What is New About Idiopathic Intracranial Hypertension? An Updated Review of Mechanism and Treatment

Cephalalgia ◽  
2006 ◽  
Vol 26 (4) ◽  
pp. 384-399 ◽  
Author(s):  
M Skau ◽  
J Brennum ◽  
F Gjerris ◽  
R Jensen
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Clinical Laboratory ◽  
Treatment Strategies ◽  
Disease Course ◽  
Causal Treatment ◽  
Associated Conditions ◽  
Patients Experience ◽  
Visual Disturbances ◽  
Neuroimaging Techniques

Idiopathic intracranial hypertension (IIH) is the syndrome of raised intracranial pressure without clinical, laboratory or radiological evidence of intracranial pathology. IIH is a relatively rare disease but rapidly increasing incidence is reported due to a global increasing incidence of obesity. Disease course is generally said to be self-limiting within a few months. However, some patients experience a disabling condition of chronic severe headache and visual disturbances for years that limit their capacity to work. Permanent visual defects are serious and not infrequent complications. The pathophysiology of IIH is still not fully understood. Advances in neuroimaging techniques have facilitated the exclusion of associated conditions that may mimic IIH. No causal treatment is yet known for IIH and existing treatment is symptomatic and rarely sufficient. The aim of this review is to provide an updated overview of this potentially disabling disease which may show a future escalating incidence due to obesity. Theories of pathogenesis, diagnostic criteria and treatment strategies are discussed.

Idiopathic intracranial hypertension

Neurology ◽  
2018 ◽  
Vol 91 (11) ◽  
pp. 515-522 ◽  
Author(s):  
Stéphanie Lenck ◽  
Ivan Radovanovic ◽  
Patrick Nicholson ◽  
Mojgan Hodaie ◽  
Timo Krings ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Interstitial Fluid ◽  
Water Exchange ◽  
Venous Blood ◽  
Glymphatic System ◽  
Associated Conditions ◽  
Outflow Pathway ◽  
The Brain

The recent discoveries of the glymphatic and lymphatic systems of the brain have helped advance our understanding of CSF physiology and may allow new insights in the understanding of idiopathic intracranial hypertension (IIH). The clinical and radiologic presentations of IIH appear to be related to congestion of the glymphatic system associated with an overflow of the lymphatic CSF outflow pathway. By revisiting the role of “vascular arachnoid granulations” in the brain, we hypothesize that an initial impairment of the transport of interstitial fluid from the glymphatic system to the venous blood of the dural sinuses may trigger the hydrodynamic cascade of IIH. Furthermore, we speculate that, similar to other water-exchange systems in the brain, a specific subtype of aquaporin is involved in this transport. This theory may eventually help to provide an underlying explanation for IIH and its associated conditions, since in most of them, the expression of several aquaporins is altered.

scholarly journals The patients’ perspective on the burden of idiopathic intracranial hypertension

2021 ◽  
Author(s):  
Max Witry ◽  
Christine Kindler ◽  
Johannes Weller ◽  
Andreas Linder ◽  
Ullrich Wüllner
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Sleep Disturbances ◽  
Online Survey ◽  
Cross Sectional Study ◽  
Increased Intracranial Pressure ◽  
Opening Pressure ◽  
Cross Sectional ◽  
Patients Perspective ◽  
Visual Disturbances

Abstract Background: Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure without evidence of a tumor or any other underlying cause. Headache and visual disturbances are frequent complaints of IIH patients, but little is known about other symptoms. In this study, we evaluated the patients’ perspective on the burden of IIH. Methods: For this cross-sectional study, we developed an online survey for patients with IIH containing standardized evaluations of headache (HIT-6), sleep (PROMIS Sleep Disturbance Scale) and depression (MDI) in relation to BMI, lumbar puncture opening pressure (LP OP) and treatment. Results: Between December 2019 and February 2020, 306 patients completed the survey. 285 (93%) were female, mean age was 36.6 years (± 10.8), mean BMI 34.2 (± 7.3) and mean LP OP at diagnosis was 37.8 cmH2O (± 9.5). 219 (72%) of the participants were obese (BMI ≥ 30); 251 (82%) reported severe impacting headaches, 140 (46%) were suffering from sleep disturbances and 169 (56%) from depression. Higher MDI scores correlated with higher BMI and increased sleep disturbances. Patients with a normalized LP opening pressure reported less headaches, less sleep disturbances and less depression than those with a constantly elevated opening pressure. Conclusion: In addition to headaches and visual disturbances, sleep disturbances and depression are frequent symptoms in IIH and contribute to the patients’ burden. Structured questionnaires can help to identify IIH patients’ needs and can lead to personalized and better treatment.

scholarly journals Neuro-ophthalmological manifestations after intramuscular medroxyprogesterone: a forme fruste of idiopathic intracranial hypertension?

2016 ◽  
Vol 8 (3) ◽  
Author(s):  
Mandreker Bahall ◽  
Antonio Jose Reyes ◽  
Kanterpersad Ramcharan ◽  
Nadeem Hosein ◽  
Karan Seegobin ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Health Care Providers ◽  
Time Use ◽  
Idiopathic Intracranial Hypertension ◽  
Intramuscular Injection ◽  
Clinical Laboratory ◽  
Fluid Pressure ◽  
Care Providers ◽  
First Time

We report a case of a 22-year-old female student nurse who presented to hospital with an acute neuro-ophthalmological syndrome characterized by papilledema, ataxia, ophthalmoplegia and headache after a single first time use of 150 mg medroxyprogesterone intramuscular injection. Clinical, laboratory, radiological and ophthalmological investigations were in keeping with the diagnosis of idiopathic intracranial hypertension but lumbar puncture did not show a raised cerebrospinal fluid pressure suggesting a <em>forme</em> <em>fruste</em> of this entity. Her neuro-ophthalmological clinical features responded well to acetazolamide and diagnostic/ therapeutic lumbar puncture. Full recovery was achieved three months after medroxyprogesterone usage. Health care providers must be aware of this adverse drug reaction.

scholarly journals Update on Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

2017 ◽  
Vol 01 (03) ◽  
pp. E224-E231 ◽  
Author(s):  
Jan Hoffmann ◽  
Arne May
Keyword(s):  
Cerebrospinal Fluid ◽  
Body Weight ◽  
Intracranial Pressure ◽  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Controlled Trials ◽  
Venous Outflow ◽  
Double Blind ◽  
Double Blind Placebo ◽  
Visual Disturbances

AbstractIdiopathic intracranial hypertension is characterized by an increase of intracranial pressure of unknown etiology. The clinical presentation is dominated by progressive visual disturbances, which are commonly the result of a papilledema, headache and cranial nerve palsies. Clinical studies have revealed over the past years that the syndrome may also be associated with olfactory disturbances, cognitive deficits and a pulsatile tinnitus.The underlying pathomechanism is probably based on a disturbance of venous outflow, which causes a reduced absorption of cerebrospinal fluid that results in an increase of cerebrospinal fluid pressure. It remains unclear if the venous outflow disturbance results from the overweight-induced increase of intrathoracic pressure, the presence of sinus vein stenoses or both conditions. Recently hormonal factors have also been discussed, but the mechanisms behind a potential influence on intracranial pressure remain unclear.Treatment of idiopathic intracranial hypertension relies mainly on an effective body weight reduction and medication with carboanhydrase inhibitors, which can reduce the production of cerebrospinal fluid and body weight. Results of the first randomized, placebo-controlled trial as well as a 12-month follow-up show that acetazolamide effectively reduces papilledema, visual disturbances and headache in idiopathic intracranial hypertension. In contrast, the evidence for the carboanhydrase inhibitors topiramate and furosemide still relies on open-label trials, because double-blind, placebo-controlled trials for their efficacy in idiopathic intracranial hypertension do not exist. In addition to the treatment with carboanhydrase inhibitors, increasing evidence suggests that the somatostatin analog octreotide may be effective in the treatment of idiopathic intracranial hypertension, but to date no randomized, double-blind, placebo-controlled trials exist to confirm this observation.

scholarly journals Idiopathic Intracranial Hypertension in a Girl with Juvenile Idiopathic Arthritis-Associated Uveitis: a case report

2019 ◽  
Author(s):  
Asaad Alkoht ◽  
Huda Alhariry ◽  
Ibrahem Hanafi ◽  
Majed Aboud
Keyword(s):  
Risk Factors ◽  
Juvenile Idiopathic Arthritis ◽  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Elevated Pressure ◽  
Optic Disc Edema ◽  
Case Presentation ◽  
Disc Edema ◽  
Asymptomatic Patients ◽  
Associated Conditions

Abstract Background Idiopathic intracranial hypertension (IIH) is a rare disorder of elevated pressure of cerebrospinal fluid (CSF) with no evident cause. It has several associated conditions and medications and it may present asymptomatically. Case presentation We here present the case of a 14-year-old girl with juvenile idiopathic arthritis (JIA)-associated uveitis who also had papilledema. She was diagnosed later with IIH as she developed headaches. To the best of our knowledge, this is the fifth case to have uveitis and IIH among children and the only one with no obvious risk factors for IIH, as all were excluded including corticosteroids, tetracyclines and weight gain Conclusions our case suggests to suspect IIH in children when optic disc edema accompany uveitis even in asymptomatic patients, considering that papilledema doesn't resolve despite uveitis recovery in this case.

scholarly journals Transverse venous stenting for the treatment of idiopathic intracranial hypertension, or pseudotumor cerebri

2018 ◽  
Vol 45 (1) ◽  
pp. E11 ◽  
Author(s):  
Justin M. Cappuzzo ◽  
Ryan M. Hess ◽  
John F. Morrison ◽  
Jason M. Davies ◽  
Kenneth V. Snyder ◽  
...  
Keyword(s):  
Pressure Gradient ◽  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Transverse Sinus ◽  
Venous Sinus ◽  
Venous Stenting ◽  
Shunt Placement ◽  
The Mean ◽  
Visual Disturbances

OBJECTIVEIdiopathic intracranial hypertension (IIH) is a commonly occurring disease, particularly among young women of child-bearing age. The underlying pathophysiology for this disease has remained largely unclear; however, the recent literature suggests that focal outflow obstruction of the transverse sinus may be the cause. The purpose of this study was to report one group’s early experience with transverse venous sinus stenting in the treatment of IIH and assess its effectiveness.METHODSThe authors performed a retrospective chart review to identify patients who had undergone stenting of an outflow-obstructed transverse venous sinus for the treatment of IIH at Gates Vascular Institute between January 2015 and November 2017. Patient demographic data of interest included age, sex, BMI, and history of smoking, hypertension, obstructive sleep apnea, hormonal contraceptive use, and acetazolamide therapy. Each patient’s presenting signs and symptoms and whether those symptoms improved with treatment were reviewed. The average opening lumbar puncture (LP) pressure preprocedure, average pressure gradient across the obstructed segment prior to stenting, treatment failure rate (need for shunt placement), and mean follow-up period were calculated.RESULTSOf the 18 patients who had undergone transverse venous stenting for IIH, 16 (88.9%) were women. The mean age of all the patients was 38.3 years (median 38 years). Mean BMI was 34.2 kg/m2 (median 33.9 kg/m2). Presenting symptoms were headache (16 patients [88.9%]), visual disturbances (13 patients [72.2%]), papilledema (8 patients [44.4%]), tinnitus (3 patients [16.7%]), and auditory bruit (3 patients [16.7%]). The mean opening LP pressure pre-procedure was 35.6 cm H2O (median 32 cm H2O). The mean pressure gradient measured proximally and distally to the area of focal obstruction within the transverse sinus was 16.5 cm H2O (median 15 cm H2O). Postprocedurally, 14 patients (77.8%) continued to have headaches; 6 (33.3%) continued to have visual disturbances. No patients continued to have auditory bruit (0%) or papilledema (0%). One patient (5.6%) had new-onset tinnitus postprocedure. Overall improvement of symptoms was noted in 16 patients (88.9%) postprocedure, with 1 patient (5.6%) requiring shunt placement and 2 other patients (11.1%) requiring postprocedural LP to monitor intracranial pressure to determine candidacy for further surgical interventions to treat residual symptoms. The mean duration of follow-up was 194.2 days.CONCLUSIONSTransverse sinus stenting is a rapidly developing technique that has shown good effectiveness and safety in the literature. Authors of the present study found that stenting a flow-obstructed transverse sinus in patients with IIH was a safe and effective way to treat the condition.

Visual loss after treatment for idiopathic intracranial hypertension with lumboperitoneal shunting

2020 ◽  
pp. 112067212092020
Author(s):  
Angeliki Androu ◽  
Dimitrios Alonistiotis ◽  
Dimitrios Isaakidis ◽  
Konstantinos Ananikas ◽  
Panagiotis Stavrakas
Keyword(s):  
Cerebrospinal Fluid ◽  
Visual Field ◽  
Intracranial Pressure ◽  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Visual Loss ◽  
Pressure Fluctuations ◽  
Visual Field Deficit ◽  
Catheter Malfunction ◽  
Visual Disturbances

Both idiopathic intracranial hypertension and intracranial cerebrospinal fluid hypotension, spontaneous or iatrogenic, display visual disturbances, including visual loss, visual field deficit, transient visual obscurations, and diplopia. We present a case of a long-standing idiopathic intracranial hypertension, primarily manifesting with visual disturbances and documented pre-existing concentric visual field deficit, treated with a lumboperitoneal shunt after conservative treatment failure, leading to an unexpected exacerbation of visual field deficit and acute visual loss, probably due to shunt catheter malfunction and subsequent intracranial hypotension. We provide a step-by-step documentation of cerebrospinal fluid volume and consequent intracranial pressure fluctuations effect on visual field alterations over a significant period of time, attempting an insight on pathogenetic mechanisms implicated in the relationship between intracranial pressure and optic nerve functionality. Interdepartmental collaboration in such cases could ensure prompt diagnosis and treatment, leading to the possibility of either halting the progression or even reversing established visual loss, sparing our patients from a lifetime disability and improving their quality of life.

scholarly journals Bilateral Transverse Sinus Angioplasty for the Treatment of Idiopathic Intracranial Hypertension – Case Report and Literature Review

2019 ◽  
Vol 38 (03) ◽  
pp. 203-209 ◽  
Author(s):  
Luciano B. Manzato ◽  
Paulo Moacir Mesquita Filho ◽  
Leonardo Frighetto ◽  
Nério Dutra Azambuja ◽  
Rafael Cordeiro ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Transverse Sinus ◽  
Venous Sinuses ◽  
Nerve Sheath ◽  
Sinus Stenosis ◽  
The Subject ◽  
Bilateral Stenting ◽  
Visual Disturbances ◽  
Dural Venous Sinuses

AbstractIdiopathic intracranial hypertension (IIH) is a disease characterized by an increase in intracranial pressure, without presence of parenchymal lesions or hydrocephalus that justify it. Over 90% of cases there is association with stenosis of the dural venous sinuses. It is characterized by headache, tinidus, nausea, vomiting and visual disturbances. Initial treatment is clinical and when it fails there is indication of invasive procedures, among them shunts and fenestration of the optic nerve sheath. Angioplasty of dural venous sinuses, when indicated, has shown an alternative with better results and less complications. We report a case of a female patient, with 27 years old, diagnosed with IIH and bilateral transverse sinus stenosis, which was treated by bilateral stenting and total resolution of symptoms. Besides describing the case we review the literature about the subject.

scholarly journals The patients’ perspective on the burden of idiopathic intracranial hypertension

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Max Witry ◽  
Christine Kindler ◽  
Johannes Weller ◽  
Andreas Linder ◽  
Ullrich Wüllner
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Sleep Disturbances ◽  
Online Survey ◽  
Cross Sectional Study ◽  
Increased Intracranial Pressure ◽  
Opening Pressure ◽  
Cross Sectional ◽  
Patients Perspective ◽  
Visual Disturbances

Abstract Background Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure without evidence of a tumor or any other underlying cause. Headache and visual disturbances are frequent complaints of IIH patients, but little is known about other symptoms. In this study, we evaluated the patients’ perspective on the burden of IIH. Methods For this cross-sectional study, we developed an online survey for patients with IIH containing standardized evaluations of headache (HIT-6), sleep (PROMIS Sleep Disturbance Scale) and depression (MDI) in relation to BMI, lumbar puncture opening pressure (LP OP) and treatment. Results Between December 2019 and February 2020, 306 patients completed the survey. 285 (93 %) were female, mean age was 36.6 years (± 10.8), mean BMI 34.2 (± 7.3) and mean LP OP at diagnosis was 37.8 cmH2O (± 9.5). 219 (72 %) of the participants were obese (BMI ≥ 30); 251 (82 %) reported severe impacting headaches, 140 (46 %) were suffering from sleep disturbances and 169 (56 %) from depression. Higher MDI scores correlated with higher BMI and increased sleep disturbances. Patients with a normalized LP opening pressure reported less headaches, less sleep disturbances and less depression than those with a constantly elevated opening pressure. Conclusions In addition to headaches and visual disturbances, sleep disturbances and depression are frequent symptoms in IIH and contribute to the patients’ burden. Structured questionnaires can help to identify IIH patients’ needs and can lead to personalized and better treatment.

scholarly journals The patients’ perspective on the burden of idiopathic intracranial Hypertension

2021 ◽  
Author(s):  
Max Witry ◽  
Christine Kindler ◽  
Johannes Weller ◽  
Andreas Linder ◽  
Ullrich Wüllner
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Sleep Disturbances ◽  
Online Survey ◽  
Cross Sectional Study ◽  
Increased Intracranial Pressure ◽  
Opening Pressure ◽  
Cross Sectional ◽  
Patients Perspective ◽  
Visual Disturbances

Abstract Background: Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure without evidence of a tumor or any other underlying cause. Headache and visual disturbances are frequent complaints of IIH patients, but little is known about other symptoms. In this study, we evaluated the patients’ perspective on the burden of IIH. Methods: For this cross-sectional study, we developed an online survey for patients with IIH containing standardized evaluations of headache (HIT-6), sleep (PROMIS Sleep Disturbance Scale) and depression (MDI) in relation to BMI, lumbar puncture opening pressure (LP OP) and treatment. Results: Between December 2019 and February 2020, 306 patients completed the survey. 285 (93%) were female, mean age was 36.6 years (± 10.8), mean BMI 34.2 (± 7.3) and mean LP OP at diagnosis was 37.8 cmH2O (± 9.5). 219 (72%) of the participants were obese (BMI ≥ 30); 251 (82%) reported severe impacting headaches, 140 (46%) were suffering from sleep disturbances and 169 (56%) from depression. Higher MDI scores correlated with higher BMI and increased sleep disturbances. Patients with a normalized LP opening pressure reported less headaches, less sleep disturbances and less depression than those with a constantly elevated opening pressure. Conclusion: In addition to headaches and visual disturbances, sleep disturbances and depression are frequent symptoms in IIH and contribute to the patients’ burden. Structured questionnaires can help to identify IIH patients’ needs and can lead to personalized and better treatment.

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