The presenting features are [8]:

• Headache: The most common symptom, almost universally present. It is generalized, throbbing, worse on waking, and aggravated by factors which temporarily increase cerebrospinal fluid pressure such as straining, coughing, or changing posture. 50 % of patients complain of nausea.
• Obesity: Up to 90 per cent of patients are overweight.
• Papilledema: Universally present. Fundus examination of every patient with headache is vital.
• Visual symptoms: Visual obscurations, persistent blurring, or scotomas are reported by 30 to 70 % of patients. Washed out vision, halos and flickering lights lasting for a few seconds may be present. Excess straining and posture change can provoke obscurations, but they can also occur spontaneously. Occasionally, sudden and permanent loss of vision results from infarction of the optic nerve. About 30 per cent of patients complain of horizontal diplopia due to sixth nerve palsy, which may be bilateral. The cause is a false localizing sign of raised intracranial pressure.

• Fatigue

  […] chronic fatigue syndrome would represent the most common and least severe and IIH the least common and most extreme. [ncbi.nlm.nih.gov]

  We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: January 31, 2019 [patientslikeme.com]

  […] diseases classified elsewhere G91.8 Other hydrocephalus G91.9 Hydrocephalus, unspecified G92 Toxic encephalopathy G93 Other disorders of brain G93.1 Anoxic brain damage, not elsewhere classified G93.2 Benign intracranial hypertension G93.3 Postviral fatigue [icd10data.com]

• Arm Pain

  Other symptoms of IIH may include pulsatile tinnitus and shoulder or arm pain. Palsies may be seen involving cranial nerves VI, VII and VIII.(2) The worldwide epidemic of obesity is certain to increase the prevalence of this disorder. [acatoday.org]

  Other common symptoms include transient altered vision, particularly on movement or bending over, intracranial noise (pulse synchronous tinnitus), stiff neck, back and arm pain, pain behind the eye, exercise intolerance, and memory difficulties. [rarediseases.org]

  Other symptoms of IIH include pulse-synchronous tinnitus, and shoulder or arm pain [ 2, 3 ]. Ophthalmologic signs of IIH include decreased visual acuity, visual field loss, and papilledema. [karger.com]

• Sneezing

  It is most severe in mornings and worsens with coughing or sneezing. [cafamily.org.uk]

  […] may be worse in the morning, or when coughing or straining; it may improve when standing up blurred or double vision temporary loss of vision – your vision may become dark or "greyed out" for a few seconds at a time; this can be triggered by coughing, sneezing [nhs.uk]

  The headache can be made worse by any activity that further increases the intracranial pressure, such as coughing and sneezing. The pain may also be experienced in the neck and shoulders. [en.wikipedia.org]

  The headache can be made worse by any activity that further increases the intracranial pressure, such as coughing and sneezing. [psychology.wikia.com]

• Hyposmia

  Do patients with idiopathic intracranial hypertension suffer from hyposmia? Med Hypotheses. 2008;71(5):816–817. 24. Khoo KF, Kunte H. Olfactory dysfunction in patients with idiopathic intracranial hypertension. Neurology. 2014;82(2):189. 25. [dovepress.com]

• Abnormal Breathing

  breathing during sleep marked by phases of paused breathing Addison’s disease, which is a disorder in which your adrenal glands don’t produce enough hormones Lyme disease, which is a chronic flu-like disease caused by a bacterium carried by ticks A birth [healthline.com]

• Nausea

  Nausea, often treated with 5-hydroxytryptamine (5-HT3) receptor antagonists, approved for perioperative and chemotherapy-induced nausea, are used off-label to treat nausea and vomiting outside of those settings. [ncbi.nlm.nih.gov]

• Vomiting

  Summary A 27-year-old woman with a history of recurrent nausea and vomiting in the setting of idiopathic intracranial hypertension (IIH) was admitted for control of unremitting nausea and vomiting. [ncbi.nlm.nih.gov]

• Hypertension

  Idiopathic intracranial hypertension and the idiopathic intracranial hypertension treatment trial. J Neuro-Ophthalmol 2013 ; 33: 1 – 3. Google Scholar | Crossref | Medline | ISI [doi.org]

  Idiopathic intracranial hypertension refers to cases of intracranial hypertension and normal brain parenchyma without ventriculomegaly or any kind of mass lesion. [ncbi.nlm.nih.gov]

  IIH UK - Idiopathic Intracranial Hypertension Ball AK, Clarke CE ; Idiopathic intracranial hypertension. Lancet Neurol. 2006 May5(5):433-42. Friedman DI ; Idiopathic intracranial hypertension. Curr Pain Headache Rep. 2007 Feb11(1):62-8. [patient.info]

• Neck Pain

  April Newsletter, Lewitt Headache & Neck Pain Clinic, PLLC, Greensboro, NC [earcentergreensboro.com]

  Positive acetazolamide-related effects on QOL appeared to be primarily mediated by improvements in visual field, neck pain, pulsatile tinnitus, and dizziness/vertigo that outweighed the side effects of acetazolamide. [ncbi.nlm.nih.gov]

  pain Blurred vision Buzzing sound in the ears (tinnitus) Dizziness Double vision (diplopia) Nausea, vomiting Vision problems such as flashing or even loss of vision Low back pain, radiating along both legs Headaches may get worse during physical activity [nlm.nih.gov]

  […] behind the eyes or when moving the eyes Pulse synchronous tinnitus (described as a swooshing sound in sync with the patient’s heartbeat) Tinnitus Hearing loss Shoulder, back, and/or neck pain Ophthalmoplegia (paralysis of extraocular muscles) There are [medtronic.com]

  […] shoulder and/or neck pain tinnitus vomiting Diagnosis of IIH: The only truly definitive diagnostic tests for IIH are a lumbar puncture (LP) (spinal tap) or or by epidural or intraventricular pressure monitoring (CSF pressure monitoring). [healthcentral.com]

• Back Pain

  Transient visual obscurations, back pain, and tinnitus (pulse synchronized and non-pulsatile) were also commonly reported. [2minutemedicine.com]

  Everyone with BIH has different sets of symptoms, but common ones include headache, visual disturbances, dizziness, back pain, nausea and vomiting, memory problems and a dislike of bright light. [netdoctor.co.uk]

  Her low-back pain, diagnosed as facet syndrome, resolved with a course of spinal manipulation. [acatoday.org]

  Pulsatile tinnitus Cranial nerve palsies (especially CN VI ) Back pain References: [3] [4] Diagnostics The following tests should always be performed Ophthalmologic examination Opthalmoscopy: bilateral papilledema Visual field test may show peripheral [amboss.com]

• Muscle Weakness

  weakness lasix (furosemide) indications when acetazolamide isn't working well enough outcomes reduce fluids by increasing urine output Operative spinal taps or shunts outcomes symptoms can immediately improve by reducing CSF pressure optic nerve sheath [step2.medbullets.com]

  At the time of hospitalization, her weight was 63 kg and her height, 158 cm, and she had severe generalized headache, nausea, muscle weakness, hyperreflexia, and photophobia. [jamanetwork.com]

  The facial nerve (seventh cranial nerve) is affected occasionally –- the result is total or partial weakness of the muscles of facial expression on one or both sides of the face.The increased pressure leads to papilledema, which is swelling of the optic [en.wikipedia.org]

  It can cause the symptoms of hypokalemia (low blood potassium levels), which include muscle weakness and tingling in the fingers. [psychology.wikia.com]

  They may increase muscle weakness in myasthenia gravis and exacerbate systemic lupus erythematosus, antacids containing aluminium and calcium reduce the absorption of all tetracyclines, and dairy products reduce absorption greatly for all but minocycline [wikivisually.com]

• Shoulder Pain

  Postpartum headache is described as headache and neck or shoulder pain during the first 6 weeks after delivery. [ncbi.nlm.nih.gov]

  Other common symptoms include pulsatile tinnitus (ringing in the ears) and neck and shoulder pain. Intracranial hypertension can be either acute or chronic. [nei.nih.gov]

  The pain may also be experienced in the neck and shoulders. Many have pulsatile tinnitus, a whooshing sensation in one or both ears (64–87%); this sound is synchronous with the pulse. [en.wikipedia.org]

  The pain may also be experienced in the neck and shoulders. [5] Many have pulsatile tinnitus, a whooshing sensation in one or both ears (64–87%); this sound is synchronous with the pulse. [5] [6] Various other symptoms, such as numbness of the extremities [en.m.wikipedia.org]

  Evan Johnson Tackles Puzzling Neck and Shoulder Pain at the North American Spine Society Dr. Evan Johnson Talks Back Pain and More at NASS 2018 Dr. Evan Johnson Talks Hip And Back Pain at NASS Annual Meeting Dr. [columbianeurosurgery.org]

• Tinnitus

  Patients without pulsatile tinnitus were excluded. Tinnitus severity was categorized based on "Tinnitus Handicap Inventory" (THI) at pre-stent, day-0, 1-month, 3-month, 6-month, 12-month, 18-month and 2-year follow-up. [ncbi.nlm.nih.gov]

• Pulsatile Tinnitus

  Patients without pulsatile tinnitus were excluded. Tinnitus severity was categorized based on "Tinnitus Handicap Inventory" (THI) at pre-stent, day-0, 1-month, 3-month, 6-month, 12-month, 18-month and 2-year follow-up. [ncbi.nlm.nih.gov]

  The most common symptoms are diffuse headaches, although various visual symptoms and pulsatile tinnitus are also common. [amboss.com]

• Hearing Impairment

  Among audiovestibular symptoms, tinnitus and dizziness are commonly reported, while vertigo and hearing impairment are infrequent reports. [ncbi.nlm.nih.gov]

• Diplopia

  Idiopathic intracranial hypertension (IIH)-sometimes called pseudotumor cerebri-is a neurologic condition distinguished by any of the following symptoms: headache, increased cerebrospinal fluid pressure, papilledema, vision loss, diplopia, tinnitus, deafness [ncbi.nlm.nih.gov]

• Blurred Vision

  The symptoms of the disease may be ordered according to prevalence as follows: headache due to increased intracranial pressure, blurred vision and diplopia. [ncbi.nlm.nih.gov]

• Visual Impairment

  In patients who have severe visual impairment or progressive visual deterioration despite medical management, surgical or interventional treatment may be considered. [dovepress.com]

  It can lead to visual impairment but prompt diagnosis and treatment in most of the cases will prevent potentially permanent visual loss. [ncbi.nlm.nih.gov]

• Enlarged Blind Spot

  The most common visual field defect was a partial arcuate defect with an enlarged blind spot, and the average (SD) CSF opening pressure was 343.5 (86.9) mm H 2 O. [2minutemedicine.com]

  Results of ophthalmologic examination revealed bilateral papilledema, an enlarged blind spot in both eyes, and a normal visual acuity. [jamanetwork.com]

  Intracranial hypertension fulfilling the following criteria: alert patient with neurological examination that either is normal or demonstrates any of the following abnormalities: a) papilloedema b) enlarged blind spot c) visual field defect (progressive [healthcentral.com]

• Photophobia

  We present a 38-year old primigravid renal transplant patient at 7 weeks of gestation who presented with 2 weeks of intense, throbbing, holocranial headache, nausea, vomiting, photophobia, diplopia and progressive visual loss. [ncbi.nlm.nih.gov]

  The patient is a 37 y/o female who presents with complaints of two months of intermittent severe frontal headaches associated with some decreased vision, nausea/vomiting and photophobia. [ultrasoundoftheweek.com]

  […] which may be worse in the morning (the most common symptom) Dizziness Nausea Transient visual obscurations characteristic of papilledema Papilledema causing impaired vision, especially peripheral visual field defects and enlargement of the blind spot Photophobia [medtronic.com]

  Nausea, vomiting Change in vision (blurred, double vision [CN palsy], photophobia Papilledema Mild blurring of the disc to gross edema with hemorrhages. In infants with open fontanel, there may be no papilledema but rather buldging fontanel. [pedemmorsels.com]

  Eye pain, photophobia, blurry vision, and nausea/vomiting are all common symptoms in IIH, but these symptoms are also present in other causes of headache. [mdedge.com]

• Headache

  Postpartum headache is described as headache and neck or shoulder pain during the first 6 weeks after delivery. [ncbi.nlm.nih.gov]

  Some with a “mixed headache syndrome” are able to differentiate between the continuous daily headache of BIH which is worse on awaking from associated more severe, but intermittent, migrainous headaches. [dx.doi.org]

• Papilledema

  This article reviews the treatment of papilledema-related visual loss in pseudotumor cerebri syndrome, one of the most common causes of papilledema encountered by neurologists. [ncbi.nlm.nih.gov]

  In all 6 patients requiring re-stent placement, papilledema resolved after the first stent; while headache recurred in all 6, papilledema recurred in only 4. [dx.doi.org]

• Meningism

  Throughout history, many terms have been used, including serous meningitis, pseudotumor cerebri, otitic hydrocephalus, angioneurotic hydrocephalus, toxic hydrocephalus, meningeal hypertension, hypertensive meningeal hydrops, pseudoabscess, intracranial [ncbi.nlm.nih.gov]

• Dizziness

  Among audiovestibular symptoms, tinnitus and dizziness are commonly reported, while vertigo and hearing impairment are infrequent reports. [ncbi.nlm.nih.gov]

  Symptom ... s may include a headache behind your eyes, ringing in your ears, nausea, vomiting, dizziness, blurred vision or double vision.The cause of pseudo tumor cerebri is unknown, but it is thought to be linked to an excess amount of cerebrospinal [sharecare.com]

  Everyone with BIH has different sets of symptoms, but common ones include headache, visual disturbances, dizziness, back pain, nausea and vomiting, memory problems and a dislike of bright light. [netdoctor.co.uk]

• Vertigo

  Among audiovestibular symptoms, tinnitus and dizziness are commonly reported, while vertigo and hearing impairment are infrequent reports. [ncbi.nlm.nih.gov]

• Visual assessment: Visual acuity, color vision, motility assessment and fundus examination
• Visual field: The most common defects are enlargement of the blind spots, generalized constriction of the fields, and scotomas caused by optic nerve damage. There may be a predilection for visual field loss in the inferior nasal quadrants. 
• Fundus fluorescein angiography: Is necessary if fundus examination is not equivocal for diagnosis of papilledema.
• Radiology: For detecting underlying pathology and especially ruling out mass lesions and hydrocephalus before doing lumbar puncture. Computed tomography (CT) scanning shows small and slit-like cerebral ventricles which may increase in volume as intracranial hypertension resolves. A similar appearance is seen on magnetic resonance imaging (MRI). Sagittal sinus thrombosis may be visualized on CT scanning as the characteristic 'empty delta' sign due to clot within the sinus. MRI is superior to CT and provides graphic images of sinus thrombosis. Occasionally MR or CT angiography may be needed to exclude sinus thrombosis or conventional venography if thrombolytic therapy is contemplated.
• Lumbar puncture: To acquire CSF sample, to measure CSF pressure and establish presence of raised pressure. At lumbar puncture the opening pressure is greater than 200 mm cerebrospinal fluid. In simple obesity the cerebrospinal fluid pressure may be as high as 250 mm; therefore the diagnostic significance of cerebrospinal fluid pressure must therefore be correlated with the clinical picture. In the few patients whose cerebrospinal fluid pressure is equivocal, continuous monitoring may demonstrate intermittent peaks of raised pressure.
• Cerebrospinal fluid analysis: The composition of the cerebrospinal fluid is entirely normal, and the presence of white cells or a raised protein concentration cast serious doubt on the diagnosis. CSF is analyzed for white blood cell and differential counts, red blood cell count, glucose, total protein, quantitative protein electrophoresis, microbial microscopy and culture for aerobic bacteria and acid-fast bacilli, cytology, cryptococcal antigens, and syphilis markers.
• Blood analysis: Complete blood count, serum iron and iron-binding capacity, erythrocyte sedimentation rate, procoagulant profile (in patients with a previous history of thrombosis or MRI evidence of dural venous sinus occlusion on MRI), antinuclear antigen (ANA) profile (e.g. anti-dsDNA and anti-ssDNA), Lyme screening test if the history is suggestive.

• Cerebrospinal Fluid Abnormality

  Cerebrospinal fluid abnormalities and elevated opening pressures have not been well documented in patients with posterior scleritis, perhaps because these patients do not frequently undergo CSF analysis. [neuroophthalmology.ca]

• Central Scotoma

  A bedside ocular examination showed 20/100 in both eyes while using glasses; no visual field cuts or obvious central scotoma was present. The patient was alert and oriented to time and place. [mdedge.com]

  Central scotomas, inferior nasal defects, and peripheral constriction are the next most common field defects. 28 It is still unclear which factors predispose to permanent visual loss. [dx.doi.org]

Weight loss is strongly recommended with exercise regime and diet management.

Pharmacologic therapy [9]:

• Acetazolamide and furosemide
• Amitriptyline or propranolol for the prophylaxis of headache
• Corticosteroids are added in case of inflammatory etiology

Surgical approach [10]:

• If visual function continues to deteriorate, optic nerve sheath fenestration (decompression), lumboperitoneal or ventriculoperitoneal shunts to divert cerebrospinal fluid and intracranial venous sinus stenting may be attempted.

Idiopathic intracranial hypertension does not increase the mortality risk. The papilledema may lead to irreversible optic neuropathy with accompanying constriction of the visual field and loss of color vision. Prolonged papilledema can adversely affect the central visual acuity as well. Timely diagnosis and treatment can preserve vision [7].

In the majority of patients with idiopathic intracranial hypertension, no cause can be identified. The most commonly affected patients are obese young women. A higher body mass indexes (BMIs) and recent weight gain is associated with the increased risk, but the causative mechanisms are not clear. Pregnancy and menstrual irregularity are also risk factors [2].

In absence of obesity, other risk factors associated with idiopathic intracranial hypertension are:

• Drugs: Exposure or withdrawal of certain exogenous substances, like corticosteroids, vitamin A (> 100,000 U/day)/retinoic acid, antibiotics, carbidopa, levodopa, amiodarone, indomethacin, levonorgestrel implants, oral contraceptives, cyclosporine, danazol, growth hormone, lithium, and phenytoin.
• Systemic diseases: Anemia, chronic respiratory insufficiency, hypertension, multiple sclerosis, Lyme disease, psittacosis, chronic renal disease, thrombocytopenic purpura, sarcoidosis, systemic lupus erythematosus and Reye syndrome.
• Disruption of cerebral venous flow: An impaired absorption of cerebrospinal fluid (CSF) may result from any compression of cerebral veins (extravascular tumors, secondary thrombosis due to coagulopathy) or a venous flow anomaly.
• Preceding minor head injury has shown some association.
• It has been suggested that an empty sella may be associated with about 4 % of cases. There is raised intracranial pressure in combination with incompetence of the diaphragm sellae. Clinical hypopituitarism does not occur, but occasionally the empty sella may harbor a prolactinoma [3].

The incidence of idiopathic intracranial hypertension in the United States has been estimated to range from 0.9 to 1.0 per 100,000 in the general population. The female preponderance over males ranges from 3:1 to 8:1. The incidence in women is 1.6-3.5 per 100,000 overall, with an incidence of 7.9-20 per 100,000 in overweight women of childbearing age (17 to 44 years). Very rarely, it is familial and may occur in more than one generation. Although men have a lower incidence, they are twice as likely as women to lose visual function as a result of papilledema [4].

The mechanism by which intracranial pressure rises is poorly understood and the contribution of various factors controversial. Since the intracranial contents are housed in a rigid container, an increase in cerebrospinal fluid pressure in theory may result from an increase in blood volume, swelling of the brain parenchyma, or an increase in the cerebrospinal fluid volume due to overproduction or malabsorption. However, there is little evidence to suggest that increased blood volume or cerebrospinal fluid production have any causative role [5].

Although idiopathic intracranial hypertension occurs more in overweight women, the role of obesity is not clear. In some cases, a familial link between obesity and idiopathic intracranial hypertension has been documented. Obesity may increase intraabdominal pressure leading to raised cardiac filling pressures. This may result in impeded venous return from the brain [6]. The increased intracranial pressure interrupts the axoplasmic flow of the optic nerves giving rise to papilledema which may lead to irreversible optic neuropathy.

Obesity is strongly correlated to idiopathic intracranial hypertension and therefore, weight management can contribute to prevention of this condition in many people. A high index of suspicion in overweight women and pregnant women complaining of visual disturbance is important for timely diagnosis and management.

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri or benign intracranial hypertension (BIH), is a syndrome of raised intracranial pressure occurring in the absence of an intracranial mass lesion or enlargement of the cerebral ventricles due to hydrocephalus. The condition occurs predominantly in obese women of childbearing age. The chronically elevated intracranial pressure leads to symptoms of headache and visual loss, with papilledema, which may lead to progressive optic atrophy and blindness [1]. It was previously called benign intracranial hypertension but the term is not preferred anymore because of the significant morbidity associated with the condition.

Definition: Idiopathic intracranial hypertension, also known as pseudotumor cerebri, is a condition in which the pressure of the fluid inside the skull is increased leading to damage to the optic nerves.

Cause: The cause is unknown, however, it occurs more in overweight women, pregnancy, and is associated with deficiency and excess of vitamin A, and some other drugs, inflammatory conditions and infectious diseases.

Symptoms: Headache, nausea, washed out vision with loss of vividness of colors and transient obscuration of vision is common. Headache may be mild to severe, and may increase with bending or coughing.

Diagnosis: Vision is tested and the presence of increased cerebrospinal fluid pressure is established by examining the optic nerve at the back of the eye with an instrument called ophthalmoscope and by examining the fluid pressure with a needle inserted in the lower part of the spine in the back.

Treatment and follow-up: Weight reduction is an important aspect of treatment of this condition. Medications are given to reduce the intracranial pressure. If the raised pressure does not respond to medication and the optic nerve is in danger of permanent damage, surgery may be done to reduce the pressure in the skull and within the optic nerve.

1. Wall M. Idiopathic intracranial hypertension (pseudotumor cerebri). Curr Neurol Neurosci Rep. Mar 2008;8(2):87-93.
2. Friedman DI, Jacobson DM. Idiopathic intracranial hypertension. J Neuroophthalmol. Jun 2004; 24(2):138-45.
3. Degnan AJ, Levy LM. Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol. 2011; 32(11):1986-93.
4. Radhakrishnan K, Ahlskog JE, Cross SA, Kurland LT, O'Fallon WM. Idiopathic intracranial hypertension (pseudotumor cerebri). Descriptive epidemiology in Rochester, Minn, 1976 to 1990. Arch Neurol. Jan 1993; 50(1):78-80.
5. Ireland B, Corbett JJ, Wallace RB. The search for causes of idiopathic intracranial hypertension. A preliminary case-control study. Arch Neurol. 1990; 47:315.
6. Increased intra-abdominal pressure and cardiac filling pressures in obesity-associated pseudotumor cerebri. Neurology. 1997; 49(2):507.
7. Rowe FJ, Sarkies NJ. Visual outcome in a prospective study of idiopathic intracranial hypertension. Arch Ophthalmol 1999; 117:1571.
8. Giuseffi V, Wall M, Siegel PZ, Rojas PB. Symptoms and disease associations in idiopathic intracranial hypertension (pseudotumor cerebri): a case-control study. Neurology 1991; 41:239.
9. Johnson LN, Krohel GB, Madsen RW, March GA Jr. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri). Ophthalmology. 1998; 105:2313.
10. Feldon SE. Visual outcomes comparing surgical techniques for management of severe idiopathic intracranial hypertension. Neurosurg Focus 2007; 23:E6.