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A 13-year-old girl with acne vulgaris presents with a headache 2 weeks after completing a course of treatment with 2 unspecified medications. Her headache began 1 week ago and is dull in quality, diffuse in location, and constant. It is worse at night and has started to wake her up from sleep. In the past 2 days, she has also developed diplopia. On examination, she is overweight. Neurologic examination is significant for bilateral papilledema and a right sixth nerve palsy. Magnetic resonance imaging (MRI) of the brain, including magnetic resonance venogram, is normal. Opening pressure on lumbar puncture is 30 cm H2O, and cerebrospinal fluid (CSF) composition is normal. Which medication would be most appropriate for her treatment?

A. Propranolol

B. Acetazolamide

C. Ibuprofen

D. Cyproheptadine

E. Sumatriptan

Answer: B

Idiopathic intracranial hypertension (IIH), previously known as pseudotumor cerebri, is characterized by elevated intracranial pressure (ICP) in the absence of any structural cause or CSF abnormality. The underlying cause is not well understood. Before puberty, girls and boys are equally likely to be affected. After puberty, there is a female predominance. Recent use of certain medications, including isotretinoin and minocycline (both often used for treatment of acne), has been associated with idiopathic intracranial hypertension. Being overweight is also associated, and weight loss is an important component of long-term management in overweight patients.

Elevated ICP should be suspected in a patient who complains of a headache that is worse when lying flat; the patient may describe this as worse when they lie down to go to sleep or that the headache is waking them up from sleep. A fundoscopic examination is essential to evaluate for papilledema, which is swelling of the optic nerve head due to increased pressure. However, early on, this finding can be absent. Neuroimaging, including venous imaging, is necessary to rule out other causes of elevated ICP such as an intracranial mass or venous sinus thrombosis. Following neuroimaging, lumbar puncture with measurement of opening pressure should be performed with the patient in the lateral decubitus position with the legs extended. IIH can be vision threatening, and close monitoring of visual function is needed.

Carbonic anhydrase inhibitors, such as acetazolamide, are first-line therapies because they decrease CSF production. Topiramate also has weak carbonic anhydrase inhibition. Propranolol and cyproheptadine are used as migraine preventives in children; ibuprofen and sumatriptan are acute therapies for migraine.

A 12-year-old boy complains of episodic severe headaches for the past 6 months. The headaches occur once ...

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