Pressure headache and blurry vision: Evaluation and treatment of idiopathic intracranial hypertension (IIH)

This chapter provides a succinct description of findings and treatment of severe headaches due to idiopathic intracranial hypertension (IIH). The editors reiterate the importance of fundoscopic evaluation of the retina to rule out optic nerve swelling (papilledema) caused by elevated intracranial pressure (ICP), while cautioning that most patients with Ehlers-Danlos syndrome (EDS) and IIH have a normal eye examination result. A lumbar puncture directly measures CSF pressure but may prove misleading due to either fluctuations in ICP or partial occlusion of the needle used in the lumbar puncture. ICP readings are more precise. Mean ICP wave amplitude (>4mmHg) may reflect decreased brain compliance and more accurately predict the need for CSF diversion. Medications such as acetazolamide or topiramate decrease the production of CSF and lower the ICP. The addition of sodium bicarbonate will prevent the metabolic acidosis associated with the use of acetazolamide and topiramate. In selected patients, a ventriculo-peritoneal or lumbo-peritoneal shunt is effective in controlling headaches and visual symptoms. In evaluating patients for IIH, the reader is also directed to other chapters on headaches for a broader differential diagnosis.