Materiały źródłowe

• #1 Pseudotumor Cerebri – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK536924/

  Pseudotumor cerebri (PTC), also known as idiopathic intracranial hypertension (IIH), is a disorder with increased intracranial pressure (ICP) and associated headaches, papilledema, vision changes, or pulsatile tinnitus in the setting of normal imaging and cerebrospinal fluid (CSF) studies. It mainly affects overweight women of childbearing age. […] There are multiple hypotheses about the etiology of PTC, including decreased CSF absorption and/or increased CSF production. […] The primary etiology is an accumulation of CSF either through decreased resorption and/or increased production; this leads to elevated intracranial pressure, the source of the associated symptoms and signs. […] Proposed mechanisms involve the vascular, hormonal, and cellular systems. Vascular changes are among the most common radiologic findings; transverse sinus stenosis suggests a vascular component.

• #2 Update on the pathophysiology and management of idiopathic intracranial hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3544160/

  Idiopathic Intracranial Hypertension (IIH) is a disease of unknown etiology typically affecting young, obese women, producing a syndrome of increased intracranial pressure without identifiable cause. […] Despite a large number of hypotheses and publications over the past decade, the etiology is still unknown. Vitamin A metabolism, adipose tissue as an actively secreting endocrine tissue, and cerebral venous abnormalities are areas of active study regarding IIH’s pathophysiology. […] There are multiple theories regarding the pathophysiology of this disorder, but there is no unifying hypothesis. […] The pathophysiologic mechanisms underlying the raised ICP in IIH remain unclear, but those proposed classically include increased brain water content, excess cerebrospinal fluid (CSF) production, reduced CSF absorption, and increased cerebral venous pressure.

• #3 Update on the pathophysiology and management of idiopathic intracranial hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3544160/

  Idiopathic Intracranial Hypertension (IIH) is a disease of unknown etiology typically affecting young, obese women, producing a syndrome of increased intracranial pressure without identifiable cause. […] Despite a large number of hypotheses and publications over the past decade, the etiology is still unknown. Vitamin A metabolism, adipose tissue as an actively secreting endocrine tissue, and cerebral venous abnormalities are areas of active study regarding IIH’s pathophysiology. […] There are multiple theories regarding the pathophysiology of this disorder, but there is no unifying hypothesis. […] The pathophysiologic mechanisms underlying the raised ICP in IIH remain unclear, but those proposed classically include increased brain water content, excess cerebrospinal fluid (CSF) production, reduced CSF absorption, and increased cerebral venous pressure.

• #4 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology characterized by chronically elevated intracranial pressure (ICP), and the most important neurologic manifestation is papilledema. […] The pathophysiology of IIH is unclear. An initial theory thought that cerebral edema played a role in the pathogenesis of elevated ICP in these patients, but later reports described the edema to represent fixation artifact (ie, from tissue preparation) rather than in vivo edema. […] Although the precise mechanism is not understood, IIH is likely due to a dysregulation of CSF dynamics involving a blend of hypersecretion of CSF at the choroidal plexus, reduced reabsorption at the arachnoid granulations, and abnormal venous pressure gradients. […] In a series reported by Farb et al, 29 patients with IIH showed demonstrable narrowing of the transverse dural venous sinus on magnetic resonance (MR) venography, whereas none of the 59 control subjects had this finding.

• #5 Idiopathic Intracranial Hypertension

  https://practicalneurology.com/articles/2020-may/idiopathic-intracranial-hypertension

  Multiple mechanisms have been postulated for IIH; however, the exact pathophysiology is unknown. Hypersecretion of CSF, outflow obstruction, and increased venous sinus pressures are 3 main mechanisms speculated to contribute to IIH. […] Hypersecretion of CSF in IIH may be linked to variations in aquaporin-1, which facilitates CSF secretion from the choroid plexus. Other studies have identified a breakdown of the blood-brain barrier in people with IIH and suggest a role for aquaporin-4. […] As etiologies of increased ICP, CSF outflow obstruction and venous sinus pressure may actually be one and the same. Smooth bilateral venous sinus stenosis is commonly observed in IIH. Disruption in the venous-CSF gradient caused by increased venous sinus pressure may reduce CSF outflow. […] Typically, IIH affects obese women of reproductive age. The relationship between obesity and elevated ICP may relate to increased intrathoracic pressure as a consequence of increased abdominal mass.

• #6 Evolving evidence in adult idiopathic intracranial hypertension: pathophysiology and management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/87/9/982

  Idiopathic intracranial hypertension (IIH) is a rare but important disease associated with significant morbidity. […] The pathophysiology of IIH is incompletely characterised; suggested underpinning mechanisms include the role of cerebrospinal fluid regulation as well as metabolic and endocrinological perspectives. […] The underlying pathogenesis of IIH is uncertain. Raised ICP is a uniform characteristic, but the mechanism by which ICP is elevated in IIH is not clear. […] Changes in the volume of blood, CSF and brain tissue influence ICP. IIH likely represents a disorder of CSF regulation, potentially through CSF hypersecretion or reduced drainage. […] The choroid plexus is the primary site of CSF secretion, generating around two-thirds of the total CSF produced, with the rest coming from extrachoroidal sources, such as the ependyma and possibly the blood brain barrier.

• #7 Pseudotumor Cerebri – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK536924/

  Pseudotumor cerebri (PTC), also known as idiopathic intracranial hypertension (IIH), is a disorder with increased intracranial pressure (ICP) and associated headaches, papilledema, vision changes, or pulsatile tinnitus in the setting of normal imaging and cerebrospinal fluid (CSF) studies. It mainly affects overweight women of childbearing age. […] There are multiple hypotheses about the etiology of PTC, including decreased CSF absorption and/or increased CSF production. […] The primary etiology is an accumulation of CSF either through decreased resorption and/or increased production; this leads to elevated intracranial pressure, the source of the associated symptoms and signs. […] Proposed mechanisms involve the vascular, hormonal, and cellular systems. Vascular changes are among the most common radiologic findings; transverse sinus stenosis suggests a vascular component.

• #8 Pseudotumor Cerebri – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK536924/

  Hormonal changes seen include Aldosterone excess (associated with obesity and PCOS), which commonly correlates with PTC and is suggested to affect the mineralocorticoid receptor of the choroid plexus, leading to increased CSF production. […] Cellular changes seen include increased outflow resistance to CSF, which has been demonstrated in multiple experimental studies and is the leading theory for the causation of PTC.

• #9 Idiopathic Intracranial Hypertension, Pseudotumor cerebri – EyeRounds.org – Ophthalmology – The University of Iowa

  http://eyerounds.org/cases/99-Pseudotumor-Cerebri.htm

  Idiopathic Intracranial Hypertension (IIH) is a condition in which the cerebrospinal fluid (CSF) is under high pressure in the absence of an intracranial mass, venous sinus thrombosis or other primary cause. It is also known as pseudotumor cerebri because patients present with signs and symptoms of a brain tumor without a brain tumor being present. […] In IIH, there is no structural obstruction to the circulation of CSF. It is believed that the problem lies in defective reabsorption of CSF either at the level of the arachnoid granulations or along cranial and spinal nerve root sheaths. There is no evidence for excessive CSF production. […] There is a female preponderance for this disease, and the peak age of onset is in the third decade of life. It is common for affected patients to be obese, and weight loss is an important part of treatment. IIH symptoms often start or worsen during a period of weight gain and resolve with 5-10% total weight loss.

• #10 Idiopathic Intracranial Hypertension

  https://practicalneurology.com/diseases-diagnoses/headache-pain/idiopathic-intracranial-hypertension/31655/

  Multiple mechanisms have been postulated for IIH; however, the exact pathophysiology is unknown. Hypersecretion of CSF, outflow obstruction, and increased venous sinus pressures are 3 main mechanisms speculated to contribute to IIH. […] Hypersecretion of CSF in IIH may be linked to variations in aquaporin-1, which facilitates CSF secretion from the choroid plexus. Other studies have identified a breakdown of the blood-brain barrier in people with IIH and suggest a role for aquaporin-4. […] As etiologies of increased ICP, CSF outflow obstruction and venous sinus pressure may actually be one and the same. Smooth bilateral venous sinus stenosis is commonly observed in IIH. Disruption in the venous-CSF gradient caused by increased venous sinus pressure may reduce CSF outflow. […] Typically, IIH affects obese women of reproductive age. The relationship between obesity and elevated ICP may relate to increased intrathoracic pressure as a consequence of increased abdominal mass. […] Etiology of headache in IIH is likely multifactorial. The pressure exerted on meninges likely activates the trigeminovascular system, leading to migraine-like symptoms. […] The pathophysiology of IIH remains elusive and IIH has serious consequences for vision if it is not treated quickly.

• #11 Idiopathic intracranial hypertension – Wikipedia

  https://en.wikipedia.org/wiki/Idiopathic_intracranial_hypertension

  Idiopathic intracranial hypertension (IIH), previously known as pseudotumor cerebri and benign intracranial hypertension, is a condition characterized by increased intracranial pressure (pressure around the brain) without a detectable cause. […] The cause of IIH is not known. The Monro-Kellie rule states that the intracranial pressure is determined by the amount of brain tissue, cerebrospinal fluid (CSF) and blood inside the bony cranial vault. Three theories therefore exist as to why the pressure might be raised in IIH: an excess of CSF production, increased volume of blood or brain tissue, or obstruction of the veins that drain blood from the brain. […] The first theory, that of increased production of cerebrospinal fluid, was proposed in early descriptions of the disease. However, there is no experimental data that supports a role for this process in IIH.

• #12 Idiopathic intracranial hypertension – wikidoc

  https://www.wikidoc.org/index.php/Idiopathic_intracranial_hypertension

  At least two primary mechanisms for the development of increased CSF pressure in primary IIH have been postulated: increased production of CSF and reduced resorption. Increased production may be the result of vasogenic extracellular brain edema, while decreased rebsorption may be due to low conductance of CSF outflow at the arachnoid villi.[2][3] […] It was argued that this evidence indicates abnormal convective transependymal water flow leading to brain edema. However, this theory remains controversial, as a similar study conducted by Bastin that used significantly disparate MR imaging protocols was unable to reproduce these findings.[4] […] Impaired CSF absorption at the superior sagittal sinus or along the spinal nerve roots could therefore explain IIH and has been documented in 75-100% of IIH patients.[2]

• #13 Idiopathic Intracranial Hypertension, Pseudotumor cerebri – EyeRounds.org – Ophthalmology – The University of Iowa

  http://eyerounds.org/cases/99-Pseudotumor-Cerebri.htm

  Idiopathic Intracranial Hypertension (IIH) is a condition in which the cerebrospinal fluid (CSF) is under high pressure in the absence of an intracranial mass, venous sinus thrombosis or other primary cause. It is also known as pseudotumor cerebri because patients present with signs and symptoms of a brain tumor without a brain tumor being present. […] In IIH, there is no structural obstruction to the circulation of CSF. It is believed that the problem lies in defective reabsorption of CSF either at the level of the arachnoid granulations or along cranial and spinal nerve root sheaths. There is no evidence for excessive CSF production. […] There is a female preponderance for this disease, and the peak age of onset is in the third decade of life. It is common for affected patients to be obese, and weight loss is an important part of treatment. IIH symptoms often start or worsen during a period of weight gain and resolve with 5-10% total weight loss.

• #14 Pseudotumor Cerebri – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK536924/

  Pseudotumor cerebri (PTC), also known as idiopathic intracranial hypertension (IIH), is a disorder with increased intracranial pressure (ICP) and associated headaches, papilledema, vision changes, or pulsatile tinnitus in the setting of normal imaging and cerebrospinal fluid (CSF) studies. It mainly affects overweight women of childbearing age. […] There are multiple hypotheses about the etiology of PTC, including decreased CSF absorption and/or increased CSF production. […] The primary etiology is an accumulation of CSF either through decreased resorption and/or increased production; this leads to elevated intracranial pressure, the source of the associated symptoms and signs. […] Proposed mechanisms involve the vascular, hormonal, and cellular systems. Vascular changes are among the most common radiologic findings; transverse sinus stenosis suggests a vascular component.

• #15 Update on the pathophysiology and management of idiopathic intracranial hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3544160/

  It seems that some patients in particular (such as obese patients, especially young women, and those with anomalous distal transverse sinuses resulting in bilateral transverse sinus stenoses (TSS)) are predisposed to developing raised ICP, which might be triggered by specific events or situations, such as weight gain, endocrine changes, hypercoagulable states, specific medications, and OSA. […] One long-standing hypothesis for the pathogenesis of IIH involves abnormal vitamin A metabolism. […] These observations regarding vitamin A may be linked to another area of emerging interest in endocrinology and IIH, the nature of adipose tissue as an actively secreting endocrine tissue. […] Increased intracranial venous pressure related to stenosis of the distal portion of the transverse cerebral sinuses is another proposed mechanism of IIH that has received substantial recent interest.

• #16 Update on the pathophysiology and management of idiopathic intracranial hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3544160/

  Numerous recent publications have shown that such transverse sinus stenoses (TSS) are found in a large majority of patients with IIH. […] Another venous mechanism proposed in the development of increased ICP in IIH patients is the occurrence of microthrombosis from thrombophilia within the cerebral veins, resulting in impaired CSF absorption.

• #17 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology characterized by chronically elevated intracranial pressure (ICP), and the most important neurologic manifestation is papilledema. […] The pathophysiology of IIH is unclear. An initial theory thought that cerebral edema played a role in the pathogenesis of elevated ICP in these patients, but later reports described the edema to represent fixation artifact (ie, from tissue preparation) rather than in vivo edema. […] Although the precise mechanism is not understood, IIH is likely due to a dysregulation of CSF dynamics involving a blend of hypersecretion of CSF at the choroidal plexus, reduced reabsorption at the arachnoid granulations, and abnormal venous pressure gradients. […] In a series reported by Farb et al, 29 patients with IIH showed demonstrable narrowing of the transverse dural venous sinus on magnetic resonance (MR) venography, whereas none of the 59 control subjects had this finding.

• #18 Pseudotumor Cerebri: Brief Review of Clinical Syndrome and Imaging Findings | American Journal of Neuroradiology

  http://www.ajnr.org/content/32/11/1986

  PTC is a clinical entity of uncertain etiology characterized by intracranial hypertension. […] The precise etiology of IIH, as the name suggests, is largely unknown, despite much clinical investigation and basic science research. […] Many cases of PTC may not, in fact, be idiopathic but rather secondary to venous thrombosis, which many argue is frequently missed if MR venography is not used to evaluate patients with suspected PTC. […] A newer theory stemming from observations of sinus thrombosis producing a clinical presentation similar to IIH suggests that most cases of IIH are due to venous outflow obstruction, which can also take the form of stenosis in the absence of thrombosis. […] Even if it is assumed that venous stenosis exists in PTC, there is controversy as to whether venous stenosis and elevated venous pressure are due to elevated ICP or constitute the proximal cause of PTC. […] The first explanation is an application of the Monroe-Kellie doctrine: Expansion of the brain within a fixed space will lead to the compression of venous sinuses, assuming that these are compressible structures, to vent CSF during systole when ICP increases.

• #19 Pseudotumor Cerebri and Papilledema – Glaucoma Today

  https://glaucomatoday.com/articles/2019-jan-feb/pseudotumor-cerebri-and-papilledema

  Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), describes the perplexing syndrome of increased intracranial pressure (ICP) in the absence of a space-occupying lesion on neuroimaging or other etiology. […] Although IIH is, by definition, idiopathic, increased ICP can be caused by increased cerebrospinal fluid (CSF) production, reduced CSF absorption, increased cerebral venous pressure, venous sinus stenosis, increased brain water content, or a combination of these mechanisms. […] One theory has postulated elevated intracranial venous pressure as both the primary mechanism and a final common pathway for IIH. […] This theory is supported by the similar clinical appearance of IIH and secondary intracranial hypertension due to cerebral venous thrombosis and other causes of obstructed venous outflow.

• #20 Pseudotumor cerebri | STROKE MANUAL

  https://www.stroke-manual.com/pseudotumor-cerebri/

  pseudotumor cerebri (idiopathic intracranial hypertension IIH) is a clinical condition characterized by an increase in intracranial pressure without evidence of intracranial space-occupying lesion, infection, hydrocephalus, or other demonstrable cause […] idiopathic intracranial hypertension IIH is reserved for patients with NO demonstrable cause […] the exact cause is unknown (as its name implies), but it is associated with some diseases or the use of certain drugs […] it is necessary to distinguish secondary forms of intracranial hypertension […] proposed mechanisms: reduced CSF absorption (as evidenced by the findings of radioisotope cisternography and other studies) […] reduced venous outflow from the brain due to truncal obesity (due to an increase in intra-abdominal pressure and an increase in cardiac filling pressure) […] stenosis in the venous system is increasingly recognized as an important factor […] the efficacy of venous stenting suggests a causal link

• #21 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #22 Intracranial hypertension due to spinal cord tumor misdiagnosed as pseudotumor cerebri syndrome: case report | BMC Neurology | Full Text

  https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-020-02000-y

  The above cases indicate that isolated initial manifestation of ICH with lack of localizing signs in the spinal cord could lead to misdiagnosis and delayed surgical intervention, which ultimately, could result in disastrous consequences. […] Interestingly, our patient was misdiagnosed as having IIH accompanied by TSS. […] Given the strong evidence gathered over the past two decades, venous sinus stenosis, in particular, TSS, has long been viewed as a contributor to the pathophysiology of IIH. […] It is postulated that in patients with IIH, venous sinus stenosis and a subsequent increase in venous pressure initially appear to be the downstream consequence of elevated CSF pressure. […] The findings of Morandi et al. in cases of benign spinal cord tumor support this theory. […] As yet, the pathophysiological mechanism underlying the association between spinal tumors and ICH is not well established.

• #23 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology characterized by chronically elevated intracranial pressure (ICP), and the most important neurologic manifestation is papilledema. […] The pathophysiology of IIH is unclear. An initial theory thought that cerebral edema played a role in the pathogenesis of elevated ICP in these patients, but later reports described the edema to represent fixation artifact (ie, from tissue preparation) rather than in vivo edema. […] Although the precise mechanism is not understood, IIH is likely due to a dysregulation of CSF dynamics involving a blend of hypersecretion of CSF at the choroidal plexus, reduced reabsorption at the arachnoid granulations, and abnormal venous pressure gradients. […] In a series reported by Farb et al, 29 patients with IIH showed demonstrable narrowing of the transverse dural venous sinus on magnetic resonance (MR) venography, whereas none of the 59 control subjects had this finding.

• #24 Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) – EyeWiki

  https://eyewiki.org/Pseudotumor_Cerebri_(Idiopathic_Intracranial_Hypertension)

  Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), is a disorder characterized by increased intracranial pressure (ICP) of unknown cause that predominantly affects obese women of childbearing age. […] The pathophysiology remains unclear but multiple hypotheses have been suggested. […] Another proposed mechanism involves stenosis of the distal portion of the transverse venous sinuses. This stenosis can result in cerebral venous hypertension and impaired CSF absorption. […] Some suggested that an increase in intraabdominal pressure, secondary to obesity, causes increased cardiac filling pressure which impedes venous return from the brain and subsequently leads to an elevated intracranial venous pressure and IIH. […] Other studies have suggested a role for vitamin A in IIH pathogenesis based on elevated serum and CSF vitamin A, retinol, and retinol binding protein levels reported in IIH patients. […] It has also been proposed that there may be microthrombosis in the sagittal sinus, of insufficient size to be seen on neuroimaging studies, which is blocking CSF absorption in the arachnoid granulations.

• #25 Pseudotumor Cerebri: Categorical Disease or Spectrum of Disease | Walid | Journal of Neurology Research

  https://www.neurores.org/index.php/neurores/article/view/12/5

  Pseudotumor cerebri, aka. idiopathic intracranial hypertension (PTC), is characterized by increased intracranial pressure and papilledema in the absence of other neurologic localizing signs. […] PTC’s etiology is not well understood. Many theories exist on PTC’s pathomechanism. The mainstream of clinical research link this disease to the water retention effect of estrogens on the central nervous system. […] Less popular theories talk about disturbances in the hypothalamic-hypophyseal-ovarian axis in response to stressful triggers, an allergic Jarisch-Herxheimer-type reaction to some undetermined infectious or noninfectious stimuli or theories that incriminate thrombophilia and/or hypofibrinolysis that lead to microthrombi in the choroid plexus leading to decreased drainage of the cerebrospinal fluid (CSF) and increased intracranial pressure.

• #26 Evolving evidence in adult idiopathic intracranial hypertension: pathophysiology and management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/87/9/982

  Dysregulation of fluid transport in IIH may be important. […] Obesity is a consistent risk factor for the development of IIH. […] Despite the association between IIH and an obese phenotype, the pathological mechanisms tying the two together are unclear, and IIH is a rare disorder, while obesity is common. […] Improvements in brain venography imaging reveal that most patients with IIH have anatomical abnormalities of the cerebral venous sinus system. […] The burden on the health service is significant and costly due to high healthcare utilisation by patients. […] Recent research momentum has led to important progress in our understanding and management of IIH.

• #27 Idiopathic Intracranial Hypertension

  https://practicalneurology.com/articles/2020-may/idiopathic-intracranial-hypertension

  Multiple mechanisms have been postulated for IIH; however, the exact pathophysiology is unknown. Hypersecretion of CSF, outflow obstruction, and increased venous sinus pressures are 3 main mechanisms speculated to contribute to IIH. […] Hypersecretion of CSF in IIH may be linked to variations in aquaporin-1, which facilitates CSF secretion from the choroid plexus. Other studies have identified a breakdown of the blood-brain barrier in people with IIH and suggest a role for aquaporin-4. […] As etiologies of increased ICP, CSF outflow obstruction and venous sinus pressure may actually be one and the same. Smooth bilateral venous sinus stenosis is commonly observed in IIH. Disruption in the venous-CSF gradient caused by increased venous sinus pressure may reduce CSF outflow. […] Typically, IIH affects obese women of reproductive age. The relationship between obesity and elevated ICP may relate to increased intrathoracic pressure as a consequence of increased abdominal mass.

• #28 Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) – EyeWiki

  https://eyewiki.org/Pseudotumor_Cerebri_(Idiopathic_Intracranial_Hypertension)

  Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), is a disorder characterized by increased intracranial pressure (ICP) of unknown cause that predominantly affects obese women of childbearing age. […] The pathophysiology remains unclear but multiple hypotheses have been suggested. […] Another proposed mechanism involves stenosis of the distal portion of the transverse venous sinuses. This stenosis can result in cerebral venous hypertension and impaired CSF absorption. […] Some suggested that an increase in intraabdominal pressure, secondary to obesity, causes increased cardiac filling pressure which impedes venous return from the brain and subsequently leads to an elevated intracranial venous pressure and IIH. […] Other studies have suggested a role for vitamin A in IIH pathogenesis based on elevated serum and CSF vitamin A, retinol, and retinol binding protein levels reported in IIH patients. […] It has also been proposed that there may be microthrombosis in the sagittal sinus, of insufficient size to be seen on neuroimaging studies, which is blocking CSF absorption in the arachnoid granulations.

• #29 Idiopathic Intracranial Hypertension | Concise Medical Knowledge

  https://www.lecturio.com/concepts/idiopathic-intracranial-hypertension/

  The exact pathogenesis of IIH is unknown, although there are several proposed mechanisms. […] Cerebral venous outflow abnormalities increased ICP. […] Obesity aldosterone excess CSF production by the choroid plexus. […] Obesity intraabdominal pressure, pleural pressure, cardiac filling pressure, and central venous pressure intracranial venous pressure and IIH.

• #30 Pseudotumor Cerebri – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK536924/

  Hormonal changes seen include Aldosterone excess (associated with obesity and PCOS), which commonly correlates with PTC and is suggested to affect the mineralocorticoid receptor of the choroid plexus, leading to increased CSF production. […] Cellular changes seen include increased outflow resistance to CSF, which has been demonstrated in multiple experimental studies and is the leading theory for the causation of PTC.

• #31 Idiopathic intracranial hypertension, hormones, and 11B-hydroxys | JPR

  https://www.dovepress.com/idiopathic-intracranial-hypertension-hormones-and-11szlig-hydroxystero-peer-reviewed-fulltext-article-JPR

  The theory describes that stimulation of the mineralocorticoid receptors in the choroid plexus epithelium increases the activity of Na+/K+ ATPase, an active transporter of sodium for potassium ions. […] Thus, the movement of sodium ions into the cerebral ventricles increases, creating an osmotic gradient to drive CSF secretion and increase ICP. […] 11-HSD1 activity is highest in hepatic and adipose tissue but has also been identified in the brain, in particular the cerebellum, neocortex, hippocampus, choroid plexus, and arachnoid granulations. […] It is possible that this local cortisol generating system could drive CSF secretion at the choroid plexus epithelium. […] Given the relationship between 11-HSD1 and ICP, inhibitors of this enzyme could play a therapeutic role in IIH. […] The role 11-HSD1 may play is potentially important in the development of raised ICP in IIH and obesity.

• #32 Update on the pathophysiology and management of idiopathic intracranial hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3544160/

  It seems that some patients in particular (such as obese patients, especially young women, and those with anomalous distal transverse sinuses resulting in bilateral transverse sinus stenoses (TSS)) are predisposed to developing raised ICP, which might be triggered by specific events or situations, such as weight gain, endocrine changes, hypercoagulable states, specific medications, and OSA. […] One long-standing hypothesis for the pathogenesis of IIH involves abnormal vitamin A metabolism. […] These observations regarding vitamin A may be linked to another area of emerging interest in endocrinology and IIH, the nature of adipose tissue as an actively secreting endocrine tissue. […] Increased intracranial venous pressure related to stenosis of the distal portion of the transverse cerebral sinuses is another proposed mechanism of IIH that has received substantial recent interest.

• #33 Idiopathic intracranial hypertension, hormones, and 11B-hydroxys | JPR

  https://www.dovepress.com/idiopathic-intracranial-hypertension-hormones-and-11szlig-hydroxystero-peer-reviewed-fulltext-article-JPR

  Idiopathic intracranial hypertension (IIH) results in raised intracranial pressure (ICP) leading to papilledema, visual dysfunction, and headaches. […] The underlying pathological mechanisms behind IIH remain unknown. […] Despite occurring almost exclusively in obese women, there have been a few studies evaluating the mechanisms by which hormones and adipokines exert their effects on ICP regulation in IIH. […] Improved understanding of the complex interplay between adipose signaling factors such as adipokines, steroid hormones, and ICP regulation may be key to the understanding and future management of IIH. […] The etiology of this debilitating condition remains undetermined. Dysregulation of CSF dynamics is key and may involve hypersecretion of CSF at the choroid plexus, reduced drainage at the arachnoid granulations and/or lymphatics, and aberrant venous pressure gradients.

• #34 Evolving evidence in adult idiopathic intracranial hypertension: pathophysiology and management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/87/9/982

  Dysregulation of fluid transport in IIH may be important. […] Obesity is a consistent risk factor for the development of IIH. […] Despite the association between IIH and an obese phenotype, the pathological mechanisms tying the two together are unclear, and IIH is a rare disorder, while obesity is common. […] Improvements in brain venography imaging reveal that most patients with IIH have anatomical abnormalities of the cerebral venous sinus system. […] The burden on the health service is significant and costly due to high healthcare utilisation by patients. […] Recent research momentum has led to important progress in our understanding and management of IIH.

• #35 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #36 Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) | 5-Minute Pediatric Consult

  https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/618165/all/Idiopathic_Intracranial_Hypertension__Pseudotumor_Cerebri_?q=Hypertension

  Pathogenesis unknown but may involve decreased CSF absorption owing to arachnoid villi dysfunction or elevated intracranial venous pressure. For example, obesity may lead to increased intra-abdominal, intrathoracic, and cardiac filling pressure, leading to elevated intracranial venous pressure. […] Numerous precipitants of IIH have been reported. In adolescents, it is clearly associated with obesity and weight gain but not clearly linked to obesity in children 11 years of age. Many weaker associations may be due to chance.

• #37 Idiopathic intracranial hypertension, hormones, and 11B-hydroxys | JPR

  https://www.dovepress.com/idiopathic-intracranial-hypertension-hormones-and-11szlig-hydroxystero-peer-reviewed-fulltext-article-JPR

  Idiopathic intracranial hypertension (IIH) results in raised intracranial pressure (ICP) leading to papilledema, visual dysfunction, and headaches. […] The underlying pathological mechanisms behind IIH remain unknown. […] Despite occurring almost exclusively in obese women, there have been a few studies evaluating the mechanisms by which hormones and adipokines exert their effects on ICP regulation in IIH. […] Improved understanding of the complex interplay between adipose signaling factors such as adipokines, steroid hormones, and ICP regulation may be key to the understanding and future management of IIH. […] The etiology of this debilitating condition remains undetermined. Dysregulation of CSF dynamics is key and may involve hypersecretion of CSF at the choroid plexus, reduced drainage at the arachnoid granulations and/or lymphatics, and aberrant venous pressure gradients.

• #38 Idiopathic intracranial hypertension, hormones, and 11B-hydroxys | JPR

  https://www.dovepress.com/idiopathic-intracranial-hypertension-hormones-and-11szlig-hydroxystero-peer-reviewed-fulltext-article-JPR

  It is unlikely that a single anatomical location for CSF dysregulation is exclusively responsible for the raised ICP in IIH, and multiple sites and mechanisms may be involved in impairing ICP homeostasis. […] Given the very strong female preponderance, steroid hormones are also likely to be involved. […] This review provides an overview of the potential role of hormones, adipokines, and cytokines in IIH and describes new potential pathogenic mechanisms in this disease. […] However, a distinctive hormonal profile has not yet been described in IIH. […] These cases suggest a potential role for female sex hormones in IIH. […] It is not clear how sex hormones could alter ICP, but interestingly, studies in rabbits have demonstrated that estrogen and progesterone are able to reduce CSF secretion by the choroid plexus, on their own and in combination.

• #39

  https://journals.lww.com/neur/fulltext/2021/69002/idiopathic_intracranial_hypertension___challenges.28.aspx

  Central body fat causes an increase in central venous pressure and increase venous pressure, reducing CSF absorption. […] Women with IIH have been found to have a unique circulating androgen excess signature, with significantly higher active testosterone but lower concentrations of the androgen precursors dehydroepiandrosterone sulfate (DHEA) and androstenedione.

• #40 Idiopathic intracranial hypertension, hormones, and 11B-hydroxys | JPR

  https://www.dovepress.com/idiopathic-intracranial-hypertension-hormones-and-11szlig-hydroxystero-peer-reviewed-fulltext-article-JPR

  The theory describes that stimulation of the mineralocorticoid receptors in the choroid plexus epithelium increases the activity of Na+/K+ ATPase, an active transporter of sodium for potassium ions. […] Thus, the movement of sodium ions into the cerebral ventricles increases, creating an osmotic gradient to drive CSF secretion and increase ICP. […] 11-HSD1 activity is highest in hepatic and adipose tissue but has also been identified in the brain, in particular the cerebellum, neocortex, hippocampus, choroid plexus, and arachnoid granulations. […] It is possible that this local cortisol generating system could drive CSF secretion at the choroid plexus epithelium. […] Given the relationship between 11-HSD1 and ICP, inhibitors of this enzyme could play a therapeutic role in IIH. […] The role 11-HSD1 may play is potentially important in the development of raised ICP in IIH and obesity.

• #41 An integrated mechanism of pediatric pseudotumor cerebri syndrome: evidence of bioenergetic and hormonal regulation of cerebrospinal fluid dynamics | Pediatric Research

  https://www.nature.com/articles/pr2014188

  Multiple lines of evidence provide support for the hypothesis that the pathophysiology of PTCS involves aberrant glucocorticoid metabolism. […] The role of this system in the dynamics of CSF production and absorption is incompletely understood; however, we suggest that proportional upregulation of HSD1 is a compensatory measure to limit existing cortisol deficiencies. […] Aldosterone appears to augment the activities of epithelial sodium channels and Na+/K+ ATPase transporters to reduce CSF potassium concentrations while increasing CSF sodium concentrations. […] The first reports of recombinant growth hormone (rGH) or IGF-1 therapy causing PTCS were published in the early 1990s. […] Both hyper- and hypothyroidism are associated with abnormal renal fluid handling, effects mediated by reduced protein abundance of renal sodium-dependent transporters and water channels.

• #42 An integrated mechanism of pediatric pseudotumor cerebri syndrome: evidence of bioenergetic and hormonal regulation of cerebrospinal fluid dynamics | Pediatric Research

  https://www.nature.com/articles/pr2014188

  Multiple lines of evidence provide support for the hypothesis that the pathophysiology of PTCS involves aberrant glucocorticoid metabolism. […] The role of this system in the dynamics of CSF production and absorption is incompletely understood; however, we suggest that proportional upregulation of HSD1 is a compensatory measure to limit existing cortisol deficiencies. […] Aldosterone appears to augment the activities of epithelial sodium channels and Na+/K+ ATPase transporters to reduce CSF potassium concentrations while increasing CSF sodium concentrations. […] The first reports of recombinant growth hormone (rGH) or IGF-1 therapy causing PTCS were published in the early 1990s. […] Both hyper- and hypothyroidism are associated with abnormal renal fluid handling, effects mediated by reduced protein abundance of renal sodium-dependent transporters and water channels.

• #43 An integrated mechanism of pediatric pseudotumor cerebri syndrome: evidence of bioenergetic and hormonal regulation of cerebrospinal fluid dynamics | Pediatric Research

  https://www.nature.com/articles/pr2014188

  Multiple lines of evidence provide support for the hypothesis that the pathophysiology of PTCS involves aberrant glucocorticoid metabolism. […] The role of this system in the dynamics of CSF production and absorption is incompletely understood; however, we suggest that proportional upregulation of HSD1 is a compensatory measure to limit existing cortisol deficiencies. […] Aldosterone appears to augment the activities of epithelial sodium channels and Na+/K+ ATPase transporters to reduce CSF potassium concentrations while increasing CSF sodium concentrations. […] The first reports of recombinant growth hormone (rGH) or IGF-1 therapy causing PTCS were published in the early 1990s. […] Both hyper- and hypothyroidism are associated with abnormal renal fluid handling, effects mediated by reduced protein abundance of renal sodium-dependent transporters and water channels.

• #44 Update on the pathophysiology and management of idiopathic intracranial hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3544160/

  It seems that some patients in particular (such as obese patients, especially young women, and those with anomalous distal transverse sinuses resulting in bilateral transverse sinus stenoses (TSS)) are predisposed to developing raised ICP, which might be triggered by specific events or situations, such as weight gain, endocrine changes, hypercoagulable states, specific medications, and OSA. […] One long-standing hypothesis for the pathogenesis of IIH involves abnormal vitamin A metabolism. […] These observations regarding vitamin A may be linked to another area of emerging interest in endocrinology and IIH, the nature of adipose tissue as an actively secreting endocrine tissue. […] Increased intracranial venous pressure related to stenosis of the distal portion of the transverse cerebral sinuses is another proposed mechanism of IIH that has received substantial recent interest.

• #45

  https://www.omim.org/entry/243200

  Buchheit et al. (1969) described 2 sisters with idiopathic intracranial hypertension with papilledema (pseudotumor cerebri). […] Warner et al. (2002) quantified vitamin A in the cerebrospinal fluid of patients with idiopathic intracranial hypertension, elevated intracranial pressure of other causes, and normal intracranial pressure. There was a significantly higher level of vitamin A in the cerebrospinal fluid of some patients with idiopathic intracranial hypertension. The authors concluded that vitamin A toxicity might play a role in the pathogenesis of idiopathic intracranial hypertension. […] Katz et al. (2002) demonstrated the presence of both somatostatin receptor types 1 (182451) and 2 (182452) in all samples of normal human choroid plexus and arachnoid granulations. The authors proposed that these receptors might be involved in the processes of cerebrospinal fluid production and absorption and, thus, might play a role in the increased intracranial pressure of idiopathic intracranial hypertension.

• #46 Vitamin A and Idiopathic Intracranial Hypertension | Pediatric Neurology Briefs

  https://pediatricneurologybriefs.com/articles/10.15844/pedneurbriefs-16-11-5

  Vitamin A levels were measured in the cerebrospinal fluid of a total of 78 patients having idiopathic intracranial hypertension (IIH;n=20), elevated pressure of other causes (E-ICP;n=19), and normal pressure (N-ICP;n=39), in a study at the University of Utah, Salt Lake City, UT. […] Higher CSF vitamin A levels found in some patients with IIH were significantly correlated (p=0.036) when compared with control E-ICP and IIH patients. […] High vitamin A levels in patients with IIH and high average vitamin A levels in patients with breakdown of the blood-brain barrier are suggestive of a toxic effect of CSF retinol on the arachnoid granulation resorption mechanism in IIH. […] Vitamin A may have a specific transport mechanism into the CSF, and it becomes toxic when the level exceeds the RBP binding capacity.

• #47 Vitamin A and Idiopathic Intracranial Hypertension | Pediatric Neurology Briefs

  https://pediatricneurologybriefs.com/articles/10.15844/pedneurbriefs-16-11-5

  Study of patients with breakdown of the blood-brain barrier may elucidate the transport mechanism of CSF vitamin A and pathogenesis of IIH. […] It is speculated that vitamin A increases CSF volume and pressure by an effect on aquaporins present in membranes of the choroid plexus that control CSF secretion.

• #48 Vitamin A and Idiopathic Intracranial Hypertension | Pediatric Neurology Briefs

  https://pediatricneurologybriefs.com/articles/10.15844/pedneurbriefs-16-11-5

  Vitamin A levels were measured in the cerebrospinal fluid of a total of 78 patients having idiopathic intracranial hypertension (IIH;n=20), elevated pressure of other causes (E-ICP;n=19), and normal pressure (N-ICP;n=39), in a study at the University of Utah, Salt Lake City, UT. […] Higher CSF vitamin A levels found in some patients with IIH were significantly correlated (p=0.036) when compared with control E-ICP and IIH patients. […] High vitamin A levels in patients with IIH and high average vitamin A levels in patients with breakdown of the blood-brain barrier are suggestive of a toxic effect of CSF retinol on the arachnoid granulation resorption mechanism in IIH. […] Vitamin A may have a specific transport mechanism into the CSF, and it becomes toxic when the level exceeds the RBP binding capacity.

• #49 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #50 Idiopathic Intracranial Hypertension

  https://practicalneurology.com/diseases-diagnoses/headache-pain/idiopathic-intracranial-hypertension/31655/

  Multiple mechanisms have been postulated for IIH; however, the exact pathophysiology is unknown. Hypersecretion of CSF, outflow obstruction, and increased venous sinus pressures are 3 main mechanisms speculated to contribute to IIH. […] Hypersecretion of CSF in IIH may be linked to variations in aquaporin-1, which facilitates CSF secretion from the choroid plexus. Other studies have identified a breakdown of the blood-brain barrier in people with IIH and suggest a role for aquaporin-4. […] As etiologies of increased ICP, CSF outflow obstruction and venous sinus pressure may actually be one and the same. Smooth bilateral venous sinus stenosis is commonly observed in IIH. Disruption in the venous-CSF gradient caused by increased venous sinus pressure may reduce CSF outflow. […] Typically, IIH affects obese women of reproductive age. The relationship between obesity and elevated ICP may relate to increased intrathoracic pressure as a consequence of increased abdominal mass. […] Etiology of headache in IIH is likely multifactorial. The pressure exerted on meninges likely activates the trigeminovascular system, leading to migraine-like symptoms. […] The pathophysiology of IIH remains elusive and IIH has serious consequences for vision if it is not treated quickly.

• #51 Idiopathic Intracranial Hypertension

  https://practicalneurology.com/articles/2020-may/idiopathic-intracranial-hypertension

  Multiple mechanisms have been postulated for IIH; however, the exact pathophysiology is unknown. Hypersecretion of CSF, outflow obstruction, and increased venous sinus pressures are 3 main mechanisms speculated to contribute to IIH. […] Hypersecretion of CSF in IIH may be linked to variations in aquaporin-1, which facilitates CSF secretion from the choroid plexus. Other studies have identified a breakdown of the blood-brain barrier in people with IIH and suggest a role for aquaporin-4. […] As etiologies of increased ICP, CSF outflow obstruction and venous sinus pressure may actually be one and the same. Smooth bilateral venous sinus stenosis is commonly observed in IIH. Disruption in the venous-CSF gradient caused by increased venous sinus pressure may reduce CSF outflow. […] Typically, IIH affects obese women of reproductive age. The relationship between obesity and elevated ICP may relate to increased intrathoracic pressure as a consequence of increased abdominal mass.

• #52 Idiopathic Intracranial Hypertension

  https://practicalneurology.com/articles/2020-may/idiopathic-intracranial-hypertension

  Multiple mechanisms have been postulated for IIH; however, the exact pathophysiology is unknown. Hypersecretion of CSF, outflow obstruction, and increased venous sinus pressures are 3 main mechanisms speculated to contribute to IIH. […] Hypersecretion of CSF in IIH may be linked to variations in aquaporin-1, which facilitates CSF secretion from the choroid plexus. Other studies have identified a breakdown of the blood-brain barrier in people with IIH and suggest a role for aquaporin-4. […] As etiologies of increased ICP, CSF outflow obstruction and venous sinus pressure may actually be one and the same. Smooth bilateral venous sinus stenosis is commonly observed in IIH. Disruption in the venous-CSF gradient caused by increased venous sinus pressure may reduce CSF outflow. […] Typically, IIH affects obese women of reproductive age. The relationship between obesity and elevated ICP may relate to increased intrathoracic pressure as a consequence of increased abdominal mass.

• #53 Idiopathic intracranial hypertension | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/idiopathic-intracranial-hypertension-1?embed_domain=hackmd.io%25252f%252540yipuafecsl2jsu8smr5njq%25252fbnjhjgjghjghjgh&lang=us

  Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a syndrome with signs and symptoms of increased intracranial pressure but where a causative mass or hydrocephalus is not identified. […] The pathogenesis is poorly understood. Various mechanisms have been proposed, including decreased CSF absorption, increased CSF production, increased intravascular volume, increased intracranial venous pressure, hormonal changes, altered aquaporin-4 channels and abnormality in function of the glymphatic system. […] Venous sinus stenosis is increasingly recognized as an important factor although whether it is the primary inciting abnormality or a potentiating factor remains to be fully established. […] A study also found an association between decreased glymphatic clearance and papilledema in patients with IIH.

• #54

  https://www.omim.org/entry/243200

  Buchheit et al. (1969) described 2 sisters with idiopathic intracranial hypertension with papilledema (pseudotumor cerebri). […] Warner et al. (2002) quantified vitamin A in the cerebrospinal fluid of patients with idiopathic intracranial hypertension, elevated intracranial pressure of other causes, and normal intracranial pressure. There was a significantly higher level of vitamin A in the cerebrospinal fluid of some patients with idiopathic intracranial hypertension. The authors concluded that vitamin A toxicity might play a role in the pathogenesis of idiopathic intracranial hypertension. […] Katz et al. (2002) demonstrated the presence of both somatostatin receptor types 1 (182451) and 2 (182452) in all samples of normal human choroid plexus and arachnoid granulations. The authors proposed that these receptors might be involved in the processes of cerebrospinal fluid production and absorption and, thus, might play a role in the increased intracranial pressure of idiopathic intracranial hypertension.

• #55 Digoxin as a Treatment for Patients With Idiopathic Intracranial Hypertension

  https://www.degruyterbrill.com/document/doi/10.7556/jaoa.2014.039/html?lang=en&srsltid=AfmBOorXf7O_sEgENAcaf7_UtKyZ9O3b8Oj9bKasV0KmkkOlb_g3FoL-

  Idiopathic intracranial hypertension (IIH) sometimes called pseudotumor cerebri is a neurologic condition distinguished by any of the following symptoms: headache, increased cerebrospinal fluid pressure, papilledema, vision loss, diplopia, tinnitus, deafness, nausea and vomiting, or sixth nerve palsy. […] The pathophysiologic process of IIH is uncertain. One theory posits that there is resistance of CSF outflow at the level of the arachnoid granulations. Other theories attribute the condition to abnormalities of the cerebral venous outflow or a change in sodium and water retention mechanisms. […] Neblett et al suggested that digoxin can be beneficial to patients with IIH. […] Digoxin acts on the ouabain-sensitive Na+/K+ ATPase receptors in the choroid plexus, thereby decreasing CSF production. […] They concluded that digoxin may be beneficial to conditions of increased CSF pressure. […] In patients with IIH and complicated medical conditions, physicians should consider digoxin. Even though the mechanism of action is not well understood, it clearly has benefits in this group of patients.

• #56 Digoxin as a Treatment for Patients With Idiopathic Intracranial Hypertension

  https://www.degruyter.com/document/doi/10.7556/jaoa.2014.039/html?lang=en

  Idiopathic intracranial hypertension (IIH) sometimes called pseudotumor cerebri is a neurologic condition distinguished by any of the following symptoms: headache, increased cerebrospinal fluid pressure, papilledema, vision loss, diplopia, tinnitus, deafness, nausea and vomiting, or sixth nerve palsy. […] The pathophysiologic process of IIH is uncertain. One theory posits that there is resistance of CSF outflow at the level of the arachnoid granulations. Other theories attribute the condition to abnormalities of the cerebral venous outflow or a change in sodium and water retention mechanisms. […] Neblett et al suggested that digoxin can be beneficial to patients with IIH. […] Digoxin acts on the ouabain-sensitive Na+/K+ ATPase receptors in the choroid plexus, thereby decreasing CSF production. […] Few cases of IIH managed with digoxin have been reported, to our knowledge. […] Even though the mechanism of action is not well understood, it clearly has benefits in this group of patients.

• #57 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #58 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #59 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #60 Idiopathic intracranial hypertension | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/idiopathic-intracranial-hypertension-1?embed_domain=hackmd.io%25252f%252540yipuafecsl2jsu8smr5njq%25252fbnjhjgjghjghjgh&lang=us

  Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a syndrome with signs and symptoms of increased intracranial pressure but where a causative mass or hydrocephalus is not identified. […] The pathogenesis is poorly understood. Various mechanisms have been proposed, including decreased CSF absorption, increased CSF production, increased intravascular volume, increased intracranial venous pressure, hormonal changes, altered aquaporin-4 channels and abnormality in function of the glymphatic system. […] Venous sinus stenosis is increasingly recognized as an important factor although whether it is the primary inciting abnormality or a potentiating factor remains to be fully established. […] A study also found an association between decreased glymphatic clearance and papilledema in patients with IIH.

• #61 Idiopathic intracranial hypertension | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/idiopathic-intracranial-hypertension-1?lang=us

  Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a syndrome with signs and symptoms of increased intracranial pressure but where a causative mass or hydrocephalus is not identified. […] The pathogenesis is poorly understood. Various mechanisms have been proposed, including decreased CSF absorption, increased CSF production, increased intravascular volume, increased intracranial venous pressure, hormonal changes, altered aquaporin-4 channels and abnormality in function of the glymphatic system. […] Venous sinus stenosis is increasingly recognized as an important factor although whether it is the primary inciting abnormality or a potentiating factor remains to be fully established. […] A study also found an association between decreased glymphatic clearance and papilledema in patients with IIH. Whether this is a primary driving cause or also the sequela of impaired venous outflow remains to be determined.

• #62

  https://journals.lww.com/neur/fulltext/2021/69002/idiopathic_intracranial_hypertension___challenges.28.aspx

  Idiopathic intracranial hypertension (IIH) is defined as a syndrome of raised intracranial pressure with normal imaging of the brain and cerebrospinal fluid (CSF) composition. […] The pathogenic mechanisms of IIH are still unclear. Multiple mechanisms have been suggested to explain the pathophysiology of IIH. […] Currently, the main pathogenic mechanism revolves around dysregulated CSF dynamics. […] A role of increase in the cerebral volume secondary to white matter edema or increase in the cerebral blood volume has been postulated, but with no evidence. […] One of the causes of IIH is the bilateral stenosis of the distal portion of the transverse cerebral sinuses which results in reduced absorption of CSF via the arachnoid granulations. […] It is suggested that the mitochondria in the perivascular astrocytes endfeet and neurons of IIH patients are pathological, resulting in an impaired metabolism at the neuro-glio-vascular interface and maybe a facet of IIH.

• #63

  https://journals.lww.com/neur/fulltext/2021/69002/idiopathic_intracranial_hypertension___challenges.28.aspx

  Idiopathic intracranial hypertension (IIH) is defined as a syndrome of raised intracranial pressure with normal imaging of the brain and cerebrospinal fluid (CSF) composition. […] The pathogenic mechanisms of IIH are still unclear. Multiple mechanisms have been suggested to explain the pathophysiology of IIH. […] Currently, the main pathogenic mechanism revolves around dysregulated CSF dynamics. […] A role of increase in the cerebral volume secondary to white matter edema or increase in the cerebral blood volume has been postulated, but with no evidence. […] One of the causes of IIH is the bilateral stenosis of the distal portion of the transverse cerebral sinuses which results in reduced absorption of CSF via the arachnoid granulations. […] It is suggested that the mitochondria in the perivascular astrocytes endfeet and neurons of IIH patients are pathological, resulting in an impaired metabolism at the neuro-glio-vascular interface and maybe a facet of IIH.

• #64 An integrated mechanism of pediatric pseudotumor cerebri syndrome: evidence of bioenergetic and hormonal regulation of cerebrospinal fluid dynamics | Pediatric Research

  https://www.nature.com/articles/pr2014188

  Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial pressure (ICP) in the setting of normal brain parenchyma and cerebrospinal fluid (CSF). […] The mechanism(s) underlying PTCS is unknown. […] PTCS occurring in the setting of associated endocrinopathies appears to be related to increased CSF production and secondarily raised resistance to CSF absorption. […] We propose that a range of metabolic and hormonal signals regulates CSF dynamics not only by influencing the resistance to CSF absorption but also by acting at the level of the choroid plexus epithelial cells to regulate CSF secretion. […] We hypothesize that in patients with PTCS, mitochondrial metabolites (glutamate and succinate), along with insulin, and steroid hormones (cortisol, 11-deoxycortisol, aldosterone, 11-deoxycorticosterone), regulate CSF production, by the choroid plexus, and CSF absorption, at the arachnoid villi, ultimately leading to raised ICP.

• #65 An integrated mechanism of pediatric pseudotumor cerebri syndrome: evidence of bioenergetic and hormonal regulation of cerebrospinal fluid dynamics | Pediatric Research

  https://www.nature.com/articles/pr2014188

  Thus, although not completely understood, through a combination of actions, both hypo- and hyperthyroidism may contribute to the development of PTCS. […] It is well-established that PTCS, in the adult and older pediatric populations, is associated with obesity. […] We present a comprehensive discussion of the hypothesis that pediatric PTCS is a neuroendocrine disorder.

• #66 Idiopathic intracranial hypertension, hormones, and 11B-hydroxys | JPR

  https://www.dovepress.com/idiopathic-intracranial-hypertension-hormones-and-11szlig-hydroxystero-peer-reviewed-fulltext-article-JPR

  It is unlikely that a single anatomical location for CSF dysregulation is exclusively responsible for the raised ICP in IIH, and multiple sites and mechanisms may be involved in impairing ICP homeostasis. […] Given the very strong female preponderance, steroid hormones are also likely to be involved. […] This review provides an overview of the potential role of hormones, adipokines, and cytokines in IIH and describes new potential pathogenic mechanisms in this disease. […] However, a distinctive hormonal profile has not yet been described in IIH. […] These cases suggest a potential role for female sex hormones in IIH. […] It is not clear how sex hormones could alter ICP, but interestingly, studies in rabbits have demonstrated that estrogen and progesterone are able to reduce CSF secretion by the choroid plexus, on their own and in combination.

• #67 Update on the pathophysiology and management of idiopathic intracranial hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3544160/

  It seems that some patients in particular (such as obese patients, especially young women, and those with anomalous distal transverse sinuses resulting in bilateral transverse sinus stenoses (TSS)) are predisposed to developing raised ICP, which might be triggered by specific events or situations, such as weight gain, endocrine changes, hypercoagulable states, specific medications, and OSA. […] One long-standing hypothesis for the pathogenesis of IIH involves abnormal vitamin A metabolism. […] These observations regarding vitamin A may be linked to another area of emerging interest in endocrinology and IIH, the nature of adipose tissue as an actively secreting endocrine tissue. […] Increased intracranial venous pressure related to stenosis of the distal portion of the transverse cerebral sinuses is another proposed mechanism of IIH that has received substantial recent interest.

• #68 Update on the pathophysiology and management of idiopathic intracranial hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3544160/

  It seems that some patients in particular (such as obese patients, especially young women, and those with anomalous distal transverse sinuses resulting in bilateral transverse sinus stenoses (TSS)) are predisposed to developing raised ICP, which might be triggered by specific events or situations, such as weight gain, endocrine changes, hypercoagulable states, specific medications, and OSA. […] One long-standing hypothesis for the pathogenesis of IIH involves abnormal vitamin A metabolism. […] These observations regarding vitamin A may be linked to another area of emerging interest in endocrinology and IIH, the nature of adipose tissue as an actively secreting endocrine tissue. […] Increased intracranial venous pressure related to stenosis of the distal portion of the transverse cerebral sinuses is another proposed mechanism of IIH that has received substantial recent interest.

• #69 Idiopathic intracranial hypertension – Wikipedia

  https://en.wikipedia.org/wiki/Idiopathic_intracranial_hypertension

  The second theory posits that either increased blood flow to the brain or increase in the brain tissue itself may result in the raised pressure. Little evidence has accumulated to support the suggestion that increased blood flow plays a role, but recently Bateman et al. in phase contrast MRA studies have quantified cerebral blood flow (CBF) in vivo and suggests that CBF is abnormally elevated in many people with IIH. […] The third theory suggests that restricted venous drainage from the brain may be impaired resulting in congestion. Many people with IIH have narrowing of the transverse sinuses. It is not clear whether this narrowing is the pathogenesis of the disease or a secondary phenomenon. It has been proposed that a positive biofeedback loop may exist, where raised ICP (intracranial pressure) causes venous narrowing in the transverse sinuses, resulting in venous hypertension (raised venous pressure), decreased CSF resorption via arachnoid granulation and further rise in ICP.

• #70 Pseudotumor Cerebri: Categorical Disease or Spectrum of Disease | Walid | Journal of Neurology Research

  https://www.neurores.org/index.php/neurores/article/view/12/5

  The definition of idiopathic intracranial hypertension requires the absence of any focal lesion that may cause an increase in intracranial pressure or ventriculomegaly. […] However, in pseudotumor cerebri, the brain tissue itself is swollen due to intracellular or intercellular water retention. […] This increases the opening pressure of lumbar puncture by contiguity and manifests itself ophthalmologically with papilledema. […] Pseudotumor cerebri patients should be scrupulously interviewed and investigated to reveal any physiologic deviations or intracranial malformations that may explain weight gain, water retention and symptoms of increased intracranial pressure. […] If manageable metabolic or organic aberrances are discovered then the trigger of increased intracranial pressure should be treated before applying the diagnosis of Idiopathic Intracranial Hypertension. […] The diagnosis of pseudotumor cerebri can be difficult in atypical cases where some elements of the Dandy criteria are missing, for example, how to differentiate functional headaches with situational elevation of opening pressure from atypical pseudotumor cerebri without papilledema.

• #71 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #72 IIH (PSEUDOTUMOR CEREBRI) SYMPTOMS, CAUSES, AND TREATMENTS.

  https://virtualheadachespecialist.com/idiopathic-intracranial-hypertension-iih-pseudotumor-cerebri-causes-symptoms-and-treatments/

  Idiopathic intracranial hypertension (IIH) was previously called pseudotumor cerebri. […] The problem of IIH involves an elevation of CSF around the brain and spinal cord, without a clear reason. […] There have been a number of theories and findings as to the causes of IIH. These include: Overproduction of the CSF, Blockage of CSF absorption into the cerebral venous sinuses due to a faulty transport mechanism, Stenosis (narrowing) of the venous sinuses, Blot clot (thrombus) blocking the inside of venous sinuses preventing the outflow of CSF and pressure build up, Compression of the venous sinus from the outside (such as brain tumor, meningioma, etc.), causing narrowing on the inside, Sex hormones such as androgens and adipose tissue may play a potential role, Some medications have been associated with IIH including lithium, retinoids (such as excess vitamin A derivatives), oral contraceptives, and tetracycline antibiotics such as doxycycline and minocycline. Rebound IIH has also been reported from corticosteroid withdrawal. […] Dural arteriovenous (AV) fistula (an artery connects to the venous sinus, allowing arterial blood flow into the venous sinus and causes much higher pressure).

• #73 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #74 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #75 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #76 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #77 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #78 An integrated mechanism of pediatric pseudotumor cerebri syndrome: evidence of bioenergetic and hormonal regulation of cerebrospinal fluid dynamics | Pediatric Research

  https://www.nature.com/articles/pr2014188

  Multiple lines of evidence provide support for the hypothesis that the pathophysiology of PTCS involves aberrant glucocorticoid metabolism. […] The role of this system in the dynamics of CSF production and absorption is incompletely understood; however, we suggest that proportional upregulation of HSD1 is a compensatory measure to limit existing cortisol deficiencies. […] Aldosterone appears to augment the activities of epithelial sodium channels and Na+/K+ ATPase transporters to reduce CSF potassium concentrations while increasing CSF sodium concentrations. […] The first reports of recombinant growth hormone (rGH) or IGF-1 therapy causing PTCS were published in the early 1990s. […] Both hyper- and hypothyroidism are associated with abnormal renal fluid handling, effects mediated by reduced protein abundance of renal sodium-dependent transporters and water channels.

• #79 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #80 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #81 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #82 IIH (PSEUDOTUMOR CEREBRI) SYMPTOMS, CAUSES, AND TREATMENTS.

  https://virtualheadachespecialist.com/idiopathic-intracranial-hypertension-iih-pseudotumor-cerebri-causes-symptoms-and-treatments/

  Idiopathic intracranial hypertension (IIH) was previously called pseudotumor cerebri. […] The problem of IIH involves an elevation of CSF around the brain and spinal cord, without a clear reason. […] There have been a number of theories and findings as to the causes of IIH. These include: Overproduction of the CSF, Blockage of CSF absorption into the cerebral venous sinuses due to a faulty transport mechanism, Stenosis (narrowing) of the venous sinuses, Blot clot (thrombus) blocking the inside of venous sinuses preventing the outflow of CSF and pressure build up, Compression of the venous sinus from the outside (such as brain tumor, meningioma, etc.), causing narrowing on the inside, Sex hormones such as androgens and adipose tissue may play a potential role, Some medications have been associated with IIH including lithium, retinoids (such as excess vitamin A derivatives), oral contraceptives, and tetracycline antibiotics such as doxycycline and minocycline. Rebound IIH has also been reported from corticosteroid withdrawal. […] Dural arteriovenous (AV) fistula (an artery connects to the venous sinus, allowing arterial blood flow into the venous sinus and causes much higher pressure).

• #83 Idiopathic Intracranial Hypertension (IIH): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1214410-overview

  Although not accepted as a method of primary surgical treatment for IIH, stenting of stenotic dural sinuses has been demonstrated to decrease ICP in patients with IIH. […] Another pathway of CSF drainage that is under study, the glymphatics, may prove to have an important role in the pathophysiology of IIH. […] Several studies show that the CSF glymphatic function is congested in IIH, resulting in elevated intracranial pressure. […] IIH commonly occurs in women who are overweight; however, the role obesity plays in this disorder is unclear. […] However, obesity appears to have no association with IIH in the pediatric population. […] Although a role for vitamin A in the pathogenesis of IIH was initially suspected, the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated no difference in CSF vitamin A levels in patients with IIH versus obese controls over a 6-month period except in those treated with acetazolamide. […] Other medications that have been implicated for resulting in IIH include tetracycline, amiodarone, oral contraceptives such as levonorgestrel (Norplant), cyclosporine, cytarabine, growth hormone, isotretinoin, levothyroxine (children), lithium carbonate and acute change of steroid use.

• #84 IIH (PSEUDOTUMOR CEREBRI) SYMPTOMS, CAUSES, AND TREATMENTS.

  https://virtualheadachespecialist.com/idiopathic-intracranial-hypertension-iih-pseudotumor-cerebri-causes-symptoms-and-treatments/

  Idiopathic intracranial hypertension (IIH) was previously called pseudotumor cerebri. […] The problem of IIH involves an elevation of CSF around the brain and spinal cord, without a clear reason. […] There have been a number of theories and findings as to the causes of IIH. These include: Overproduction of the CSF, Blockage of CSF absorption into the cerebral venous sinuses due to a faulty transport mechanism, Stenosis (narrowing) of the venous sinuses, Blot clot (thrombus) blocking the inside of venous sinuses preventing the outflow of CSF and pressure build up, Compression of the venous sinus from the outside (such as brain tumor, meningioma, etc.), causing narrowing on the inside, Sex hormones such as androgens and adipose tissue may play a potential role, Some medications have been associated with IIH including lithium, retinoids (such as excess vitamin A derivatives), oral contraceptives, and tetracycline antibiotics such as doxycycline and minocycline. Rebound IIH has also been reported from corticosteroid withdrawal. […] Dural arteriovenous (AV) fistula (an artery connects to the venous sinus, allowing arterial blood flow into the venous sinus and causes much higher pressure).

• #85 European Headache Federation guideline on idiopathic intracranial hypertension | The Journal of Headache and Pain | Full Text

  https://thejournalofheadacheandpain.biomedcentral.com/articles/10.1186/s10194-018-0919-2

  The pathophysiological mechanism has traditionally been thought to be due to compression against the petrous ligament or the ridge of the petrous temporal bone, or stretching along the intracranial course of the nerve. […] There is a clear association between IIH and weight with over 90-95% of patients being obese. Additionally, IIH is reported in the context of gaining 5-15% of body weight. Weight loss is the only established disease modifying therapy in IIH. […] There is growing interest in the role of gut neuro peptides in IIH. The gut peptide glucagon-like peptide-1 (GLP-1) regulates insulin secretion and weight, and currently GLP-1 mimetic drugs are used extensively to treat diabetes (without risk of hypoglycaemia) and obesity. Recent in vitro assays have demonstrated that the GLP-1 receptor agonist exendin-4 reduces CSF secretion. Additionally, clinically relevant doses of exendin-4 dramatically reduced ICP in rodents with raised ICP.

• #86 Idiopathic intracranial hypertension: consensus guidelines on management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/89/10/1088

  Where the LP OP does not fit the clinical picture, it should be interpreted with caution. […] The main principles of management of IIH are: to treat the underlying disease, to protect the vision, and to minimise the headache morbidity. […] Weight loss is the only disease-modifying therapy in typical IIH. […] The current Cochrane review on IIH management reported on the use of acetazolamide, a carbonic anhydrase inhibitor, in IIH. […] Acetazolamide could be prescribed for those with IIH symptoms. […] The literature pertaining to shunt type is observational and mainly case series based. […] CSF diversion is generally not recommended as a treatment for headache alone in IIH. […] Neurovascular stenting is not currently a treatment for headache in IIH.

• #87 Managing idiopathic intracranial hypertension in the eye clinic | Eye

  https://www.nature.com/articles/s41433-024-03140-y

  The use of acetazolamide, a carbonic anhydrase inhibitor, is the most widespread treatment currently used to treat IIH to help with reduction of ICP and to protect the vision. […] The IIH weight trial (IIHWT) was a United Kingdom (UK) multicenter randomised controlled trial that evaluated the effect of bariatric surgery and a community weight management intervention on intracranial pressure in women with active IIH and a body mass index of 35kg/m2 or greater. […] The decision to proceed to surgical management in medically refractory cases should be made on a case by case basis. […] The surgical management of IIH includes optic nerve sheath fenestration (ONSF), CSF diversion procedures and venous stenting. […] The newest option for treatment is neurovascular stenting.

• #88 Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment – UpToDate

  https://www.uptodate.com/contents/idiopathic-intracranial-hypertension-pseudotumor-cerebri-prognosis-and-treatment

  Venous sinus stenting is a relatively new and somewhat controversial treatment option for IIH. Its use results from the observation that many patients with IIH have apparent stenoses of the transverse venous sinus or other cerebral veins, although whether this is a primary or secondary phenomenon is uncertain.

• #89 The Genetics of Idiopathic Intracranial Hypertension (IIH): Integration of Population Studies and Clinical Data | medRxiv

  https://www.medrxiv.org/content/10.1101/2023.06.03.23290934v1.full-text

  Idiopathic intracranial hypertension (IIH) is a condition characterized by increased intracranial pressure without a known cause. It mainly affects overweight, reproductive-age women, and its genetic basis remains unknown. […] The pathogenic mechanisms of IIH are uncertain. Dysregulation of ICP is an important direction of investigation. Three chief intracranial mechanisms have been proposed to cause raised ICP, including disorderly cerebrospinal fluid (CSF) dynamics such as CSF hypersecretion and outflow obstruction, and increased venous sinus pressure. […] The mechanism by which weight loss improves IIH is uncertain. […] New metabolic therapeutic targets are emerging for IIH, the most hopeful being glucagon-like peptide 1 (GLP-1) receptor agonism. […] The choroid plexus epithelium is critical for the production, composition, and absorption of the CSF.

• #90 European Headache Federation guideline on idiopathic intracranial hypertension | The Journal of Headache and Pain | Full Text

  https://thejournalofheadacheandpain.biomedcentral.com/articles/10.1186/s10194-018-0919-2

  The pathophysiological mechanism has traditionally been thought to be due to compression against the petrous ligament or the ridge of the petrous temporal bone, or stretching along the intracranial course of the nerve. […] There is a clear association between IIH and weight with over 90-95% of patients being obese. Additionally, IIH is reported in the context of gaining 5-15% of body weight. Weight loss is the only established disease modifying therapy in IIH. […] There is growing interest in the role of gut neuro peptides in IIH. The gut peptide glucagon-like peptide-1 (GLP-1) regulates insulin secretion and weight, and currently GLP-1 mimetic drugs are used extensively to treat diabetes (without risk of hypoglycaemia) and obesity. Recent in vitro assays have demonstrated that the GLP-1 receptor agonist exendin-4 reduces CSF secretion. Additionally, clinically relevant doses of exendin-4 dramatically reduced ICP in rodents with raised ICP.

• #91 European Headache Federation guideline on idiopathic intracranial hypertension | The Journal of Headache and Pain | Full Text

  https://thejournalofheadacheandpain.biomedcentral.com/articles/10.1186/s10194-018-0919-2

  The pathophysiological mechanism has traditionally been thought to be due to compression against the petrous ligament or the ridge of the petrous temporal bone, or stretching along the intracranial course of the nerve. […] There is a clear association between IIH and weight with over 90-95% of patients being obese. Additionally, IIH is reported in the context of gaining 5-15% of body weight. Weight loss is the only established disease modifying therapy in IIH. […] There is growing interest in the role of gut neuro peptides in IIH. The gut peptide glucagon-like peptide-1 (GLP-1) regulates insulin secretion and weight, and currently GLP-1 mimetic drugs are used extensively to treat diabetes (without risk of hypoglycaemia) and obesity. Recent in vitro assays have demonstrated that the GLP-1 receptor agonist exendin-4 reduces CSF secretion. Additionally, clinically relevant doses of exendin-4 dramatically reduced ICP in rodents with raised ICP.

• #92 Update on the pathophysiology and management of idiopathic intracranial hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3544160/

  Idiopathic Intracranial Hypertension (IIH) is a disease of unknown etiology typically affecting young, obese women, producing a syndrome of increased intracranial pressure without identifiable cause. […] Despite a large number of hypotheses and publications over the past decade, the etiology is still unknown. Vitamin A metabolism, adipose tissue as an actively secreting endocrine tissue, and cerebral venous abnormalities are areas of active study regarding IIH’s pathophysiology. […] There are multiple theories regarding the pathophysiology of this disorder, but there is no unifying hypothesis. […] The pathophysiologic mechanisms underlying the raised ICP in IIH remain unclear, but those proposed classically include increased brain water content, excess cerebrospinal fluid (CSF) production, reduced CSF absorption, and increased cerebral venous pressure.

• #93 Evolving evidence in adult idiopathic intracranial hypertension: pathophysiology and management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/87/9/982

  Idiopathic intracranial hypertension (IIH) is a rare but important disease associated with significant morbidity. […] The pathophysiology of IIH is incompletely characterised; suggested underpinning mechanisms include the role of cerebrospinal fluid regulation as well as metabolic and endocrinological perspectives. […] The underlying pathogenesis of IIH is uncertain. Raised ICP is a uniform characteristic, but the mechanism by which ICP is elevated in IIH is not clear. […] Changes in the volume of blood, CSF and brain tissue influence ICP. IIH likely represents a disorder of CSF regulation, potentially through CSF hypersecretion or reduced drainage. […] The choroid plexus is the primary site of CSF secretion, generating around two-thirds of the total CSF produced, with the rest coming from extrachoroidal sources, such as the ependyma and possibly the blood brain barrier.

• #94 Pseudotumor Cerebri – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK536924/

  Hormonal changes seen include Aldosterone excess (associated with obesity and PCOS), which commonly correlates with PTC and is suggested to affect the mineralocorticoid receptor of the choroid plexus, leading to increased CSF production. […] Cellular changes seen include increased outflow resistance to CSF, which has been demonstrated in multiple experimental studies and is the leading theory for the causation of PTC.

• #95 Idiopathic Intracranial Hypertension, Pseudotumor cerebri – EyeRounds.org – Ophthalmology – The University of Iowa

  http://eyerounds.org/cases/99-Pseudotumor-Cerebri.htm

  Idiopathic Intracranial Hypertension (IIH) is a condition in which the cerebrospinal fluid (CSF) is under high pressure in the absence of an intracranial mass, venous sinus thrombosis or other primary cause. It is also known as pseudotumor cerebri because patients present with signs and symptoms of a brain tumor without a brain tumor being present. […] In IIH, there is no structural obstruction to the circulation of CSF. It is believed that the problem lies in defective reabsorption of CSF either at the level of the arachnoid granulations or along cranial and spinal nerve root sheaths. There is no evidence for excessive CSF production. […] There is a female preponderance for this disease, and the peak age of onset is in the third decade of life. It is common for affected patients to be obese, and weight loss is an important part of treatment. IIH symptoms often start or worsen during a period of weight gain and resolve with 5-10% total weight loss.

• #96 Update on the pathophysiology and management of idiopathic intracranial hypertension

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3544160/

  Numerous recent publications have shown that such transverse sinus stenoses (TSS) are found in a large majority of patients with IIH. […] Another venous mechanism proposed in the development of increased ICP in IIH patients is the occurrence of microthrombosis from thrombophilia within the cerebral veins, resulting in impaired CSF absorption.

• #97 Idiopathic intracranial hypertension | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/idiopathic-intracranial-hypertension-1?lang=us

  Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a syndrome with signs and symptoms of increased intracranial pressure but where a causative mass or hydrocephalus is not identified. […] The pathogenesis is poorly understood. Various mechanisms have been proposed, including decreased CSF absorption, increased CSF production, increased intravascular volume, increased intracranial venous pressure, hormonal changes, altered aquaporin-4 channels and abnormality in function of the glymphatic system. […] Venous sinus stenosis is increasingly recognized as an important factor although whether it is the primary inciting abnormality or a potentiating factor remains to be fully established. […] A study also found an association between decreased glymphatic clearance and papilledema in patients with IIH. Whether this is a primary driving cause or also the sequela of impaired venous outflow remains to be determined.

• #98 Idiopathic intracranial hypertension, hormones, and 11B-hydroxys | JPR

  https://www.dovepress.com/idiopathic-intracranial-hypertension-hormones-and-11szlig-hydroxystero-peer-reviewed-fulltext-article-JPR

  Idiopathic intracranial hypertension (IIH) results in raised intracranial pressure (ICP) leading to papilledema, visual dysfunction, and headaches. […] The underlying pathological mechanisms behind IIH remain unknown. […] Despite occurring almost exclusively in obese women, there have been a few studies evaluating the mechanisms by which hormones and adipokines exert their effects on ICP regulation in IIH. […] Improved understanding of the complex interplay between adipose signaling factors such as adipokines, steroid hormones, and ICP regulation may be key to the understanding and future management of IIH. […] The etiology of this debilitating condition remains undetermined. Dysregulation of CSF dynamics is key and may involve hypersecretion of CSF at the choroid plexus, reduced drainage at the arachnoid granulations and/or lymphatics, and aberrant venous pressure gradients.

• #99 Idiopathic intracranial hypertension: from concise history to current management | The Egyptian Journal of Neurology, Psychiatry and Neurosurgery | Full Text

  https://ejnpn.springeropen.com/articles/10.1186/s41983-023-00730-7

  Idiopathic intracranial hypertension (IIH), known as benign intracranial hypertension (BIH) since the report of Quincke in 1893, was described as a rare disorder of elevated intracranial hypertension with normal cerebrospinal fluid (CSF) and without any pathology. […] The pathophysiology remains unknown; however, some theories relate to its mechanism, including obesity and metabolic dysregulation. […] The underlying mechanism of IIH has been unknown; however, the significant risk factor identified is weight gain-induced signs and symptoms of IIH. […] Some proposed hypotheses of IIH include overproduction of CSF, outflow obstruction, increased central venous pressure, metabolism, and hormonal changes commonly found in obese patients. […] Central obesity may elevate intraabdominal, pleura, cardiac filling, and central venous pressure impairing CSF flows.

• #100 Managing idiopathic intracranial hypertension in the eye clinic | Eye

  https://www.nature.com/articles/s41433-024-03140-y

  Idiopathic intracranial hypertension (IIH) is a neuro-ophthalmological condition characterised by a raised intracranial pressure and papilloedema that causes disabling headaches. […] The knowledge of the underlying pathophysiology is evolving. […] IIH is emerging as a systemic metabolic disease distinct from people living with obesity alone. […] The incidence of IIH is increasing worldwide, and as many ophthalmologists now diagnose and manage IIH, high quality research and practice guidelines are required to provide excellence and equity of care. […] Despite clear diagnostic criteria which are widely accepted, there is evidence of diagnostic difficulty for some in confirming a diagnosis of IIH. […] Papilloedema indicates a possible serious underlying condition that requires immediate work up.

• #101 The Genetics of Idiopathic Intracranial Hypertension (IIH): Integration of Population Studies and Clinical Data | medRxiv

  https://www.medrxiv.org/content/10.1101/2023.06.03.23290934v1.full-text

  Enrichment of the GWAS results and with choroid plexus argue for a role of cilia function in IIH and PAP. […] Our study revealed no single etiology for IIH/PAP. Instead, we propose a curated and ranked list of variants and genes that were revealed for IHH/PAP as candidates for further investigation. […] The only statistically confident IIH-associated variant that was identified by FinnGen Fz9 is rs545417105 within the intron of LRRFIP1.

• #102 European Headache Federation guideline on idiopathic intracranial hypertension | The Journal of Headache and Pain | Full Text

  https://thejournalofheadacheandpain.biomedcentral.com/articles/10.1186/s10194-018-0919-2

  Idiopathic Intracranial Hypertension (IIH) is characterized by an elevation of intracranial pressure (ICP) with no identifiable cause. Despite the fact that its aetiology remains largely unknown, the observations made in a significant number of recent clinical studies and the resulting increase in the understanding of its clinical picture have led to modifications in its diagnostic classification and provide the basis for evidence-based treatment strategies. […] The term pseudotumor cerebri, in the past commonly used as a synonym for IIH, is now used as an umbrella term that describes the chronic elevation of ICP regardless of its aetiology and further subdivides in the primary (IIH) and secondary forms. […] IIH can cause other symptoms, as well as headache and visual disturbances. Recent data however suggests that other systems may also be affected in IIH, presumably as the result of increased ICP, although the exact mechanisms leading to these alterations remain largely unknown.

Zobacz więcej