent Cochrane review reported: the two included randomised controlled trials showed modest benefits for acetazolamide for some outcomes, there is insufficient evidence to recommend or reject the efficacy of this intervention, or any other treatments currently available, for treating people with IIH. The IIHTT is the largest RCT to date and reports improvement in visual field function in patients with mild visual loss when treated with acetazolamide. Benefits were most marked in those with the most marked papilloedema. However high doses of acetazolamide were used (greater than 40% of patients were treated with 4 g of acetazolamide daily) and this may not be tolerable. Previous studies have demonstrated that 48% of patients discontinue acetazolamide when doses of just 1500 mg are utilised. Side effects include paraesthesia, dysgeusia, vomiting and diarrhoea as well as malaise, fatigue and depression. Acetazolamide is the only therapeutic that has been evaluated in RCT and is regarded as the first line therapy for IIH. However, not all clinicians prescribe acetazolamide for IIH due to the limitations of the evidence base highlighted by the 2015 Cochrane review in conjunction with the potential side effect profile.

• #4 Idiopathic Intracranial Hypertension

  https://practicalneurology.com/diseases-diagnoses/headache-pain/idiopathic-intracranial-hypertension/31655/

  Topiramate is an evidence-based medication for the preventive treatment of migraine that is a weak inhibitor of carbonic anhydrase and often used in the treatment of IIH. […] Furosemide, a loop diuretic, also inhibits carbonic anhydrase. […] Octreotide, a somatostatin analogue, has been shown to reduce ICP and improve headache. […] Obesity is associated with IIH and individuals with IIH who have a body mass index (BMI) greater than 40 kg/m2 are at higher risk for vision loss, making weight loss critical in the treatment of IIH. […] Surgical intervention should be considered for individuals who are at immediate risk of vision loss and have medically refractory disease. […] There are 3 main surgical options for IIH treatment. These include CSF-diverting procedures: LP or ventriculoperitoneal (VP) shunting, optic nerve sheath fenestration (ONSF), and venous sinus stenting.

• #4 Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment – UpToDate

  https://www.uptodate.com/contents/idiopathic-intracranial-hypertension-pseudotumor-cerebri-prognosis-and-treatment/print

  Patients with IIH who appear to benefit from surgical intervention include those who fail, are intolerant to, or are noncompliant with maximum medical therapy. […] The two main surgical procedures in IIH are optic nerve sheath fenestration (ONSF) and cerebrospinal fluid (CSF) shunting procedures. […] ONSF appears to be an effective procedure in patients who have progressive vision loss despite medical therapy. […] CSF shunting procedures include ventriculoperitoneal shunt (VPS) or lumboperitoneal shunt (LPS). […] Venous sinus stenting is a relatively new and somewhat controversial treatment option for IIH. […] 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.

• #4 Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) – EyeWiki

  https://eyewiki.org/Pseudotumor_Cerebri_(Idiopathic_Intracranial_Hypertension)

  Among the options available, acetazolamide, a carbonic anhydrase inhibitors, is believed to reduce the rate of CSF production and is the first-line medical treatment for IIH. […] Surgical management should be the option of choice among patients with refractory headaches or more severe/ rapidly progressive visual field loss when all other options have failed to prevent progressive visual loss. […] The two most used procedures are CSF diversion via shunt and optic nerve sheath fenestration. […] Optic nerve sheath fenestration is the preferred surgical procedure for papilledema with associated severe vision loss but no or minimal ICP symptoms (such as headache). […] CSF shunting produces rapid reduction in ICP and is therefore most beneficial among patients with vision loss and symptoms of raised ICP.

• #4 Idiopathic intracranial hypertension | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/idiopathic-intracranial-hypertension-1?lang=us

  Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a syndrome with signs and symptoms of increased intracranial pressure but where a causative mass or hydrocephalus is not identified. […] First-line treatment options include weight loss in patients with a BMI 30kg/m2, with weight loss of ~15% possibly being curative, and carbonic anhydrase inhibitors such as acetazolamide and topiramate. […] Invasive treatment options, usually reserved for refractory cases, include venous sinus stenting for transverse sinus stenosis, which is typically reserved for severe cases with a trans-stenotic gradient of 8 mmHg and has been increasingly shown to be effective. […] Venous sinus stenting has been reported to improve headache in ~80% of cases, tinnitus in ~95%, papilledema in ~90%, and visual symptoms in ~90%, with a reduction in opening pressure by ~15 cmH2O. […] Other invasive options include internal jugular venous decompression, bariatric surgery as a surgical weight loss strategy, optic nerve sheath fenestration (only if vision is acutely threatened in fulminant idiopathic intracranial hypertension), and serial CSF letting or CSF shunting.

• #4 Idiopathic Intracranial Hypertension (IIH) Treatment & Management: Approach Considerations, Pharmacologic Therapy, Optic Nerve Sheath Fenestration, CSF Diversion, and Venous Sinus Stenting

  https://emedicine.medscape.com/article/1214410-treatment

  Management guidelines are based on symptoms and extent of visual impairment at presentation. If there is no immediate threat to vision, medical therapy is recommended. In the event of an immediate threat to visual function, a temporary CSF draining procedure (ie, placement of a lumbar drain) is immediately performed, and a definitive surgical plan is made; either a ventriculoperitoneal shunt or optic nerve sheath fenestration. […] The primary treatment objectives for patients with idiopathic intracranial hypertension (IIH) are to preserve optic nerve function, manage elevated intracranial pressure (ICP), and alleviate symptoms. […] The initial treatment typically involves administering acetazolamide, dosed according to the patient’s symptoms, tolerance, and visual function. If progressive visual field loss occurs despite maximal medical therapy, urgent surgical intervention may be necessary.

• #4 Intracranial Hypertension or Pseudotumor Cerebri: The Basics – Migraine Canada

  https://migrainecanada.org/intracranial-hypertension-or-pseudotumor-cerebri-the-basics/

  Idiopathic Intracranial Hypertension (IIH), also known as pseudotumor cerebri, is a condition where the pressure inside the skull increases without an obvious cause. […] Losing weight, even as little as 10% of body weight, can significantly improve symptoms and protect vision. Weight loss is challenging, but crucial for managing IIH. […] In severe cases where vision is at risk, surgical shunting may be considered. This involves placing a tube to drain fluid from the brain ventricles to the abdomen (ventriculo-peritoneal shunt) or from the spine to the abdomen (lumbo-peritoneal shunt). These procedures carry high risks and should only be performed by experts. […] Physicians may recommend treatments like topiramate and Botox for headache control in these patients. […] Acetazolamide is a diuretic that can improve vision but may not be as effective for headaches. Side effects include nausea, tingling in extremities, and fatigue.

• #4 Idiopathic intracranial hypertension: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000351.htm

  Treatment is aimed at the cause of IIH. The main goal of treatments is to preserve vision and reduce the severity of headaches. […] A lumbar puncture (spinal tap) can help relieve pressure in the brain and prevent vision problems. Repeat lumbar punctures are helpful for pregnant women in order to delay surgery until after delivery. […] Other treatments may include: fluid or salt restriction, medicines such as corticosteroids, acetazolamide, furosemide, and topiramate, shunting procedures to relieve pressure from spinal fluid buildup, surgery to relieve pressure on the optic nerve, weight loss, treatment of the underlying disease, such as vitamin A overdose. […] People will need to have their vision closely monitored. There can be vision loss, which is sometimes permanent. Follow-up MRI or CT scans may be done to rule out problems such as tumors or hydrocephalus (buildup of fluid inside the skull). […] In some cases, the pressure inside the brain remains high for many years. Symptoms can return in some people. A small number of people have symptoms that slowly get worse and lead to blindness.

• #4 DigitalCommons@PCOM – Research Day: A holistic approach to idiopathic intracranial hypertension treatment and management

  https://digitalcommons.pcom.edu/research_day/research_day_GA_2024/researchGA2024/55/

  A holistic approach to idiopathic intracranial hypertension treatment and management […] Effective IIH management requires a comprehensive care team to ensure that the patient feels supported in all aspects of health management. This approach should have a PCP serving as the center of a coordinated care model encompassing various resources (e.g., mental health professionals, dietitians, osteopathic manipulative treatment.) Emphasizing the importance of a holistic strategy that addresses both mental and physical aspects of the disease can improve positive outcomes in patient care and disease management. Until further research sheds light on the underlying pathophysiology of IIH, the focus should shift towards establishing holistic treatment protocols and developing multifaceted care teams to promote the best outcomes for patients. This approach will empower patients and allow them to foster a sense of control in the face of this challenging condition.

• #4 Idiopathic intracranial hypertension – Wikipedia

  https://en.wikipedia.org/wiki/Idiopathic_intracranial_hypertension

  Treatment includes a healthy diet, salt restriction, and exercise. […] The medication acetazolamide may also be used along with the above measures. […] A small percentage of people may require surgery to relieve the pressure. […] The primary goal in treatment of IIH is the prevention of visual loss and blindness, as well as symptom control. […] IIH is treated mainly through the reduction of CSF pressure and IIH may resolve after initial treatment, may go into spontaneous remission (although it can still relapse at a later stage), or may continue chronically. […] There are three main treatment approaches: weight loss, different medications and surgical interventions. […] The first step in symptom control is drainage of cerebrospinal fluid by lumbar puncture. […] The best-studied medical treatment for intracranial hypertension is acetazolamide (Diamox), which acts by inhibiting the enzyme carbonic anhydrase, and it reduces CSF production by six to 57 percent.

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