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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.
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]
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: November 30, 2018[patientslikeme.com]
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: November 22, 2018[patientslikeme.com]
  • In one study of 185 participants, people in the acetalomazide group had an increased risk of decreased CO 2 , diarrhoea, dysgeusia , fatigue, nausea, paresthesia, tinnitus and vomiting compared to people in the placebo group.[cochrane.org]
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 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]
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 the case of a 22-year-old woman who presented with worsening headache, nausea, vomiting, blurry vision, diplopia, visual loss, and facial numbness 14 weeks after surgical resection of adrenocorticotropic hormone-positive pituitary adenoma.[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
  • Previously described MR imaging signs of intracranial hypertension were identified in 8%-61% of patients with idiopathic intracranial hypertension. Correlation among the 3 modes of evaluation was highly significant (P .0001).[ncbi.nlm.nih.gov]
  • Idiopathic intracranial hypertension is a disease with uncertain etiology.[ncbi.nlm.nih.gov]
  • Idiopathic intracranial hypertension is a variety of intracranial hypertension that is extremely rare in men. Obesity and hypogonadism are the most important predictive factors.[ncbi.nlm.nih.gov]
Tinnitus
  • Pulsatile tinnitus is experienced by most patients with idiopathic intracranial hypertension.[ncbi.nlm.nih.gov]
  • 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]
Pulsatile Tinnitus
  • Pulsatile tinnitus is experienced by most patients with idiopathic intracranial hypertension.[ncbi.nlm.nih.gov]
  • Venous sinus stenting is an effective treatment for pulsatile tinnitus in patients with IIH and venous sinus stenosis.[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]
  • 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]
  • 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]
  • 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]
  • You have sudden neck pain or cannot move your arms or legs. You have sudden trouble breathing. You are confused or cannot think clearly. When should I seek immediate care? You have a severe headache. You have a seizure.[drugs.com]
Back Pain
  • Transient visual obscurations, back pain, and tinnitus (pulse synchronized and non-pulsatile) were also commonly reported.[2minutemedicine.com]
  • Her low-back pain, diagnosed as facet syndrome, resolved with a course of spinal manipulation.[acatoday.org]
  • 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 Causes The exact cause of pseudotumor cerebri in most individuals is unknown, but it may be linked to an excess amount of cerebrospinal fluid within the bony confines of your skull.[mayoclinic.org]
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]
  • pain. [1] [2] Complications may include vision loss . [2] Risk factors include being overweight or a recent increase in weight. [1] Tetracycline may also trigger the condition. [2] The diagnosis is based on symptoms and a high intracranial pressure found[en.wikipedia.org]
  • […] intracranial hypertension ( IIH ) is a condition characterized by increased intracranial pressure (pressure around the brain) without a detectable cause. [2] The main symptoms are headache , vision problems, ringing in the ears with the heartbeat , and shoulder[en.m.wikipedia.org]
  • Evan Johnson Tackles Puzzling Neck and Shoulder Pain at the North American Spine Society Dr. Evan Johnson Talks Hip And Back Pain at NASS Annual Meeting Dr. Feldstein a Parent Favorite with Patients’ Choice Award Dr.[columbianeurosurgery.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]
  • 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]
  • The 19-year-old woman presented with headache and diplopia. She was diagnosed with IIH since she was five years of age and had been non-responsive to lumbar cerebrospinal fluid (CSF) drainage and acetazolamide treatment.[ncbi.nlm.nih.gov]
  • We present the case of a 22-year-old woman who presented with worsening headache, nausea, vomiting, blurry vision, diplopia, visual loss, and facial numbness 14 weeks after surgical resection of adrenocorticotropic hormone-positive pituitary adenoma.[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]
  • 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]
  • 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]
  • 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]
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]
Photopsia
  • Pathophysiology A mismatch between production and resorption of CSF (cause unkown) ICP damage to structures of the CNS and especially to the optical nerve fibers References: [2] Clinical features Diffuse headaches Visual symptoms Transient vision loss Photopsia[amboss.com]
  • […] tinnitus) Nausea, vomiting or dizziness Blurred or dimmed vision Brief episodes of blindness, lasting only a few seconds and affecting one or both eyes (visual obscurations) Difficulty seeing to the side Double vision (diplopia) Seeing light flashes (photopsia[mayoclinic.org]
  • The presentation is usually with headaches, visual problems (transient or gradual visual loss), pulse-synchronous tinnitus , photopsia and eye pain 15 .[radiopaedia.org]
  • Ophthalmologic signs are frequent and include papilledema, transient loss of acuity (30 percent experience permanent loss), impaired visual fields, photopsias (flashes), diplopia and a painful eye.[acatoday.org]
Photophobia
  • […] 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]
  • 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]
  • Other presentations include anorexia, increasing head size, pain behind the orbit, photophobia, head tilt and preference for knee-chest position. Patients can also be asymptomatic with papilledema noticed incidentally on physical examination.[pediatriceducation.org]
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]
  • ; no band for high-pressure headaches.[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]
  • 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]
  • 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]
  • Body mass index, papilledema, visual acuity, and perimetric mean deviation were compared at the end of the 6-month study period.[ncbi.nlm.nih.gov]
  • The various types of retinal folds associated with papilledema reflect biodynamic processes and show an ACZ treatment effect.[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]
  • 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]
  • Symptoms Pseudotumor cerebri signs and symptoms may include: Moderate to severe headaches that may originate behind your eyes and worsen with eye movement Ringing in the ears that pulses in time with your heartbeat (pulsatile tinnitus) Nausea, vomiting or dizziness[mayoclinic.org]
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]
  • Several weeks before her visit, her headaches worsened and were accompanied by vertigo and visual disturbances. Seen in the emergency department with headache and reduced visual acuity, she was again given a diagnosis of chronic migraine.[acatoday.org]
Neck Stiffness
  • stiffness, painful shoulder joints, wrist joints and knees, difficulty maintaining balance, etc.[myvmc.com]
  • Related Cases Disease Idiopathic Intracranial Hypertension Pseudotumor cerebri Benign Tumors Blood and Blood Disorders Eye Diseases High Blood Pressure Venous Sinus Thrombosis Symptom/Presentation Bleeding and Bruising Blurred Vision Hypertension Neck[pediatriceducation.org]
  • She denied photophobia, phonophobia, neck stiffness, fever, vomiting, cough, numbness or weakness in her extremities, or pain anywhere else in her body. Over the past 5 days, the patient noticed her vision had become increasingly blurry.[mdedge.com]
  • At admission, the results of neurologic examinations were normal and neck stiffness was not observed. Blood pressure was normal (88/52 mm Hg). An ophthalmologic test revealed normal visual acuity and a normal visual field.[ajnr.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.[adc.bmj.com]

Treatment

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.

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:

  • 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].

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.

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Last updated: 2018-06-21 17:29