An 18-year-old woman comes to the clinic due to persistent headaches that have occurred daily for 3 months. The headaches are holocranial, pulsating, and especially bothersome at night. Ibuprofen has not helped the pain. The patient has double vision when looking to the side and pain with eye movements. She has no history of head trauma prior to the onset of symptoms and has had no fever, vomiting, photophobia, or weakness. The patient has no chronic medical conditions. Her mother and sister have migraine headaches. Temperature is 37 C (98.6 F), blood pressure is 130/85 mm Hg, and pulse is 90/min. BMI is 34 kg/m2. She is alert and oriented. Physical examination shows no nuchal rigidity or sinus tenderness. Visual acuity is 20/20. The pupils react normally to light and accommodation. Funduscopic examination shows marked blurring of the optic disc margins bilaterally. She has impaired lateral gaze with the left eye. CT scan of the head with and without contrast is normal. Which of the following is the most appropriate next step in management of this patient?
Idiopathic intracranial hypertension | |
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CSF = cerebrospinal fluid. | |
This patient has pulsatile headaches (worse at night), abducens nerve (CN VI) palsy (double vision), and optic disc edema, symptoms suggestive of increased intracranial pressure (ICP). In the context of a normal CT scan, these findings are likely due to idiopathic intracranial hypertension (IIH). This condition is most commonly seen in young, obese (BMI ≥30 kg/m2) women and is also associated with certain medications (eg, retinoids, tetracyclines, growth hormone).
Patients with IIH typically have holocranial or lateralized headache, vision changes (blurry vision, diplopia), and pulsatile tinnitus ("whooshing" sound in the ears). Abducens nerve (CN VI) palsy (lateral rectus palsy) is a nonlocalizing sign of high ICP and can be seen in several different conditions.
Initial evaluation of IIH includes complete ocular examination and neuroimaging to exclude secondary causes of intracranial hypertension (eg, mass, hemorrhage). MRI, often with venography to rule out cerebral venous thrombosis, is the preferred imaging modality; however, CT scan is generally faster and easier to obtain in emergencies.
After a space-occupying lesion is excluded, lumbar puncture can be performed safely and is indicated to document elevated opening pressure (>250 mm H2O). Cerebrospinal fluid studies (eg, cell counts) are normal in IIH.
(Choice A) Erythrocyte sedimentation rate is a nonspecific marker of inflammation that is particularly important when temporal arteritis is suspected as the cause of headache. Although temporal arteritis commonly presents with headache and visual symptoms, it almost never occurs in patients age <50, constitutional symptoms (eg, fever, weight loss) are common, and increased ICP is not seen.
(Choice C) MR angiogram is indicated if subarachnoid hemorrhage (SAH) is suspected. However, headache from SAH is classically sudden in onset (ie, thunderclap) in contrast to this patient's 3-month history. MR angiography is also indicated for suspected trigeminal neuralgia, but the pain is typically isolated to the face (trigeminal nerve [CN V] distribution), and elevated ICP is not seen.
(Choice D) Oral corticosteroids are indicated when headache is due to temporal arteritis, which is highly unlikely in this patient age <50 with elevated ICP. Intravenous corticosteroids are used when neuroimaging reveals elevated ICP resulting from mass effect (eg, tumor, abscess). On occasion, in patients with confirmed IIH (ie, after lumbar puncture) and impending visual loss, corticosteroids can be used as bridging therapy while awaiting definitive surgical treatment.
(Choice E) Oral propranolol can be used for migraine prophylaxis. Migraines classically cause unilateral, throbbing headaches and photophobia. Although this patient has a family history of migraine, her holocranial headache and optic disc edema are suggestive of IIH rather than migraine disorder.
Educational objective:
Idiopathic intracranial hypertension typically presents in young, obese women with headache, vision changes, papilledema, and sometimes abducens nerve (CN VI) palsy. Diagnosis is confirmed by lumbar puncture showing elevated opening pressure and normal cell counts.