An update on the pathophysiology of idiopathic intracranial hypertension alias pseudotumor cerebri

Abstract

İdiyopatik intrakraniyal hipertansiyonun -diğer adıyla psödotümör serebrininpatofizyolojisi üzerine bir güncelleme SummaryIdiopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure of unknown cause, leading to severe headache, papilledema and visual disturbances. Its former name, pseudotumor cerebri, has gained popularity recently. The strongest and most consistent risk factors of IIH are obesity and female gender. Infrequently, IIH may present in the absence of papilledema showing a headache profile similar to chronic daily headache with migrainous features. There have been several proposed mechanisms to explain the etiology of this disorder associated with various clinical conditions. In recent years, some inflammatory factors, natriuretic peptides and aquaporins have been proposed as possible contributors of the pathogenesis. On the other hand, some investigators have reported that bilateral transverse sinus stenosis is seen in the majority of IIH patients; therefore, dural sinus stent placement is used in some patients. No single theory has been able to provide a comprehensive answer, and there is no consensus about the exact cause of IIH. The aim of this review was to discuss the new insights on the mysterious pathogenesis of IIH.

Transverse Sinus Stenosis Is the Most Sensitive MR Imaging Correlate of Idiopathic Intracranial Hypertension

“…Idiopathic intracranial hypertension is a poorly understood condition, the hallmark of which is elevation of CSF opening pressure without an identifiable intracranial mass or ventriculomegaly. 1,[15][16][17] It shares many similarities with a condition of visual impairment and intracranial pressure observed in astronauts after prolonged exposure to conditions of microgravity. [18][19][20] It may also share common pathophysiologic pathways with acute mountain sickness, a condition in which recent MR imaging studies have focused on alterations of arterial inflow, cerebral white matter diffusivity, and venous outflow restriction.…”

“…[18][19][20] It may also share common pathophysiologic pathways with acute mountain sickness, a condition in which recent MR imaging studies have focused on alterations of arterial inflow, cerebral white matter diffusivity, and venous outflow restriction. 21 Explanations for the mechanisms underlying IIH have focused on overproduction of CSF; impaired resorption of CSF 22 ; dysautoregulation of cerebral blood flow 23 ; dysregulation of fluid homeostasis, among others, in turn mediated by underlying endocrinopathies; fluid and electrolyte shifts with subtle white matter edema 24 ; dysregulation of aquaporin 1 and aquaporin 4 receptors 16,25,26 ; or proinflammatory states 27,28 due to mitochondrial dysregulation or circulating signaling leptins. [29][30][31][32] A preponderance of evidence suggests a high incidence of IIH in women of childbearing age with an elevated body mass index, supporting hypotheses that metabolic or hormonal factors may play a strong role.…”

Osmolality of Cerebrospinal Fluid from Patients with Idiopathic Intracranial Hypertension (IIH)

“…Proposed mechanisms behind IIH include brain parenchymal edema, increased cerebral blood volume, excessive CSF production, venous outflow obstruction and compromised CSF resorption. In recent years, more details about epidemiology and phenotypical presentation have been published along with a proposal of possible contribution of inflammatory factors [ 13 – 21 ]. For a review on analyses of CSF composition in patients with IIH, see Baykan [ 13 ].…”

“…In recent years, more details about epidemiology and phenotypical presentation have been published along with a proposal of possible contribution of inflammatory factors [ 13 – 21 ]. For a review on analyses of CSF composition in patients with IIH, see Baykan [ 13 ]. In this study we investigated the possibility of excess CSF production.…”

What is the impact of having a family history of migraine on migraine characteristics?

“…Sekonder etyolojiye sahip olan başağrıları anamnez, klinik muayene ve görüntüleme yöntemleri yardımıyla dışlandı. [14] Hastalık başlangıç yaşı, baş ağrısının süresi ve sıklığı, aura varlığı ve tipi, baş ağrısının klinik özellikleri ve lokalizasyonu, ağrının şiddeti, tetikleyici faktörler, komorbid hastalıklar, aile öyküsü varlığı, VAS ve ASC skorları, allodini varlığı, MIDAS skorları gibi klinik ve demografik bilgiler her hasta için baş ağrısı uzmanı tarafından yapılan yüz yüze görüşmelerde standart bir anket formu uygulanarak kaydedildi.…”