Idiopathic Intracranial Hypertension

2019 ◽  
pp. 47-54
Author(s):  
Matthew J. Thurtell ◽  
Robert L. Tomsak

Idiopathic intracranial hypertension is a syndrome of increased intracranial pressure of unknown cause that most often occurs in obese women of childbearing age, although it can also occur in children, men, and older adults. Papilledema is usually present and can cause irreversible vision loss if left untreated. In this chapter, we begin by reviewing the symptoms of this condition, which include headache, transient visual obscurations, and pulse-synchronous tinnitus. We next review the clinical signs and common findings on formal visual field testing. We then discuss the diagnostic evaluation and review the diagnostic criteria for this condition. Lastly, we review the goals and management of this condition, which must be tailored depending on the severity of symptoms, papilledema, and vision loss.


2021 ◽  
pp. 112067212110057
Author(s):  
Chandler Mitchell ◽  
Sunu Mathew ◽  
Alon Harris ◽  
Matthew Lang ◽  
Devin Mackay ◽  
...  

Idiopathic intracranial hypertension (IIH) is characterized by raised intracranial pressure of unknown origin that primarily afflicts obese women of childbearing age. There are several treatment options, but currently there are none that are effective for the entire affected population. The lack of a universally effective treatment is related to an incomplete understanding of the etiology of the condition and the lack of a well-defined pathophysiological mechanism for the disease process. Classically, IIH has been thought of as a diagnosis of exclusion once radiographical imaging has ruled out all other causes of elevated intracranial pressure. Today, we know that imaging does capture subtle changes, and might provide keys to finally understand the pathogenesis of IIH so that a definitive treatment can be discovered or developed. Recently, advancements in radiography, optical coherence tomography, and electroretinography have shown promise for the future of IIH evaluation. A topic within IIH imaging that has recently sparked interest is the possibility that the severity of papilledema may have an association with the size of the optic canal. In this article, we also discuss the recent studies on the relationship between asymmetric papilledema and optic canal size.



Author(s):  
Jong-Won Bae ◽  
Kyu-Yup Lee ◽  
Myung Hoon Yoo ◽  
Da Jung Jung

Idiopathic intracranial hypertension (IIH) is a disorder that commonly occurs in obese young women of childbearing age and is characterized by symptoms such as pulsatile tinnitus, dizziness, headache, nausea, vomiting, and visual loss without any structural or vascular abnormalities in the intracranial cavity. We recently experienced a case of a 33-year-old obese woman who presented with right-sided pulsatile tinnitus, which is an early symptom for IIH. The patient was successfully treated with weight reduction and carbonic anhydrase inhibitor (acetazolamide). Pulsatile tinnitus requires thorough diagnosis and examination because it can be cured if the anatomical or functional cause is identified and treated. When obese women of childbearing age present with pulsatile tinnitus at the otorhinolaryngology outpatient department, treatment for IIH should be initiated after appropriate examination and diagnosis as pulsatile tinnitus may be the only symptom for IIH.



2020 ◽  
pp. 6054-6059
Author(s):  
Alexandra Sinclair

Idiopathic intracranial hypertension (IIH) (also called pseudotumour cerebri and, previously, benign intracranial hypertension) is a syndrome of raised intracranial pressure in the absence of an intracranial mass lesion, enlargement of the cerebral ventricles, or venous sinus thrombosis. IIH affects predominantly obese women of childbearing age (>90%). The condition has considerable morbidity from permanent visual loss (up to 25% of cases) and chronic disabling headaches, which result in poor quality of life. Patients presenting acutely with papilloedema must be evaluated urgently for secondary causes of raised intracranial pressure (e.g. space occupying lesion and venous thrombosis). After this, the priority is to assess accurately the threat to vision. In most patients, the condition becomes chronic and the disease burden is mostly from chronic headaches, which need active management, alongside visual monitoring. This chapter does not cover paediatric IIH.



2020 ◽  
pp. 112067212096904
Author(s):  
Mousumi Banerjee ◽  
Swati Phuljhele Aalok ◽  
Deepti Vibha

Idiopathic Intracranial Hypertension (IIH), also known as pseudotumor cerebri is a clinical syndrome of unknown etiology, which typically affects young, obese women of childbearing age and is characterized by increased intracranial pressure and bilateral papilledema. Unilateral and highly asymmetrical papilledema is a rare presentation in IIH occurring in less than 4% of patients with definite IIH and can poses a diagnostic challenge for the treating physician as it usually raises the suspicion of localized ocular pathology. As per current consensus, papilledema results in stasis of axoplasmic transport due to mechanical compression, leading to secondary vascular changes of venous dilation and hemorrhage. Given this mechanism, the underlying reasons for unilateral and asymmetrical papilledema remain unclear.



2011 ◽  
pp. 29-35
Author(s):  
Matthew J. Thurtell ◽  
Robert L. Tomsak ◽  
Robert B. Daroff

Idiopathic intracranial hypertension is a syndrome of raised intracranial pressure of unknown cause that most often occurs in obese young women. Bilateral papilledema is usually present and can cause severe, irreversible vision loss if left untreated. In this chapter, we review the symptoms, signs, evaluation, and management of idiopathic intracranial hypertension.



2021 ◽  
Vol 6 (2) ◽  
Author(s):  
Gao C ◽  
Zhang H ◽  
Fu J

Idiopathic Intracranial Hypertension (IIH) or Pseudotumor Cerebri (PTC) is a disorder of young obese females and characterized by headache, papilledema and raised intracranial pressure. However, it is in the absence of known pathological cause. Due to the uncertainty of etiology, it may lead to misdiagnosis and grave clinical prognosis. IIH is typically treated with Lumboperitoneal Shunting (LPS) and Ventriculoperitoneal Shunting (VPS), but shunts are prone to dysfunctions and infection, resulting in recurrent headaches in many patients after this treatment. We report a case of 41-year-old obese female (BMI: 30.9) with IIH, who has a history of hypertension (Blood pressure: 150/100 mmHg) and elevated intracranial pressure (Open pressure: 450 mmH2 O). After the failure of several medical treatments, the patient was offered LPS because of vision loss and headache, but the postoperative symptoms (intermittent headache, mainly total craniocerebral prickling pain with neck and shoulder pain) were not significantly relieved for 11 years. Therefore, considering the blockage of the primary shunt tube and the small ventricle in the patient, it has difficulty in puncture ventricle puncture. We have to treat with the stereotactic VPS (SVPS) for her exacerbation symptoms. More surprisingly, the hypertension was in the normal range (<115/80 mmHg) after the surgery (without taking antihypertensive medication). To compare the surgical therapeutic effects and complications of LPS and the SVPS in the treatment of idiopathic intracranial hypertension. Cerebrospinal Fluid (CSF) diversion with VPS or LPS is usually performed when the main symptom is vision loss; it also stabilizes headache and papilledema. LPS significantly alleviates symptoms in the short term, but due to excessive shunt of LPS for a long time, it is easy to be complicated with Chiari malformation and slit ventricle syndrome. Therefore, we encourage operating the SVPS on our patients for the favorable long-term outcome.



Author(s):  
Ore-ofe O. Adesina ◽  
Sudama Reddi ◽  
Deborah I. Friedman ◽  
Kathleen Digre

Idiopathic intracranial hypertension (IIH) is a disorder of raised intracranial pressure, almost always associated with papilloedema, in the absence of underlying central nervous system pathology. It is a rare disease, with an annual incidence of around 1/100,000 persons with an onset between 11 and 58 years. It is predominantly seen in obese women of childbearing age (incidence 10–20/100,000), but can affect any age, race, or sex. The two morbidities associated with IIH are vision loss and headache, with headache ultimately affecting over 90% of patients. The exact mechanisms underlying IIH related headache pain are still unknown, but it is often debilitating and significantly impacts quality of life. The goal of treatment is to reduce intracranial pressure to minimize vision loss and headaches. Effective medical and surgical interventions are available for treatment of headache in IIH and are tailored to each individual patient. Overall, the prognosis for treatment of headache in IIH is good.



Author(s):  
Bernhard Schmidt ◽  
Marek Czosnyka ◽  
Danilo Cardim ◽  
Zofia Czosnyka ◽  
Bernhard Rosengarten

Abstract Purpose Idiopathic intracranial hypertension (IIH) usually occurs in obese women of childbearing age. Typical symptoms are headache and sight impairment. Lumbar puncture (LP) is routinely used for both diagnosis and therapy (via cerebrospinal fluid drainage) of IIH. In this study, noninvasively assessed intracranial pressure (nICP) was compared to LP pressure (LPP) in order to clarify its feasibility for the diagnosis of IIH. Materials and Methods nICP was calculated using continuous signals of arterial blood pressure and cerebral blood flow velocity in the middle cerebral artery, a method which has been introduced recently. In 26 patients (f = 24, m = 2; age: 33 ± 11 years), nICP was assessed one hour prior to LPP. If LPP was > 20 cmH2O, lumbar drainage was performed, LPP was measured again, and also nICP was reassessed. Results In total, LPP and nICP correlated with R = 0.85 (p < 0.001; N = 38). The mean difference of nICP-LPP was 0.45 ± 4.93 cmH2O. The capability of nICP to diagnose increased LPP (LPP > 20 cmH2O) was assessed by ROC analysis. The optimal cutoff for nICP was close to 20 cmH2O with both a sensitivity and specificity of 0.92. Presuming 20 cmH2O as a critical threshold for the indication of lumbar drainage, the clinical implications would coincide in both methods in 35 of 38 cases. Conclusion The TCD-based nICP assessment seems to be suitable for a pre-diagnosis of increased LPP and might eliminated the need for painful lumbar puncture if low nICP is detected.



2018 ◽  
Vol 5 (2) ◽  
pp. 51-54
Author(s):  
Sepideh Paybast ◽  
Farhad Assarzadegan ◽  
Behnam Safarpoor Lima ◽  
Mohsen Koosha

Background: Idiopathic intracranial hypertension (IIH) is an increased intracranial pressure with normal cerebrospinal fluid (CSF) characteristic in the absence of identifiable causes. The most important complication of this disorder is visual impairment. So far, no comprehensive study has been done on the relationship between the opening CSF pressure and visual field defect in IIH. Methods: In this study, 35 patients with increased intracranial pressure who fulfilled modified Dandy’s criteria underwent ophthalmologic examination and lumbar puncture. The opening CSF pressure was categorized into mild (25-30), moderate (30-40) and severe (>40). The degree of visual field defect was reported both quantitatively and qualitatively. Eventually, the statistical relationship was established among these variables. Results: The mean opening CSF pressure was 33.71 CmH2o. Twelve patients had minor CSF pressure, whereas in 14 and 9 patients the CSF pressure was respectively moderate and severe. There was not statistically significant relationship between the visual field defect and CSF pressure. The most common patterns of visual field involvement were enlarged blind spot and peripheral restriction. Conclusion: The most important morbidity in IIH is visual impairment. According to the findings, the visual field impairment is not pertinent to CSF pressure. In other words, neither high CSF pressure predicts intense visual defect, nor low CSF pressure indicates minimal visual impairment.



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