A lumbar puncture is performed in order to confirm increased pressure. An elevated opening pressure greater than 200 mm H2O and no sign of inflammation on cerebrospinal fluid analysis supports the diagnosis of pseudotumor cerebri.
Neuroimaging with CT or MRI should be obtained in patients suspected of having pseudotumor cerebri. Imaging should demonstrate normal findings, as presence of a space-occupying lesion would indicate a specific underlying cause behind the intracranial hypertension, which would immediately rule out pseudotumor cerebri.
For patients with pseudotumor cerebri who are obese, weight loss is recommended, as studies have shown reduction in symptoms following weight loss.
Carbonic anhydrase inhibitors such as acetazolamide are believed to reduce the rate of cerebrospinal fluid production, and their use has been shown to have associated improvement in outcomes in patients with pseudotumor cerebri.
Topiramate is an anti-epileptic medication that inhibits carbonic anhydrase, and it's also commonly used in the treatment of migraine headaches, making it an attractive candidate for therapy in pseudotumor cerebri. It is thought to help mitigate symptoms by reducing production of cerebrospinal fluid, similar to acetazolamide.
For patients whose condition is refractory to pharmacologic management, there are surgical options that may be considered. One of these procedures is cerebrospinal fluid (CSF) shunting, in which a device is inserted to divert CSF into the peritoneum in order to relieve pressure. Another is optic nerve sheath fenestration, in which incisions are made in the optic nerve sheath, again to relieve pressure.
Part of the management of pseudotumor cerebri includes discontinuing any medications or supplements known to be associated with increased risk of developing the condition. There are multiple medications with these associations, however the most prominent to know are tetracyclines and vitamin A-containing compounds.
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