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Idiopathic intracranial hypertension

Secondary to increased venous sinus pressure.

Predilection for young women who are likely to be obese.

Idiopathic intracranial hypertension is related to elevated intracranial pressure without a known cause.

Diagnosis includes: symptoms of elevated intracranial pressure, such as headache, tinnitus, diplopia, and/or visual disturbances without secondary etiologies, edema on fundus examination and elevated CSF pressure on lumbar puncture performed in the lateral decubitus position,  with an opening pressure of greater than 25 cm of water in adults and greater than 28 cm of water in children.

Chronic daily headaches, normal neurological examination (except for papilledema) and normal lab examination (except for an empty sella).

May have a severe lateralizing pulsatile headache, with intracranial noises, double vision, and nausea.

Unremitting headaches with episodic worsening, accompanied by pulsatile tinnitus, transient visual difficulties and diplopia may occur.

It primarily affects women of child bearing age.

IIH is associated strongly with obesity.

IIH prevalence has increased to 9.9 per 100,000 in 2022, and correlates with increasing US obesity rates.

Its pathogenesis may be related to hypersecretion and/or decreased absorption of CSF related to increase levels of androgens, glucocorticoids, glucagon-like peptide, and inflammatory cytokines associated with central opacity.

Visual acuity is preserved until late in the course of the illness.

Bilateral optic disc edema is often present.

The CSF has an opening pressure of greater than 25 cm of water, and the CSF is normal.

Neuroimaging includes a normal brain MRI.

Treatment includes weight reduction, surgical treatment with either lumbar to peritoneal shunting, or fenestration of the optic nerve sheath, and lowering of intracranial pressure with acetazolamide.

First line management for IIH is a carbonic anhydrase inhibitor such as a acetazolamide, which causes a rapid reduction intracranial pressure by decreasing CSF production.

The combination of a carbonic anhydrase  inhibitor and weight loss of 5 to 10% is effective in improving peripheral vision, papilledema, and vision quality of life measures.

Bariatric surgery and GLP-one receptor agonists have demonstrated decreased intracranial pressure compared with control patients.

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