Long term outcomes of idiopathic intracranial hypertension: Observational study and literature review
Introduction
Idiopathic intracranial hypertension (IIH) is a debilitating condition that predominantly affects obese young women of child-bearing age [1,2]. The pathophysiology of IIH remains poorly characterized [3]. The most common clinical symptoms of IIH include headaches, transient visual obscurations (TVOs), pulsatile tinnitus, and diplopia [3]. Medical treatment options include acetazolamide and topiramate [3]. Weight loss in this condition is beneficial [[4], [5], [6]]. In medically refractory cases, surgical interventions such as optic nerve sheath fenestration (ONSF), cerebrospinal fluid (CSF) diversion procedures, or venous sinus stenting can be considered [7,8].
A subset of IIH cases can be reoccurring and severe, requiring ongoing treatment [9,10]. It follows that an understanding of the long-term outcomes of IIH is crucial to minimise potential but serious visual loss. The aim of this study was to assess the long-term visual and symptomatic outcomes of patients diagnosed with IIH.
Section snippets
Methods
This study was a retrospective observational chart review of all patients diagnosed with IIH at Auckland District Health Board (ADHB), Department of Ophthalmology from June 2005 to March 2017. Patients who met the revised modified Dandy criteria for IIH were included [11]. This included patients with signs and symptoms of raised intracranial pressure (ICP) such as papilledema, neuroimaging (computerised tomography with venography (CTV) or magnetic resonance imaging with venography (MRV))
Results
Data were analysed from 132 patients who met the inclusion criteria. Patient baseline characteristics and follow-up information is reported in Table 1. Patients were young (28.1 ± 9.7 years), and mostly females (90.9 %). Body mass index (BMI) data were available for 124 out of 132 patients (93.9 %) (Table 1). Mean BMI was 35.9 ± 7.9 kg/m2. Only 9 patients (6.8 %) (2/9 were men) had a normal BMI of 20–24.9 kg/m2 based on World Health Organisation obesity grades [14]. CSF opening pressure at
Discussion
This study is one of the largest studies (>100 patients) of IIH patients with follow-up longer than one year. Our key findings are that treatment of IIH results in significant resolution of symptoms: headache reduced by nearly half (87.6 % vs 48.8 %), TVO decreased from 27.2 % vs 7.4 %, and diplopia from 16.5 % vs 0.8 %. However, medical treatment is associated significant adverse events and morbidity. One-third (34.2 %) of patients started on acetazolamide discontinued the medication due to
CRediT author statement
Helen Danesh-Meyer: Conceptualization, Methodology. William Xu: Data curation, Analysis Zak Prime: Data curation, Analysis. Taras Papchenko: Conception, Analysis. All authors were involved in the writing and editing process of this manuscript.
Funding statement
This project was supported by a University of Auckland Faculty of Medical and Health Sciences Scholarship
Ethics committee approval: NZ Health and Disability Ethics Committee.
Declaration of Competing Interest
On behalf of all authors, the corresponding author states that there is no conflict of interest.
References (39)
- et al.
Re-evaluating the incidence of idiopathic intracranial hypertension in an era of increasing obesity
Ophthalmology
(2017) - et al.
Understanding idiopathic intracranial hypertension: Mechanisms, management, and future directions
Lancet Neurol.
(2016) - et al.
The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri)
Ophthalmology
(1998) - et al.
Visual outcomes and headache following interventions for idiopathic intracranial hypertension
J. Clin. Neurosci.
(2014) - et al.
Long-term follow-up of pseudotumor cerebri syndrome in Prepubertal Children, adolescents, and adults
Pediatr. Neurol.
(2019) - et al.
Idiopathic intracranial hypertension: Are there predictors for visual outcome or recurrences?
Clin. Neurol. Neurosurg.
(2019) - et al.
The idiopathic intracranial hypertension treatment trial clinical profile at baseline
JAMA Neurol.
(2014) - et al.
Weight reduction for a better visual outcome in idiopathic intracranial hypertension
Arq. Bras. Oftalmol. [Internet]
(2018) - et al.
Effects of weight loss on the course of idiopathic intracranial hypertension in women
Neurology
(1998) - et al.
Meta-analysis of csf diversion procedures and dural venous sinus stenting in the setting of medically refractory idiopathic intracranial hypertension
Am. J. Neuroradiol.
(2015)
Weight gain and recurrence in idiopathic intracranial hypertension: a case-control study
Neurology
Idiopathic intracranial hypertension: risk of recurrences
Neurology
Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children
Neurology
R: a Language and Environment for Statistical Computing [Internet]
Proper method for calculating average visual acuity
J. Refract. Surg.
BMI Classification [Internet]
Safety and tolerability of acetazolamide in the idiopathic intracranial hypertension treatment trial
J. Neuro-Ophthalmol.
Clinical course and prognosis of pseudotumor cerebri. A prospective study of 24 patients
Acta Neurol. Scand. [Internet]
A randomised controlled trial of treatment for idiopathic intracranial hypertension
J. Neurol.
Cited by (3)
Presumptive Idiopathic Intracranial Hypertension Based on Neuroimaging Findings: A Referral Pattern Study
2023, Journal of Neuro-Ophthalmology