Idiopathic Intracranial Hypertension: Prognostic Factors and Multidisciplinary Management

Abstract

Background: Idiopathic intracranial hypertension (IIH) mostly affects young obese women and can lead to permanent visual impairment. However, prognostic factors and therapeutic strategy remain unclear.

Methods: We retrospectively collected data from all patients diagnosed and managed for IIH in our university center from January 2001 to December 2016.

Results: Seventy-nine patients were diagnosed with IIH. Bilateral transverse sinus stenosis (TSS) was found in 74% of the population. Visual outcome at 6 months was poor for 46% of patients, including all patients presenting weight gain of at least 5% since diagnosis (p < 0.001), whereas mean body mass index at diagnosis was not different between patients with poor versus good outcome (32.9 ± 7.7 versus 34.6 ± 9.4 kg·m^-2). Other significant factors of poor prognosis were bilateral TSS (OR = 5.2; 95 CI: 1.24-24.9; p = 0.024). Thirteen patients with poor outcome after 6-month assessment underwent unilateral TSS stenting leading to visual improvement in 11 cases.

Conclusion: Weight gain, rather than initial weight, emerged as the leading factor of poor visual outcome in patients with IIH, followed by presence of bilateral TSS. Consequently, first-line treatment must include dietary measures to control weight. Unilateral stenting appears to be a safe second-line treatment option for patients with bilateral TSS.

Figure 1

Signs of idiopathic intracranial hypertension on cerebral MRI, T2-weighted images. 1: empty sella, 2: flattening of the posterior aspect of the globe, 3: tortuous optic nerve, and 4: distention of the perioptic subarachnoid space.

Figure 2

Bilateral transverse sinus stenosis on cerebral MR angiography; time-resolved imaging of contrast kinetic. Arrows: transverse sinus stenosis.

Figure 3

Stenting of right transverse sinus stenosis in a patient with bilateral stenosis, conventional angiography. (a) Right transverse sinus stenosis, (b) condition after treatment, and (c, d) stent placement.

Figure 4

Proposed mechanisms for idiopathic intracranial hypertension pathogenesis. AQP1: aquaporin 1, CSF: cerebrospinal fluid, and TSS: transverse sinus stenosis.

Figure 5

Algorithm for management of patients with idiopathic intracranial hypertension. CSF: cerebrospinal fluid, MRA: magnetic resonance angiography, and TSS: transverse sinus stenosis.