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Year : 2010  |  Volume : 58  |  Issue : 5  |  Page : 818-819

Idiopathic intracranial hypertension presenting as unilateral papilledema

Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India

Date of Acceptance22-Jul-2010
Date of Web Publication28-Oct-2010

Correspondence Address:
Ashalatha Radhakrishnan
Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.72208

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How to cite this article:
Wattamwar PR, Baheti NN, Radhakrishnan A. Idiopathic intracranial hypertension presenting as unilateral papilledema. Neurol India 2010;58:818-9

How to cite this URL:
Wattamwar PR, Baheti NN, Radhakrishnan A. Idiopathic intracranial hypertension presenting as unilateral papilledema. Neurol India [serial online] 2010 [cited 2022 Aug 15];58:818-9. Available from: https://www.neurologyindia.com/text.asp?2010/58/5/818/72208


Idiopathic intracranial hypertension (IIH) is a common cause of bilateral papilledema without any other focal deficits. Although classically bilateral, unilateral and highly asymmetrical papilledema is rarely described in IIH. [1] This atypical presentation can pose a diagnostic challenge if the treating physician is unaware of such association.

A 32-year-old obese woman presented with a 4-month history of transient visual obscurations in the right eye without headache or raised intracranial pressure symptoms. Her past medical history was unremarkable. Examination showed normal visual acuity in both eyes. Optic fundi showed absent venous pulsations with papilledema in the right eye [Figure 1]a; left optic fundus was normal. The remainder of the neurological examination was unremarkable. Magnetic resonance imaging [Figure 2] showed empty sella, buckling of optic nerves and prominent perioptic cerebrospinal fluid (CSF) spaces bilaterally. Orbital and retro-orbital structures were normal, and no other structural lesion or ventriculomegaly was noted. MR venogram was normal.
Figure 1 :(a) Right eye fundus photographs showing papilledema at presentation; (b) resolving papilledema at 3 months

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Figure 2 :Brain MRI (1.5 Tesla). (a) Sagittal T2-weighted image showing empty sella (white arrow); (b) axial T2-weighted image showing buckling of optic nerves with normal retro-orbital structures; (c) coronal T2-fat saturated image showing increased CSF spaces around bilateral optic nerves; (d) T1 post-contrast coronal image showing empty sella and normal paracavernous structures

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The possibility of an atypical presentation of IIH with unilateral papilledema was considered. Cerebrospinal fluid opening pressure was 290 mm of water with normal composition. She was initiated on acetazolamide, her symptoms improved, reinforcing the diagnosis of IIH. At 3-month follow-up, she remained asymptomatic with partial resolution of papilledema [Figure 1]b.

Unilateral papilledema usually results from orbital or retro-orbital mass lesions; however, it is also reported with IIH. The exact cause(s) is unknown; however, various mechanisms have been proposed, like anomalous optic nerve sheath, variations of the trabecular meshwork of fibrous adhesions in the subarachnoid space surrounding the optic nerve and anatomical difference in lamina cribrosa. [2],[3],[4] A recent study suggests compartmentation of the subarachnoid space of the optic nerve as a cause of asymmetric papilledema in IIH. [5]

Lepore [1] noted that patients with unilateral papilledema are significantly older than those with bilateral papilledema; however, there was no difference between the groups with respect to disease duration, symptoms, severity, visual performance measures, CSF opening pressure and clinical course. Prompt evaluation and early diagnosis and management can prevent complications like permanent visual loss. [5] A treating physician/ ophthalmologist should be aware of such an atypical albeit rare presentation of IIH, and a lumbar puncture along with monitoring of CSF opening pressure should be considered in cases of unilateral optic disc edema if there is no obvious cause determined by neuroimaging.

  References Top

1.Lepore FE. Unilateral and highly asymmetric papilledema in pseudotumor cerebri. Neurology 1992;42:676-8.  Back to cited text no. 1
2.Moster ML. Unilateral disk edema in a young woman. Surv Ophthalmol 1995;39:409-16.  Back to cited text no. 2
3.Maxner CE, Freedman MI, Corbett JJ. Asymmetric papilledema and visual loss in pseudotumor cerebri. Can J Neurol Sci 1987;14:593-6.  Back to cited text no. 3
4.Huna-Baron R, Landau K, Rosenberg M, Warren FA, Kupersmith MJ. Unilateral swollen disc due to increased intracranial pressure. Neurology 2001;56:1588-90.  Back to cited text no. 4
5.Killer HE, Jaggi GP, Flammer J, Miller NR, Huber AR, Mironov A. Cerebrospinal fluid dynamics between the intracranial and the subarachnoid space of the optic nerve. Is it always bidirectional? Brain 2007;130:514-20.  Back to cited text no. 5


  [Figure 1], [Figure 2]

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