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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 -
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 -
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 -
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 -
Current Opinion in Neurology Feb 2021Negative findings on neuroimaging are part of the diagnostic criteria for idiopathic intracranial hypertension (IIH), a syndrome characterized by increased intracranial... (Review)
Review
PURPOSE OF REVIEW
Negative findings on neuroimaging are part of the diagnostic criteria for idiopathic intracranial hypertension (IIH), a syndrome characterized by increased intracranial pressure (ICP). Some positive neuroimaging findings are associated with increased ICP, but their role in diagnosis of IIH has not been established. We provide an overview of these findings and their relevance for diagnosis of raised intracranial pressure.
RECENT FINDINGS
MRI acquisition techniques have significantly improved in the last few decades leading to better characterization of the intracranial changes associated with IIH, including empty sella turcica, optic nerve tortuosity, distension of the optic nerve sheath, posterior globe flattening, slit-like ventricles, and venous sinus stenosis. These may be MRI biomarkers of increased ICP. Prevalence difference between people with and without increased ICP, and reversibility of these MRI findings following treatment of increased ICP inform evaluation of their diagnostic potential.
SUMMARY
MRI and magnetic resonance venography findings are important tools in the diagnosis of IIH. Empty sella turcica, optic nerve protrusion, distension of the optic nerve sheath, optic nerve tortuosity, posterior globe flattening, and transverse sinus stenosis have been found to be the most promising diagnostic markers for IIH, although absence of these findings does not rule out the diagnosis.
Topics: Biomarkers; Constriction, Pathologic; Humans; Intracranial Hypertension; Magnetic Resonance Imaging; Neuroimaging; Optic Nerve; Pseudotumor Cerebri
PubMed: 33230036
DOI: 10.1097/WCO.0000000000000885 -
Current Pain and Headache Reports Jul 2019Pseudotumor cerebri syndrome (PTCS) may affect both children and adults; however, the risk factors and clinical presentation vary greatly between these populations. This... (Review)
Review
PURPOSE OF REVIEW
Pseudotumor cerebri syndrome (PTCS) may affect both children and adults; however, the risk factors and clinical presentation vary greatly between these populations. This review aims to highlight the entity of PTCS in children and the unique considerations in this population; review the epidemiology and demographics; discuss the clinical presentation, revised diagnostic criteria, and approach to evaluation; review management strategies; and discuss the prognosis and long-term outcomes in children with PTCS.
RECENT FINDINGS
Clinical presentation can be variable in children and may be less obvious than in their adult counterparts. Papilledema can also be challenging to diagnose in this population. The upper limits for opening pressure on lumbar puncture differ in children, with a cut-off of 25 cm H20 (or 28 cm H2O in a sedated or obese child). Morbidity related to visual loss, pain and reduced quality of life lends urgency towards accurately identifying, evaluating and managing children with PTCS. There are no randomised controlled studies to allow for evidence-based recommendations for the management of PTCS in children. Further studies are needed to clarify and consolidate management approaches in this population.
Topics: Adolescent; Child; Female; Humans; Male; Pseudotumor Cerebri; Syndrome
PubMed: 31292773
DOI: 10.1007/s11916-019-0795-8 -
Neurology India 2021Rise in intracranial tension (ICT) has varied clinical presentation which can range from subtle disturbances like headache to frank neurologic impairment. An important...
Rise in intracranial tension (ICT) has varied clinical presentation which can range from subtle disturbances like headache to frank neurologic impairment. An important aspect is rapidity of rise of ICT. Pseudotumor cerebri is associated with many syndromes, toxication, and drugs. Our case is a unique one given the rarity of eltroxin, which is otherwise relatively safe drug and commonly used in this part of the world, induced Pseudotumor cerebri. Our patient had dramatic response to discontinuation of levothyroxine.
Topics: Headache; Humans; Pseudotumor Cerebri; Syndrome; Thyroxine
PubMed: 34747819
DOI: 10.4103/0028-3886.329602 -
Seminars in Pediatric Neurology May 2017Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial pressure in the setting of normal brain parenchyma and cerebrospinal fluid. PTCS... (Review)
Review
Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial pressure in the setting of normal brain parenchyma and cerebrospinal fluid. PTCS can occur in the pediatric and adult populations and, if untreated, may lead to permanent visual loss. In this review, discussion will focus on PTCS in the pediatric population and will outline its distinct epidemiology and key elements of diagnosis, evaluation and management. Finally, although the precise mechanisms are unclear, the underlying pathophysiology will be considered.
Topics: Brain; Child; Disease Management; Humans; Pseudotumor Cerebri
PubMed: 28941525
DOI: 10.1016/j.spen.2017.04.002 -
Indian Journal of Ophthalmology Oct 2014The aim was to identify Pseudotumor cerebri treatment options and assess their efficacy. (Review)
Review
AIMS
The aim was to identify Pseudotumor cerebri treatment options and assess their efficacy.
SETTING AND DESIGN
Review article.
MATERIALS AND METHODS
Existing literature and the authors' experience were reviewed.
RESULTS
Treatment options range from observation to surgical intervention. Weight loss and medical treatment may be utilized in cases without vision loss or in combination with surgical treatment. Cerebrospinal fluid shunting procedures and/or optic nerve sheath decompression is indicated for severe vision loss or headache unresponsive to medical management. The recent use of endovascular stenting of transverse sinus stenoses has also demonstrated benefit in patients with pseudotumor cerebri.
CONCLUSION
While each treatment form may be successful individually, a multimodal approach is typically utilized with treatments selected on a case-by-case basis.
Topics: Disease Management; Humans; Pseudotumor Cerebri
PubMed: 25449933
DOI: 10.4103/0301-4738.145991 -
Hormone Research in Paediatrics 2014Idiopathic intracranial hypertension (IIH), also known as primary pseudotumor cerebri syndrome (PTCS), is a condition of unknown etiology which affects primarily... (Review)
Review
Idiopathic intracranial hypertension (IIH), also known as primary pseudotumor cerebri syndrome (PTCS), is a condition of unknown etiology which affects primarily overweight, reproductive-aged women and causes increased intracranial pressure (ICP). This review discusses the recently revised diagnostic criteria for PTCS for adults and children. Additionally, the role of obesity in the epidemiology, etiology, and management of IIH as well as the current knowledge of obesity profiles and markers in IIH are reviewed. We also highlight the emerging, unifying theory of the neuroendocrine effects on the mineralocorticoid receptor to explain a possible mechanism for the increased cerebrospinal fluid production and ICP in secondary PTCS.
Topics: Adult; Child; Female; Humans; Male; Obesity; Pseudotumor Cerebri
PubMed: 24642712
DOI: 10.1159/000357730