Edit concept Question Editor Create issue ticket

Idiopathic Intracranial Hypertension

Pseudotumor Cerebri

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri or benign intracranial hypertension (BIH), is a disorder characterized by chronically elevated intracranial pressure and signs and symptoms resulting from the raised intracranial pressure in the absence of any detectable underlying causative lesion or pathology. 


Presentation

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]
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 28, 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 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]
Intermittent Fever
  • A 14-year-old girl was referred for evaluation of headache with episodes of transient blurring of vision, and intermittent fever for 4 weeks.[ncbi.nlm.nih.gov]
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]
  • […] 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, nausea[ncbi.nlm.nih.gov]
  • Our case lacked the typical symptoms of IIH, such as headache or nausea; therefore, it is necessary to carefully determine the cause of bilateral abducens nerve palsies.[ncbi.nlm.nih.gov]
  • Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is often a debilitating condition characterized by headaches, blurry vision, nausea, and vomiting.[ncbi.nlm.nih.gov]
  • 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]
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]
  • […] 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, nausea and vomiting[ncbi.nlm.nih.gov]
  • Idiopathic intracranial hypertension (IIH) is a syndrome of increased intracranial pressure and presents as an intractable headache, vomiting, and ophthalmologic manifestations.[ncbi.nlm.nih.gov]
  • Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is often a debilitating condition characterized by headaches, blurry vision, nausea, and vomiting.[ncbi.nlm.nih.gov]
  • Patient A presented with vomiting and examination showed ophthalmoplaegia and papilloedema. She was treated with acetazolamide, furosemide and therapeutic lumbar punctures.[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]
  • hypertension of unknown cause, papilledema of indeterminate etiology, intracranial pressure without brain tumor, benign intracranial hypertension, and idiopathic intracranial hypertension.[oadoi.org]
Skin Lesion
  • On examination she was conscious and febrile, with multiple annular purpuric skin lesions present over the face and back. Neurological examination revealed a bilaterally extensor plantar response, with bilateral papilloedema.[ncbi.nlm.nih.gov]
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]
  • Among audiovestibular symptoms, tinnitus and dizziness are commonly reported, while vertigo and hearing impairment are infrequent reports.[ncbi.nlm.nih.gov]
  • This was accompanied by headaches, otalgia, tinnitus, and vertigo.[ncbi.nlm.nih.gov]
  • 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]
  • The typical patient symptom profile is the presence of daily headache, pulse synchronous tinnitus, transient visual obscurations, and papilledema with its associated visual loss.[ncbi.nlm.nih.gov]
Pulsatile Tinnitus
  • OBJECTIVE: Evaluate the role of venous sinus stenting in the treatment of pulsatile tinnitus among patients with Idiopathic Intracranial Hypertension (IIH) and significant venous sinus stenosis.[ncbi.nlm.nih.gov]
  • PURPOSE: This aimed to evaluate the prevalence and extent of bilateral sigmoid sinus dehiscence (SSD) and to explore the presence of idiopathic intracranial hypertension (IIH) in patients with unilateral pulsatile tinnitus (PT) with CTA/V.[ncbi.nlm.nih.gov]
  • Idiopathic intracranial hypertension (IIH) is defined by elevated intracranial pressure and associated headaches, changes in vision and pulsatile tinnitus, among other symptoms. It occurs most frequently in young, obese women.[ncbi.nlm.nih.gov]
  • Undiagnosed patients may present to otolaryngologists with pulsatile tinnitus, dizziness, sleep apnea, and spontaneous cerebrospinal fluid leaks.[ncbi.nlm.nih.gov]
Hearing Impairment
  • Among audiovestibular symptoms, tinnitus and dizziness are commonly reported, while vertigo and hearing impairment are infrequent reports.[ncbi.nlm.nih.gov]
Neck Pain
  • 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]
  • April Newsletter, Lewitt Headache & Neck Pain Clinic, PLLC, Greensboro, NC[earcentergreensboro.com]
  • Pregnancy Sarcoidosis (inflammation of the lymph nodes, lungs, liver, eyes, skin, or other tissues) Systemic lupus erythematosis Turner syndrome Symptoms may include any of the following: Headaches, throbbing, daily, irregular and worse in the morning Neck[nlm.nih.gov]
  • pain Ophthalmoplegia (paralysis of extraocular muscles) There are four criteria for diagnosing IIH (Modified Dandy Criteria) 5 : High intracranial pressure (ICP).[medtronic.com]
  • 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]
  • pain References: [3] [4] Diagnostics The following tests should always be performed Ophthalmologic examination Opthalmoscopy: bilateral papilledema Visual field test may show peripheral loss of vision MRI Lumbar puncture Elevated opening pressure 20–[amboss.com]
  • pain, radiating along both legs Headaches may get worse during physical activity, especially when you tighten the stomach muscles during coughing or straining.[nlm.nih.gov]
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 main symptoms are headache, vision problems, ringing in the ears with the heartbeat, and shoulder pain. Complications may include vision loss.Risk factors include being overweight or a recent increase in weight.[en.wikipedia.org]
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]
  • This study reports the case of a 23-year-old man with idiopathic intracranial hypertension (IIH) who presented with blurred vision and diplopia, without accompanying headache.[ncbi.nlm.nih.gov]
  • 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]
  • One patient had horizontal diplopia due to an acute sixth nerve palsy along with severe constriction of visual fields while the second patient had symptoms of blurred vision.[ncbi.nlm.nih.gov]
  • 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]
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]
  • This study reports the case of a 23-year-old man with idiopathic intracranial hypertension (IIH) who presented with blurred vision and diplopia, without accompanying headache.[ncbi.nlm.nih.gov]
  • We report a case of 33year-old obese female (BMI 36.9) who presented with a severe headache and blurred vision for one week, found to have idiopathic intracranial hypertension with rapid worsening of symptoms suggestive of a fulminant course of disease[ncbi.nlm.nih.gov]
  • A 14 year-old Caucasian boy with idiopathic intracranial hypertension (IIH) presented with blurred vision in his left eye. Visual acuity was 20/20, right eye, and 20/80, left eye, and funduscopy revealed bilateral papilledema.[ncbi.nlm.nih.gov]
  • One patient had horizontal diplopia due to an acute sixth nerve palsy along with severe constriction of visual fields while the second patient had symptoms of blurred vision.[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]
  • Visual field charting: enlarged blind spot and peripheral field construction. Lumbar puncture, if not contra-indicated by clinical features and pressure measurement. Monitor intracranial pressure if in doubt as to the diagnosis.[patient.info]
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]
Scotoma
  • 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.[symptoma.com]
  • In addition, the monocular visual disturbances, called scotoma, and any loss of vision experienced must last less than an hour and be reversible and confirmed by a doctor's examination the during attack or just after.[livestrong.com]
  • Transient reduction of vision ('greying out') on bending or stooping, halo or a short episode of visual Catherine wheel flashes, persistent blurring, scotoma or horizontal diplopia may also occur. Nausea, vomiting, drowsiness.[patient.info]
  • 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]
Headache
  • The most common headache phenotypes were migraine (52%), tension-type headache (22%), probable migraine (16%), and probable tension-type headache (4%).[ncbi.nlm.nih.gov]
  • Postpartum headache is described as headache and neck or shoulder pain during the first 6 weeks after delivery.[ncbi.nlm.nih.gov]
  • We present a case report of a 29-year-old female with a maximum BMI of 50.3 and a 5-year history of severe headaches and moderate papilledema due to IIH. She also developed migraine headaches.[ncbi.nlm.nih.gov]
  • Headache frequency as well as ELH symptoms and PTA significantly improved after LP.[ncbi.nlm.nih.gov]
  • Headache. 2011 Nov-Dec;51(10):1537-9. doi: 10.1111/j.1526-4610.2011.01971.x. Epub 2011 Aug 29.[ncbi.nlm.nih.gov]
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]
  • We report a case of a 28-year old female with HIV on antiretroviral therapy, who presented to us, with papilledema. Her fundus examination revealed superficial hemorrhages and Roth's spots along with papilledema.[ncbi.nlm.nih.gov]
  • When papilledema is absent, the diagnosis becomes less clear. Some physicians have argued that the absence of papilledema rules out IIH, whereas others maintain that elevated OP is sufficient for diagnosis.[ncbi.nlm.nih.gov]
  • Resolution of RF, but not of PPW and CF, was associated with significant reduction in RNFL thickness, ONH volume, and papilledema grade.[ncbi.nlm.nih.gov]
  • It is associated with papilledema 348.2 Excludes hypertensive encephalopathy ( 437.2 ) ICD-9-CM Volume 2 Index entries containing back-references to 348.2 :[icd9data.com]
Dizziness
  • Among audiovestibular symptoms, tinnitus and dizziness are commonly reported, while vertigo and hearing impairment are infrequent reports.[ncbi.nlm.nih.gov]
  • Undiagnosed patients may present to otolaryngologists with pulsatile tinnitus, dizziness, sleep apnea, and spontaneous cerebrospinal fluid leaks.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • This was accompanied by headaches, otalgia, tinnitus, and vertigo.[ncbi.nlm.nih.gov]
  • 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]
  • The electronic medical records were mined for presence of the following: body mass index, age, headache, vision changes, tinnitus, and vertigo.[ncbi.nlm.nih.gov]
Nystagmus
  • Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is a variant syndrome of internuclear ophthalmoplegia, consisting of primary gaze exotropia, adduction impairment, nystagmus of the abducting eye, and vertical gaze-evoked nystagmus.[ncbi.nlm.nih.gov]
  • In this report, we describe the management of a child with bilateral cataract, nystagmus, and comitant sensory esotropia.[ncbi.nlm.nih.gov]
  • Other neurological abnormalities reported have included facial paresis, neck pain, seizures, hyperreflexia, bruit, hypoglossal nerve palsy, nystagmus, and choreiform movements, 10 11 but these features are sufficiently rare that diagnosis of BIH should[dx.doi.org]

Workup

  • 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.
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]

Treatment

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

Pharmacologic therapy [9]:

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.

Prognosis

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].

Etiology

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:

Epidemiology

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].

Sex distribution
Age distribution

Pathophysiology

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.

Prevention

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.

Summary

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.

Patient Information

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.

References

Article

  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.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2019-07-11 20:00