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American Journal of Therapeutics 2020
Topics: Adolescent; Aripiprazole; Bipolar Disorder; Drug Substitution; Female; Humans; Pseudotumor Cerebri; Quetiapine Fumarate
PubMed: 31356344
DOI: 10.1097/MJT.0000000000001006 -
Seminars in Neurology Jun 2020Pseudotumor cerebri syndrome (PTCS) is a rare condition in children presenting with headache and papilledema from increased intracranial pressure that can cause... (Review)
Review
Pseudotumor cerebri syndrome (PTCS) is a rare condition in children presenting with headache and papilledema from increased intracranial pressure that can cause significant morbidity. This can be idiopathic, also known as idiopathic intracranial hypertension or primary intracranial hypertension, or can be secondary to medications and associated medical conditions. Given the threat to vision, early detection and treatment is needed in all age groups. However, identifying papilledema or pseudopapilledema in children presents unique challenges sometimes as a result of differences between prepubertal and postpubertal children, further elucidating the complex pathophysiology. Management requires brain imaging, lumbar puncture, and frequent eye exams with medical and rarely surgical treatment. Visual outcomes in children are favorable if caught early and management can be prolonged over years. Pediatric PTCS is different from adult PTCS in many ways, and this review will focus on the most updated definitions of the disease, theories of pathophysiology, management, and treatment in the pediatric population.
Topics: Adolescent; Child; Eye Diseases, Hereditary; Humans; Optic Nerve Diseases; Papilledema; Pseudotumor Cerebri
PubMed: 32422670
DOI: 10.1055/s-0040-1708847 -
Clinical & Experimental Ophthalmology Aug 2023Little is known about the presentation and prognosis of asymptomatic idiopathic intracranial hypertension (IIH). Papilloedema can be found incidentally on routine fundus... (Observational Study)
Observational Study
BACKGROUND
Little is known about the presentation and prognosis of asymptomatic idiopathic intracranial hypertension (IIH). Papilloedema can be found incidentally on routine fundus examination, with many of these patients actually having symptoms on direct questioning. The aim was to evaluate visual and headache outcomes in people with IIH who present with or without symptoms.
METHODS
Prospective observational cohort study, between 2012 and 2021, 343 people with confirmed IIH diagnosis were enrolled in the IIH:Life database. Outcomes such as vision (LogMAR); Humphrey visual field perimetric mean deviation (PMD) and optical coherence tomography (OCT) and headache were evaluated using LOESS (locally weighted scatterplot smoothing) graphs and regression analysis.
RESULTS
One hundred and twenty-one people had incidentally found papilloedema, with 36 people with completely asymptomatic presentations. Those with asymptomatic IIH at diagnosis had similar visual prognosis compared to those with symptomatic disease. Sixty-six percent of the asymptomatic cohort became symptomatic during follow-up, and of these the predominant symptom was headache (96%). Headache frequency during follow-up was lower in the asymptomatic cohort.
CONCLUSIONS
The prognosis of those with IIH who present with or without symptoms is similar.
Topics: Humans; Pseudotumor Cerebri; Papilledema; Prevalence; Prospective Studies; Prognosis; Headache; Intracranial Hypertension
PubMed: 37243483
DOI: 10.1111/ceo.14256 -
Expert Review of Neurotherapeutics Jun 2021: Idiopathic intracranial hypertension is a condition characterized by raised intracranial pressure, papilledema, and normal neuroimaging (aside from radiological signs... (Review)
Review
: Idiopathic intracranial hypertension is a condition characterized by raised intracranial pressure, papilledema, and normal neuroimaging (aside from radiological signs of raised intracranial pressure). Symptoms of idiopathic intracranial hypertension include chronic headaches and for some, visual loss. New treatments are unmet clinical needs.: The aim of this review is to present the evidence base and considered opinion on outcome measures to determine successful management of idiopathic intracranial hypertension.: Less invasive measures of disease activity such as optical coherence tomography will continue to grow in this field, both as a measure of papilledema, and potentially as a surrogate for intracranial pressure and visual function. As a highly disabling aspect of the disease is headache, treatment outcomes for headache morbidity need to be appropriately chosen and standardized to allow comparison between trials.
Topics: Headache; Humans; Intracranial Hypertension; Papilledema; Pseudotumor Cerebri; Tomography, Optical Coherence
PubMed: 34047224
DOI: 10.1080/14737175.2021.1931127 -
Cephalalgia : An International Journal... Mar 2015Idiopathic intracranial hypertension or pseudotumour cerebri is primarily a disorder of young obese women characterised by symptoms and signs associated with raised... (Review)
Review
BACKGROUND
Idiopathic intracranial hypertension or pseudotumour cerebri is primarily a disorder of young obese women characterised by symptoms and signs associated with raised intracranial pressure in the absence of a space-occupying lesion or other identifiable cause.
SUMMARY
The overall incidence of idiopathic intracranial hypertension is approximately two per 100,000, but is considerably higher among obese individuals and, given the global obesity epidemic, is likely to rise further. The pathophysiology of this condition is poorly understood, but most theories focus on the presence of intracranial venous hypertension and/or increased cerebrospinal fluid outflow resistance and how this relates to obesity. A lack of randomised clinical trials has resulted in unsatisfactory treatment guidelines and although weight loss is important, especially when used in conjunction with drugs that reduce cerebrospinal fluid production, resistant cases remain difficult to manage and patients invariably undergo neurosurgical shunting procedures. The use of transverse cerebral sinus stenting remains contentious and long-term benefits are yet to be determined.
CONCLUSION
An understanding of the clinical features, diagnostic work-up and therapeutic options available for patients with idiopathic intracranial hypertension is important both for neurologists and ophthalmologists as visual loss maybe permanent if untreated.
Topics: Female; Humans; Obesity; Optic Nerve Diseases; Pseudotumor Cerebri; Risk Factors; Sex Factors
PubMed: 24847166
DOI: 10.1177/0333102414534329 -
Neurosurgery Mar 2017Intracranial pressure (ICP) is the pressure inside the bony calvarium and can be affected by a variety of processes, such as intracranial masses and edema, obstruction... (Review)
Review
Intracranial pressure (ICP) is the pressure inside the bony calvarium and can be affected by a variety of processes, such as intracranial masses and edema, obstruction or leakage of cerebrospinal fluid, and obstruction of venous outflow. This review focuses on the imaging of 2 important but less well understood ICP disorders: idiopathic intracranial hypertension and spontaneous intracranial hypotension. Both of these ICP disorders have salient imaging findings that are important to recognize to help prevent their misdiagnosis from other common neurological disorders.
Topics: Humans; Intracranial Hypotension; Intracranial Pressure; Magnetic Resonance Imaging; Pseudotumor Cerebri
PubMed: 27471977
DOI: 10.1227/NEU.0000000000001362 -
Expert Review of Neurotherapeutics Sep 2019: Diagnosis and appropriate management of patients with the pseudotumor cerebri syndrome are imperative to prevent or minimize permanent visual loss and headache-related... (Review)
Review
: Diagnosis and appropriate management of patients with the pseudotumor cerebri syndrome are imperative to prevent or minimize permanent visual loss and headache-related disability. : Steps in management, including making the correct diagnosis, techniques to assess the patient's visual status, medical treatment of intracranial hypertension and the associated headaches, weight management strategies, surgical treatments and stenting are reviewed incorporating the most recent medical evidence. : As the pathogenesis of the pseudotumor cerebri syndrome is still unknown, many of the currently employed management strategies incorporate a 'plumbing approach' to decrease cerebrospinal fluid (CSF) pressure. The Idiopathic Intracranial Hypertension Trial (IIHTT) taught us that the disorder markedly affects visual and overall quality of life, and that reducing pressure alone is not enough to make patients well, even those considered to have 'mild' vision loss. Other than the IIHTT, the evidence supporting the use of various treatments is meager. The course of the disorder can be unpredictable, and the clinician(s) managing these patients are often uncertain about which treatments to employ. Moreover, the desired modalities and specialists are not universally available in all locations. An individualized and detailed approach to the various manifestations and nuances of the disorder is essential.
Topics: Humans; Pseudotumor Cerebri
PubMed: 31478394
DOI: 10.1080/14737175.2019.1660163 -
Continuum (Minneapolis, Minn.) Oct 2019Idiopathic intracranial hypertension is a syndrome of increased intracranial pressure of unclear etiology that most often occurs in obese women of childbearing age but... (Review)
Review
PURPOSE OF REVIEW
Idiopathic intracranial hypertension is a syndrome of increased intracranial pressure of unclear etiology that most often occurs in obese women of childbearing age but can also occur in men, children, and older adults. This article reviews the diagnostic criteria, clinical features, neuroimaging findings, differential diagnosis, and management options for this condition.
RECENT FINDINGS
Recent population studies have found that the annual incidence of idiopathic intracranial hypertension is increasing in association with obesity rates, whereas recent scientific studies indicate a possible role for androgen sex hormones and adipose tissue in the pathogenesis of the disease. Prospective clinical trials have demonstrated a role for weight loss, acetazolamide, and topiramate in the management of mild disease. A recently begun randomized multicenter trial of surgical interventions will provide insight into the indications for surgical intervention, optimal timing and choice of intervention, and long-term outcomes.
SUMMARY
Idiopathic intracranial hypertension is a disorder producing symptoms and signs of increased intracranial pressure in the absence of an alternative cause. The main goals of treatment are to preserve visual function and alleviate symptoms, which can usually be achieved with a combination of weight loss, medical therapies, and surgical interventions depending on the severity of symptoms and vision loss, response to treatment, and subsequent clinical course.
Topics: Adolescent; Adult; Female; Humans; Pseudotumor Cerebri; Young Adult
PubMed: 31584538
DOI: 10.1212/CON.0000000000000770 -
Clinical Rheumatology Apr 2021
Topics: Humans; Pseudotumor Cerebri
PubMed: 33174108
DOI: 10.1007/s10067-020-05503-8 -
Endocrine Practice : Official Journal... Nov 2019
Topics: Adult; Female; Humans; Pseudotumor Cerebri
PubMed: 30865524
DOI: 10.4158/EP-2019-0096