Pathogenesis Of Idiopathic Intracranial Hypertension Research Articles

What's New in Childhood Idiopathic Intracranial Hypertension?

Idiopathic Intracranial Hypertension (IIH) occurs quite rarely in the paediatric population and its clinical features differ from adults in many ways. Childhood IIH has no sex predilection, and obesity does not appear to be an important factor, especially in prepubertal patients. Newer associations of this disease have been reported that were never reported in the past, mainly due to better understanding of the disease process, changing clinical practice, and technological advancement. Understanding of childhood IIH is very limited as most of the studies regarding the pathogenesis of IIH relate to adults. Prompt diagnosis and treatment is important as children can sustain loss of visual field and acuity despite apparently adequate treatment. Children with IIH should be managed by a multi–disciplinary team consisting of a general paediatrician, a neuro–ophthalmologist, a neurologist, a neurosurgeon, and when appropriate a paediatric neuro–intensivist. The goal of treatment is to relieve symptoms and to preserve vision by normalizing intracranial pressure. Children with IIH should be kept under close surveillance and evaluated for visual acuity, visual fields, colour vision, pupillary function and optic disc oedema.

Idiopathic intracranial hypertension (pseudotumor cerebri)

To review the current standard of care in the diagnosis and treatment of idiopathic intracranial hypertension (IIH). We also discuss recent advances in the understanding of the pathogenesis of IIH with emerging trends in management of this syndrome. There has been increasing literature suggesting a role of vitamin A, adipokines, sleep disorders, and venous sinus stenosis in the pathogenesis of IIH. Newer medical treatment options like topiramate and surgical treatment options like stereotactic ventriculoperitoneal shunting have been found to be attractive alternatives. There has also been an emerging interest in cerebral venous sinus stenting, though its role and utility remain debatable. This article seeks to present a systematic approach to the management of IIH. Many newer treatment modalities are being explored for IIH refractory to standard medical therapy, but their efficacy and safety must be demonstrated in large studies before they can be adopted as part of standard treatment.

Exploring the pathogenesis of IIH: An inflammatory perspective

Idiopathic intracranial hypertension (IIH) is a common blinding condition amongst the young obese female population (20 per 100,000) characterised by elevated intracranial pressure (ICP). The aetiology of IIH is not known. In this review we explore the literature investigating the pathogenesis of IIH and suggest additional hypotheses. Chronic inflammation is emerging as an aetiological factor in the pathogenesis of obesity and we propose that this may be a feature of IIH. Obesity is also related to dysregulation of cortisol production by the pre-receptor enzyme, 11β-hydroxysteroid dehydrogenase, and we speculate that this may have a role in the pathogenesis of obesity and raised ICP seen in IIH.

Obesity may not be a risk factor for idiopathic intracranial hypertension in Asians

Idiopathic intracranial hypertension (IIH) is a clinical condition characterized by signs and symptoms of increased intracranial pressure. We demonstrated the clinical presentation of IIH of Asians and investigated any ethnic difference in pathogenesis. Ten patients who fulfilled the modified Dandy criteria and four with presumed diagnosis for IIH were included. Only one patient (7.1%) was obese according to body mass index, seven were overweight, and six weighed normal. Obesity was not frequently found in Caucasians with IIH. This data indirectly suggest that obesity may not play a major role in the pathogenesis of IIH in Asians. Awareness of this is helpful in early diagnosis and treatment of IIH in Asians.

Idiopathic intracranial hypertension: Can studies of obesity provide the key to understanding pathogenesis?

The pathogenesis of idiopathic intracranial hypertension (IIH) is poorly understood. Several mechanisms have been suggested, but no one mechanism has been able to account for all manifestations of the disease. Although IIH predominantly affects obese, premenopausal women, little is known about whether or how the obesity contributes to the IIH. Obesity is a heterogeneous condition, consisting of different phenotypes that are influenced by the regional distribution of adipose tissue. This review explores the literature to integrate current knowledge on the relationships between obesity and IIH. The review evaluates the hypotheses that dysregulation of insulin, glucose metabolism, sex hormones, adipokines, glucocorticoids, lipids and free fatty acids in obesity could predispose to IIH. One potential common pathway linking metabolic disorders to the pathogenesis of IHH is a thrombotic tendency due to dysregulation of haemostatic risk factors. This could cause either occult cerebral sinus thrombosis or partial thrombosis of the parasagittal venous lacunae, with subsequent impaired resorption of cerebrospinal fluid and venous hypertension. Investigations that evaluate obesity, fat metabolism, endocrinological dysregulation and thrombotic tendency in patients with IIH are required so that pathogenic mechanisms can be clarified and management strategies in IIH can be improved.

Retinol-Binding Protein and Retinol Analysis in Cerebrospinal Fluid and Serum of Patients With and Without Idiopathic Intracranial Hypertension

Several studies have implicated vitamin A-related compounds in the pathogenesis of idiopathic intracranial hypertension (IIH). The goal of this study was to compare cerebrospinal fluid (CSF) and serum concentrations of retinol and retinol-binding protein (RBP) in subjects with and without IIH. CSF and serum samples were collected from 87 subjects. The study population was composed of subjects with IIH (IIH group, n = 28), subjects with non-IIH neurologic conditions (neurology controls, n = 42), and subjects undergoing preoperative lumbar puncture but with no known neurologic conditions (anesthesia controls, n = 17). RBP levels (nM) were determined using radial immunodiffusion, and retinol levels (nM) were determined using high-performance liquid chromatography. The retinol/RBP ratio was greater in CSF than in serum, especially in subjects with IIH. The finding of increased levels of unbound retinol in the CSF of subjects with IIH provides further evidence that vitamin A may be involved in the pathogenesis of IIH. Comparative statistical analyses revealed multivariate relationships that demonstrate the need to further investigate correlations between vitamin A and RBP levels in CSF and serum.

408: Persistent venous stenosis and pressure gradient immediately post-lumbar puncture in idiopathic intracranial hypertension (IIH)

The pathogenesis of IIH remains unclear. Note has been made of the high incidence of venous stenoses on angiographic and magnetic resonance studies in patients. Some authors have suggested that these stenoses are pathogenic, whereas others have suggested that the stenoses are a result, rather than the cause of IIH.

Vitamin A in the cerebrospinal fluid of patients with and without idiopathic intracranial hypertension.

We quantified vitamin A in the cerebrospinal fluid of patients with idiopathic intracranial hypertension, elevated intracranial pressure of other causes and normal intracranial pressure. Vitamin A could be detected by high-pressure liquid chromatography in most of the specimens. There was a significantly higher level of vitamin A in the cerebrospinal fluid of some patients with idiopathic intracranial hypertension. Vitamin A toxicity may play a role in the pathogenesis of idiopathic intracranial hypertension.

Retinol-Binding Protein in Idiopathic Intracranial Hypertension (IIH)

We postulated that an alteration in endogenous vitamin A (retinol) metabolism plays a causal role in the pathogenesis of idiopathic intracranial hypertension (IIH). Serum retinol was determined by a fluorometric method from 40 control subjects and 58 patients with idiopathic intracranial hypertension (IIH). Retinol binding protein (RBP) was also assayed by quantitative radial immunodiffusion in 17 control subjects and 30 patients with IIH. Mean retinol values were higher in the IIH group compared with the control group, but did not reach a significant level. However, seven of 30 patients with IIH had high RBP levels, but none of the control subjects did. This data suggests that IIH is associated with an abnormality in vitamin A metabolism that is linked to its transport system.