-
Cephalalgia : An International Journal... Apr 2021To review and discuss the clinical presentation and treatment of idiopathic intracranial hypertension. (Review)
Review
OBJECTIVE
To review and discuss the clinical presentation and treatment of idiopathic intracranial hypertension.
DISCUSSION
Visual alterations and headache are the two main symptoms of idiopathic intracranial hypertension, although additional features including cranial nerve palsies, cognitive deficits, olfactory deficits and tinnitus are not uncommon. The headache associated with idiopathic intracranial hypertension frequently has a migrainous phenotype. The underlying cause of the disorder has not yet been elucidated. Several hypotheses have been postulated but none of them can explain the full clinical picture. Therapeutic options remain limited, focusing mainly on reduction in body weight and the reduction of CSF production with carbonic anhydrase inhibitors.
CONCLUSION
The accurate diagnosis of idiopathic intracranial hypertension is essential as visual deterioration due to papilledema may be irreversible. Given its phenotypic similarity and frequent overlap with chronic migraine it is essential to consider idiopathic intracranial hypertension in the diagnostic workup of chronic headache; in particular, when considering its increasing prevalence. Understanding in detail the pathophysiological mechanisms behind the associated headache would also allow study of current and future therapeutic options in a structured way.
Topics: Acetazolamide; Analgesics; Headache; Headache Disorders; Humans; Intracranial Hypertension; Migraine Disorders; Papilledema; Pseudotumor Cerebri; Vision Disorders; Weight Reduction Programs
PubMed: 33631966
DOI: 10.1177/0333102421997093 -
Journal of Clinical Neuroscience :... Jan 2022Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure, manifested by papilledema and radiological findings, in the absence of an... (Review)
Review
Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure, manifested by papilledema and radiological findings, in the absence of an identifiable casual factor. The primary symptoms include headache, vision loss, and pulsatile tinnitus, and are recognized to have profound impacts on quality of life and visual function. IIH demonstrates a strong predilection towards obese women of reproductive age, and the population incidence is rising with the growing prevalence of obesity worldwide. The pathophysiology involves dysregulation of cerebrospinal fluid (CSF) dynamics and venous sinus pressure, and recent studies highlighting the pathogenic role of metabolic and hormonal factors have led to the identification of several pharmacological targets and development of novel therapeutic agents. The overarching treatment goals include symptomatic alleviation and prevention of permanent vision loss. The Idiopathic Intracranial Hypertension Treatment Trial, the first of its kind randomized controlled trial on IIH, provides class I evidence for treatment with weight loss and acetazolamide. In medically refractive or fulminant cases, optic nerve sheath fenestration, CSF diversion, and venous sinus stenting, have been successfully implemented. However, there are few high-quality prospective studies investigating the treatment and natural history of IIH, highlighting the compelling need for further research to determine the optimal treatment regimen.
Topics: Female; Humans; Intracranial Hypertension; Prospective Studies; Pseudotumor Cerebri; Quality of Life; Stents
PubMed: 34929642
DOI: 10.1016/j.jocn.2021.11.029 -
Neurologic Clinics Feb 2017Idiopathic intracranial hypertension (IIH) is a disorder of elevated intracranial pressure of unknown cause occurring predominantly in young women of childbearing age.... (Review)
Review
Idiopathic intracranial hypertension (IIH) is a disorder of elevated intracranial pressure of unknown cause occurring predominantly in young women of childbearing age. The typical patient symptom profile is the presence of daily headache, pulse synchronous tinnitus, transient visual obscurations, and papilledema with its associated visual loss. Although surgical procedures are performed for those who fail medical therapy, their relative efficacy remains unclear. The main morbidity of IIH is from visual loss. This visual loss is present in most patients and can usually be reversed if recognized early in the patients' course and treated.
Topics: Acetazolamide; Diuretics; Humans; Papilledema; Pseudotumor Cerebri; Randomized Controlled Trials as Topic; Vision Disorders
PubMed: 27886895
DOI: 10.1016/j.ncl.2016.08.004 -
Journal of Neurology, Neurosurgery, and... Oct 2018The aim was to capture interdisciplinary expertise from a large group of clinicians, reflecting practice from across the UK and further, to inform subsequent development... (Review)
Review
UNLABELLED
The aim was to capture interdisciplinary expertise from a large group of clinicians, reflecting practice from across the UK and further, to inform subsequent development of a national consensus guidance for optimal management of idiopathic intracranial hypertension (IIH).
METHODS
Between September 2015 and October 2017, a specialist interest group including neurology, neurosurgery, neuroradiology, ophthalmology, nursing, primary care doctors and patient representatives met. An initial UK survey of attitudes and practice in IIH was sent to a wide group of physicians and surgeons who investigate and manage IIH regularly. A comprehensive systematic literature review was performed to assemble the foundations of the statements. An international panel along with four national professional bodies, namely the Association of British Neurologists, British Association for the Study of Headache, the Society of British Neurological Surgeons and the Royal College of Ophthalmologists critically reviewed the statements.
RESULTS
Over 20 questions were constructed: one based on the diagnostic principles for optimal investigation of papilloedema and 21 for the management of IIH. Three main principles were identified: (1) to treat the underlying disease; (2) to protect the vision; and (3) to minimise the headache morbidity. Statements presented provide insight to uncertainties in IIH where research opportunities exist.
CONCLUSIONS
In collaboration with many different specialists, professions and patient representatives, we have developed guidance statements for the investigation and management of adult IIH.
Topics: Consensus; Headache; Humans; Pseudotumor Cerebri
PubMed: 29903905
DOI: 10.1136/jnnp-2017-317440 -
Neurology India 2021Idiopathic intracranial hypertension (IIH) is defined as a syndrome of raised intracranial pressure with normal imaging of the brain and cerebrospinal fluid (CSF)... (Review)
Review
Idiopathic intracranial hypertension (IIH) is defined as a syndrome of raised intracranial pressure with normal imaging of the brain and cerebrospinal fluid (CSF) composition. There is a rising incidence and prevalence of this disease related to the increased prevalence of obesity. It typically affects women of working age, and headache is the predominant morbidity in over 90%. The disease is also more prevalent in young males. There are many controversies and myths that surround IIH. There are currently few treatment options for IIH, management is typically medical with those experiencing progressive visual loss undergoing surgical procedures. Weight loss and venous sinus stenting are a few therapies directed at the etiology.
Topics: Female; Headache; Humans; Intracranial Hypertension; Male; Pseudotumor Cerebri; Stents; Vision Disorders
PubMed: 35103000
DOI: 10.4103/0028-3886.332276 -
Survey of Ophthalmology 2022Papilledema is optic nerve head edema secondary to raised intracranial pressure (ICP). It is distinct from other causes of optic disk edema in that visual function is... (Review)
Review
Papilledema is optic nerve head edema secondary to raised intracranial pressure (ICP). It is distinct from other causes of optic disk edema in that visual function is usually normal in the acute phase. Papilledema is caused by transmission of elevated ICP to the subarachnoid space surrounding the optic nerve that hinders axoplasmic transport within ganglion cell axons. There is ongoing controversy as to whether axoplasmic flow stasis is produced by physical compression of axons or microvascular ischemia. The most common cause of papilledema, especially in patients under the age of 50, is idiopathic intracranial hypertension (IIH); however, conditions that decrease cerebrospinal fluid (CSF) outflow by either causing CSF derangements or mechanically blocking CSF outflow channels, and rarely conditions that increase CSF production, can be the culprit. When papilledema is suspected clinically, blood pressure should be measured, and pseudopapilledema should be ruled out. Magnetic resonance imaging of the brain and orbits with venography sequences is the preferred neuroimaging modality that should be performed next to look for indirect imaging signs of increased ICP and to rule out nonidiopathic causes. Lumbar puncture with measurement of opening pressure and evaluation of CSF composition should then be performed. In patients not in a typical demographic group for IIH, further investigations should be conducted to assess for underlying causes of increased ICP. Magnetic resonance imaging of the neck and spine, magnetic resonance angiography of the brain, computed tomography of the chest, complete blood count, and creatinine testing should be able to identify most secondary causes of intracranial hypertension. Treatment for patients with papilledema should be targeted toward the underlying etiology. Most patients with IIH respond to weight loss and oral acetazolamide. For patients with decreased central acuity and constricted visual fields at presentation, as well as patients who do not respond to treatment with acetazolamide, surgical treatments should be considered, with ventriculoperitoneal shunting being the typical procedure of choice.
Topics: Acetazolamide; Humans; Intracranial Hypertension; Optic Nerve Diseases; Papilledema; Pseudotumor Cerebri
PubMed: 34813854
DOI: 10.1016/j.survophthal.2021.11.007 -
Seminars in Neurology Dec 2019The presentation of idiopathic intracranial hypertension (IIH) in pediatric populations has several important distinctions from that in adults, especially among... (Review)
Review
The presentation of idiopathic intracranial hypertension (IIH) in pediatric populations has several important distinctions from that in adults, especially among prepubertal patients, in which there is no apparent association with gender or obesity. Pediatric patients are more likely to be asymptomatic or present with atypical symptoms than their adult counterparts, posing a diagnostic challenge in some cases. It is important to be aware of the ways in which diagnostic criteria for IIH are modified from that of adults. Ideal treatment practices and the natural history of pediatric IIH remain unclear. Acetazolamide is the mainstay of medical treatment, but some patients with significant visual loss may require surgical intervention. Multicenter studies to accrue a large number of cases and future prospective studies will help to better define pediatric IIH and to formulate consensus guidelines for treatment and management of these patients.
Topics: Child; Humans; Papilledema; Pseudotumor Cerebri; Vision Disorders
PubMed: 31847041
DOI: 10.1055/s-0039-1698743 -
The Lancet. Neurology Jan 2016Idiopathic intracranial hypertension is a disorder characterised by raised intracranial pressure that predominantly affects young, obese women. Pathogenesis has not been... (Review)
Review
Idiopathic intracranial hypertension is a disorder characterised by raised intracranial pressure that predominantly affects young, obese women. Pathogenesis has not been fully elucidated, but several causal factors have been proposed. Symptoms can include headaches, visual loss, pulsatile tinnitus, and back and neck pain, but the clinical presentation is highly variable. Although few studies have been done to support evidence-based management, several recent advances have the potential to enhance understanding of the causes of the disease and to guide treatment decisions. Investigators of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) reported beneficial effects of acetazolamide in patients with mild visual loss. Studies have also established weight loss as an effective disease-modifying treatment, and further clinical trials to investigate new treatments are underway. The incidence of idiopathic intracranial hypertension is expected to increase as rates of obesity increase; efforts to reduce diagnostic delays and identify new, effective approaches to treatment will be key to meeting the needs of a growing number of patients.
Topics: Animals; Comprehension; Disease Management; Forecasting; Humans; Intracranial Hypertension; Obesity; Pseudotumor Cerebri
PubMed: 26700907
DOI: 10.1016/S1474-4422(15)00298-7 -
Neurologic Clinics May 2024Pseudotumor cerebri syndrome is a syndrome of increased cerebrospinal fluid pressure without ventriculomegaly, mass lesion, or meningeal abnormality. It is either... (Review)
Review
Pseudotumor cerebri syndrome is a syndrome of increased cerebrospinal fluid pressure without ventriculomegaly, mass lesion, or meningeal abnormality. It is either primary (idiopathic intracranial hypertension, IIH) or secondary. A secondary cause is unlikely when adhering to the diagnostic criteria. Permanent visual loss occurs if undetected or untreated, and the associated headaches may be debilitating. Fulminant disease may result in blindness despite aggressive treatment. This study addresses the diagnosis and management of IIH including new insights into the pathobiology of IIH, updates in therapeutics and causes of overdiagnosis.
Topics: Humans; Pseudotumor Cerebri; Intracranial Hypertension; Headache; Vision Disorders; Syndrome; Papilledema
PubMed: 38575259
DOI: 10.1016/j.ncl.2024.02.001 -
Current Opinion in Ophthalmology Sep 2016Recent findings in the literature regarding the epidemiology, diagnosis, and treatment of pediatric pseudotumor cerebri (PTC) are described. In the past, children with... (Review)
Review
PURPOSE OF REVIEW
Recent findings in the literature regarding the epidemiology, diagnosis, and treatment of pediatric pseudotumor cerebri (PTC) are described. In the past, children with PTC have been treated like adult cases; however, the literature highlights important differences between children and adults with PTC.
RECENT FINDINGS
Revised diagnostic criteria define an opening cerebrospinal fluid pressure greater than 28 cm water as elevated in the pediatric population. Obesity is an important risk factor for primary and secondary PTC in post-pubertal children. Magnetic resonance imaging shows findings suggestive of elevated intracranial pressure in children with PTC, similar to those of adults with PTC. Diamox and weight loss are effective treatments for PTC patients with mild visual field loss. Severe papilledema, decreased vision, and optical coherence tomography measures at presentation identify patients at increased risk for subsequent visual loss.
SUMMARY
Findings summarized in the recent literature may change practice patterns in the diagnosis and treatment of pediatric PTC. Although there are many similarities between the adult and pediatric populations, the differences should be considered when managing children with PTC.
Topics: Acetazolamide; Carbonic Anhydrase Inhibitors; Cerebrospinal Fluid Pressure; Child; Humans; Magnetic Resonance Imaging; Obesity; Papilledema; Pseudotumor Cerebri; Risk Factors
PubMed: 27491010
DOI: 10.1097/ICU.0000000000000300