\u201cBenign intracranial hypertension\u201d is a misleading name for a more serious disease: analysis of the visual outcome in cases with idiopathic intracranial hypertension

Ali R Hamdan,Eslam El Sayed El Khateeb,Osama Elsoghiar,Amr M Tayel

doi:10.1186/s41984-019-0064-5

Ali R Hamdan, Eslam El Sayed El Khateeb + Show 2 more

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https://doi.org/10.1186/s41984-019-0064-5

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Abstract

ObjectiveAssessment of visual field outcomes following the medical or surgical management of increased intracranial pressure in patients with idiopathic intracranial hypertension.Patient and methodsThirty-two patients diagnosed with idiopathic intracranial hypertension who met all the modified Dandy criteria were included in this study. Magnetic resonance brain imaging with contrast and magnetic resonance venography were performed to exclude the presence of secondary causes of intracranial hypertension. Following normal neuroimaging, a lumbar puncture was performed for all patients with papilledema to measure the cerebrospinal fluid opening pressure. The assessment of visual function, including visual acuity, fundus examination (dilated fundus examination to confirm the presence and the grade of papilledema and to exclude the other ocular causes of optic disc swelling), and formal visual field assessment by perimetry when visual acuity was more than 1/60 were performed by the ophthalmologist before and after medical or surgical treatment.ResultsThirty-two patients were included, the majority of whom were females of childbearing age (29/32; 90.6%). All patients complained of headache. Visual assessment revealed the presence of blurry vision in all patients. Papilledema with different grades was observed in 31/32 (96.9%) patients, while the other patient (3.1%) showed optic atrophy; 30/32 (93.8%) patients had visual acuity ˃ 1/60. The average visual field was − 18 dB ± 9.97 SD in the right eye and − 19.47 dB ± 5.43 SD in the left eye. Twenty-nine patients received medical treatment in the form of acetazolamide, which succeeded in controlling idiopathic intracranial hypertension in 24/29 (82.7%) patients, while a theco-peritoneal shunt was inserted in 8/32 (25%) patients. After management, statistically significant improvements in cerebrospinal fluid opening pressure, presenting manifestation, visual acuity, and field were noticed.ConclusionsPermanent visual loss is the most feared complication of idiopathic intracranial hypertension; therefore, early diagnosis and multidisciplinary treatment are necessary to improve visual acuity and visual field.

Highlights

Idiopathic intracranial hypertension is a neurological disease that is characterized by elevated intracranial pressure in the absence of a recognizable cause [1, 2]
Because of the significant visual morbidity associated with pseudotumor cerebri (PTC), the use of the adjective “benign” is no longer considered appropriate terminology; instead, physicians prefer to use the term idiopathic intracranial hypertension (IIH) for such cases
The mean body mass index (BMI) was 33.2 ± 3.912 SD kg/m2, and the mean Cerebrospinal fluid (CSF) opening pressure obtained by LP at the time of diagnosis was 38.2 ± 14.141 SD cm H2O

Summary

Introduction

Idiopathic intracranial hypertension is a neurological disease that is characterized by elevated intracranial pressure in the absence of a recognizable cause [1, 2]. The condition was called pseudotumor cerebri (PTC) in 1914. Foley suggested calling it “benign intracranial hypertension” because it appeared to have a much more benign neurological prognosis than other mass lesions or infections. Because of the significant visual morbidity associated with PTC, the use of the adjective “benign” is no longer considered appropriate terminology; instead, physicians prefer to use the term idiopathic intracranial hypertension (IIH) for such cases. The most common presentation of patients with idiopathic intracranial hypertension is headache, visual changes, and papilledema, which, if left untreated, may lead to permanent loss of vision [3]. The poor visual outcome is usually associated with early onset of reduced vision, optic disc hemorrhages, grade 4 papilledema, and a high body mass index [4]. Magnetic resonance imaging (MRI) of the brain with intravenous contrast and magnetic resonance venography (MRV) are considered the modalities of choice to exclude an intracranial mass or venous sinus occlusion

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