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Headache in a Well-Appearing Young Woman



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Am Fam Physician. 2009 Nov 15;80(10):1143-1144.

A healthy-appearing 23-year-old woman with no notable medical history presented with a headache, neck stiffness, and visual changes that have persisted for 10 days. The patient reported double vision, blurred vision, and transient visual changes when opening her eyes; however, she had normal visual acuity. She also reported paresthesia in her left arm and hand. She denied having fevers, myalgias, or incontinence. She was afebrile at presentation. Examination revealed tachycardia (heart rate of 110 beats per minute), a body mass index of 35 kg per m2, and nuchal rigidity. Her pupils were equal in size, round, and reactive to light and accommodation. Her vision was 20/20 in both eyes. Funduscopic examination was performed (see accompanying figure).

Question

Based on the patient's history and physical examination, which one of the following is the most likely diagnosis?

A. Aseptic meningitis.

B. Complicated migraine headache.

C. Idiopathic intracranial hypertension.

D. Multiple sclerosis.

E. Primary brain tumor.

Discussion

The answer is C: idiopathic intracranial hypertension. The accompanying figure shows widening and blurring of the optic disc margin with compensatory reduction of cup diameter, which is consistent with papilledema. Idiopathic intracranial hypertension, or pseudotumor cerebri, is a syndrome of increased intracranial pressure without a space-occupying lesion. Patients typically present with headache, visual changes, tinnitus, and paresthesia. Brain imaging should be performed initially to rule out a space-occupying lesion and to ensure normal ventricular size. It is then safe to perform a lumbar puncture to demonstrate an elevated opening pressure (greater than 25 cm of water) in the lateral decubitus position.1 When performing a lumbar puncture, it is appropriate to send cerebrospinal fluid for cytology and culture, although specific tests for herpes simplex virus, viral, and Lyme infections are necessary only if the presentation suggests these diagnoses.

Although idiopathic intracranial hypertension is rare, with an incidence of one in 100,000 persons per year, it is 20 times more common in women 20 to 44 years of age who are more than 20 percent above their ideal body weight.2 The syndrome is very rare in persons older than 45 years.

Headache with nuchal rigidity suggests meningeal inflammation, even without a fever. However, papilledema occurs in less than 5 percent of acute bacterial meningitis cases and is very rare with aseptic meningitis.3

Although a complicated migraine headache may be associated with neurologic findings, it does not lead to papilledema.

Multiple sclerosis is more common in young women and can cause a variety of neurologic symptoms, including visual complications from optic neuritis. Optic neuritis causes a papillitis that is difficult to distinguish from papilledema, but papillitis leads to decreased visual acuity.4

A brain tumor must be considered as a cause of papilledema before performing the lumbar puncture. However, in a young woman who is obese with no known cancer history, the probability of a brain tumor is less than that of idiopathic intracranial hypertension.5

Summary Table

Condition Characteristics

Meningitis

Headache, nausea, and nuchal rigidity, with or without fever; papilledema is rare, especially in aseptic meningitis

Complicated migraine headache

Typically, photophobia, phonophobia, and nausea; neurologic changes may occur, but papilledema is not present

Idiopathic intracranial hypertension (pseudotumor cerebri)

Headache, nuchal rigidity, papilledema; sensory changes include tinnitus, visual changes, and paresthesia; elevated opening pressure on lumbar puncture; normal visual acuity in acute presentation

Multiple sclerosis

Optic changes and urinary retention are often present, and multiple distinct motor or sensory deficits may occur; papillitis with optic neuritis, but decreased visual acuity

Brain tumor

May cause headache, nuchal rigidity, sensory changes, papilledema, fever, fatigue, and weight loss; must be considered before idiopathic intracranial hypertension

Summary Table

View Table

Summary Table

Condition Characteristics

Meningitis

Headache, nausea, and nuchal rigidity, with or without fever; papilledema is rare, especially in aseptic meningitis

Complicated migraine headache

Typically, photophobia, phonophobia, and nausea; neurologic changes may occur, but papilledema is not present

Idiopathic intracranial hypertension (pseudotumor cerebri)

Headache, nuchal rigidity, papilledema; sensory changes include tinnitus, visual changes, and paresthesia; elevated opening pressure on lumbar puncture; normal visual acuity in acute presentation

Multiple sclerosis

Optic changes and urinary retention are often present, and multiple distinct motor or sensory deficits may occur; papillitis with optic neuritis, but decreased visual acuity

Brain tumor

May cause headache, nuchal rigidity, sensory changes, papilledema, fever, fatigue, and weight loss; must be considered before idiopathic intracranial hypertension

Address correspondence to James C. Higgins, CAPT (Ret.), MC, USN, at James.Higgins@med.navy.mil. Reprints are not available from the authors.

The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the U.S. Navy Medical Department or the U.S. Navy at large.

Author disclosure: Nothing to disclose.

REFERENCES

1. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology. 2002;59(10):1492–1495.

2. Friedman DI. Pseudotumor cerebri. Neurol Clin. 2004;22(1):99–131.

3. Mandell GL, Bennett JE, Dolin R. Principles and Practice of Infectious Diseases. 7th ed. Philadelphia, Pa.: Churchill Livingstone; 2009.

4. Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa.: Saunders; 2008.

5. Greenlee RT, Murray T, Bolden S, Wingo PA. Cancer statistics, 2000. CA Cancer J Clin. 2000;50(1):7–33.

Contributing editor for Photo Quiz is John E. Delzell, Jr., MD, MSPH.

The editors of AFP welcome submissions for Photo Quiz. Guidelines for preparing and submitting a Photo Quiz manuscript can be found in the Authors' Guide at http://www.aafp.org/afp/photoquizinfo. To be considered for publication, submissions must meet these guidelines. E-mail submissions to afpphoto@aafp.org.



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