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Pseudotumor cerebri ("benign" idiopathic intracranial hypertension), MedPix™ : 433 - Medical Image Database and Atlas
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More Like This ? Pseudotumor cerebri ("benign" idiopathic intracranial hypertension)
Topic 433 - Created: 1998-01-26 00:00:00-05 - Modified: 2010-05-08 09:52:44-04
ACR Index: 1.3

Idiopathic intracranial hypertension (IIH) - i.e. not produced by an expansile intracranial mass lesion - is often called "pseudotumor cerebri". Reported by Heinrich Quincke in 1893 and by Max Nonne in 1904, it was originally called "serous meningitis". It is most common in overweight women 20-50 years old. The clinical presentation usually includes headaches or visual problems.

» Reported causes include:
• Hypervitaminosis A
- by increasing interstitial water?
[PubMed REF] - Click for HyperLink
• Dural sinus thrombosis/stenosis
- increasing intracranial venous pressure
- by deceasing CSF absorption
• Pregnancy
- increased venous pressure
• Obesity
- increased venous pressure

» The diagnostic criteria include:
• secondary features of increased pressure (e.g. papilledema)
• elevated CSF pressure (>250 mm H2O) by LP in lateral decubitus position
• normal cerebrospinal fluid chemistry and cells
• normal cerebral anatomy by CT/MR imaging

» LP Results in psedudotumor cerebri:
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» The physiology may be related to:
• cerebral brain interstitial edema
• hyperemic brain swelling - vasocongestion
• imbalance between CSF production and resorption
• venous hypertension, dural sinus stenosis (including bilateral transverse sinus stenosis - [PubMed REF] Click for HyperLink

» Recent literature has demonstrated that the transverse sinus stenosis may be secondary to increased intracranial pressure - and can be reversed with CSF shunting alone - no vascular intervention. It may also recur when intracranial pressure rises [PubMed REF]

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» Thus, we have a classic "chicken and egg" problem: Is pseudo tumor caused by sinus stenosis or is it causing the stenosis?

» Hypervitaminosis A may cause IIH. This was described in arctic explorer who ate polar bear liver, as well as acne patients treated with retinoic acid.

» Patients with IIH may have elevation of both CSF and serum Vitamin A, w/o exogenous sources.
Click for HyperLink
[PubMed REF] - Click for HyperLink

• Treatment options
» Reduce

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Contributor Credits

Submitted by: James G. Smirniotopoulos, M.D. - Author Info
Affiliation: Uniformed Services University
Approved By: James G. Smirniotopoulos, M.D. - Editor Info
Affiliation: Uniformed Services University


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