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Table 2 Clinical stages of IIH and their corresponding recommended treatment. (Translated and reproduced from the website of the German Society for Neurology – DGN. https://dgn.org [24])

From: Long-term telemetric intracranial pressure monitoring for diagnosis and therapy optimisation of idiopathic intracranial hypertension

Clinical Stage

Corresponding Recommended Treatment Strategy

Stage I:

IIH without focal neurological deficits (mild papilledema)

• Weight reduction

• Acetazolamide (with regular control of blood Potassium level)

• Alternatively: Topiramat + Furosemide (with regular control of blood Potassium level)

Stage II:

IIH with:

 • Significant papilledema and/or

 • Sight impairment or visual field loss

• Treatment for Stage I with:

• Successive LPs (nearly every 2 weeks) until pressure is < 20 cm H2O

Goals:

• Reduction of Pressure < 18 cm H2O

And

Additional Measures:S

• Reduction of acetazolamide Dosage

And

Stage III:

IIH with:

 • Progressive loss of sight and/or

 • Rapid progressive onset of sight impairment or Visual field loss.

• Treatment for Stage I and II with:

• Neuroradiological Intervention (stenting) and/or

• Surgical Intervention

o CSF diversion (VP, lumbo-peritoneal Shunting)

o Optic nerve sheath fenestration (ONSF)

• Normalising of sight

• Regular Control of CSF opening pressure and clinical symptoms