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Symposium Idiopathic Intracranial Hypertension (Pseudotumor cerebri)

07.10.2017, Düsseldorf

Ophthalmological Signs and Symtoms of Idiopathic Intracranial Hypertension

Meeting Abstract

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  • Renate Unsöld - Düsseldorf

Symposium Idiopathic Intracranial Hypertension (Pseudotumor cerebri). Düsseldorf, 07.-07.10.2017. Düsseldorf: German Medical Science GMS Publishing House; 2017. Doc17siih04

doi: 10.3205/17siih04, urn:nbn:de:0183-17siih046

This is the English version of the article.
The German version can be found at: http://www.egms.de/de/meetings/siih2017/17siih04.shtml

Published: November 30, 2017

© 2017 Unsöld.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.


Outline

Text

Ophthalmological signs and symptoms particularly papilledema and transient visual obscurations are hallmarks of idiopathic intracranial hypertension. Analysis of optic disc findings in 86 patients showed in 8 cases no papilledema at all in both eyes despite prooven increase of intracranial pressure and optic nerve sheath distention at that time. In 10 cases unilateral papilledema was observed in the presence of bilateral optic nerve sheath distention. Chronic papilledema with only slightly prominent optic dics without leakage in fluorescein angiography was seen in 15 cases leading to the impression of an optic disc anomaly causing considerable delay in diagnosis.

Transient uni- and bilateral visual obscurations are frequently the leading symptoms of intracranial hypertension, usually associated with chronic papilledema. In some patients with preexisting papilledema or chronically increased intracranial pressure treated effectily by medical, interventional (stents) or neurosurgical (shunts)means, a following increase of intracranial pressure did not lead to redevelopment of papilledema. We suspect histological changes within the optic disc following papilledema preventing further development of papilledema. Therefore funduscopy must not not be considered a safe criterion of intracranial pressure in the follow up of patients with idiopathic intracranial hypertension .

Funduscopy showing clear papilledema confirms the suspicion of increased intracranial pressure, absence of papilledema does not rule it out. In these patients repeated spinal taps and measurement of intracranial pressure is needed. Interestingly the same phenomenon seems to represent a protecting mechanism against optic nerve fiber damage.Visual field defects in most oft he 86 patients were and stayed even over years of raised intracranial pressure surprisingly moderate. Mild relative arcuate scotomas and/or an enlarged blind spot where observed in 46 of 86 patients, in 32 cases bilaterally, in 14 unilaterally. After reduction of intracranial pressure visual field defects showed in about two thirds of the patients clear recovery and disappeared in the remaning third.

More severe and deeper scotomas were seen in 12 patients unilaterally, the other side showing mild relative scotomas and/or an enlarged blind spot. In 8 of these patients there was some recovery after reduction of intracranial pressure. In 6 of 86 cases we observed severe and mostly absolute arcuate scotomas resulting in considerable concentric visual field constriction without major recovery after therapy corresponding to severe optic atrophy.

In 14 of 86 cases there were and developed no visual field defects at all. Surprisingly there was no clear correlation between the height and duration of intracranial pressure.

It is not clear why in the majority of cases with intracranial hypertention even over a long period of time there is only moderate optic nerve fiber damage, while a small number of patients with similar height and duration of intracranial pressure develops severe optic atrophy. Possibly the same phenomenon which prevents redevelopment of papilledema in patients with previous or chronic papilledema represents a protective mechanism against nerve fiber damage. Histologic changes within the optic disc may reduce the gradient between intracranial and intraocular pressure. Possibly this mechanism does not work in all patients. Other factors – as we could observe in three patient – are hypoplastic optic discs (crowded disc) and optic disc drusen, increasing the damaging effects of optic disc swelling, as has been shown in anterior ischemic optic neuropathy.

The following conclusions may be helpful:

I. Papilledema and visual obscurations are the most commmon ophthalmological signs and symptoms of idiopathic intracranial hypertension.

II. Unilaterallity or absence of papilledema in prooven increase of intracranial pressure and distention of the optic nerve sheath are not rare. The do not exclude the diagnosis of intracranial hypertension and must not delay examation of intracranial pressure by spinal tap when other signs and symptoms indicate elevated pressure.

III. Even in cases with long standing increase of intracranial pressure the majority of patients shows only moderate visual field defects and damage to the optic nerve.

IV. A considerable number of patients with previous papilledema do not redevelop optic disc swelling when intracranial pressure raises again. Presence of papilledema indicates increased intracranial pressure, it´s absence does not rule it out. If there is any doubt, one should go ahead and perform a spinal tap.

V. Changes within the optic disc after previous papilledema may not only prevent redevelopment of papilledema when intracranial pressure raises again,it may also represent a protective mechanism against optic nerve fiber damage.


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