Pseudotumor cerebri ("benign" idiopathic intracranial hypertension), MedPix™ : 433

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Location: Brain and Neuro	Sublocation: CSF and Subarachnoid	Category: Clinical Exam Finding or Sign
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Pseudotumor cerebri ("benign" idiopathic intracranial hypertension)
Topic 433 - Created: 1998-01-26 00:00:00-05 - Modified: 2011-12-21 09:35:19-05

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.

Â» Reported causes include:
â€¢ Hypervitaminosis A
- by increasing interstitial water?
[PubMed] - - LINK -
â€¢ Dural sinus thrombosis/stenosis
- increasing intracranial venous pressure
- by deceasing CSF absorption
â€¢ Pregnancy
- increased venous pressure
â€¢ Obesity
- increased venous pressure

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

Â» LP Results in psedudotumor cerebri:
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Â» The physiology may be related to:
â€¢ cerebral brain interstitial edema
â€¢ hyperemic brain swelling - vasocongestion
â€¢ imbalance between CSF production and resorption
â€¢ venous hypertension
â€¢ dural sinus thrombosis or stenosis (including bilateral transverse sinus stenosis) - [PubMed] - LINK -

Â» 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] - - LINK -
[PubMed] - - LINK -

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

Â» 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.

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

â€¢ Treatment options
Â» Reduce

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Title: Pseudotumor cerebri ("benign" idiopathic intracranial hypertension) ::

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