Severe conditions associated with secondary headaches

Learn the three severe conditions associated with secondary headaches and how to diagnose and treat them.
Last update26th Nov 2020

Secondary headaches are, by definition, due to associated medical conditions. Let’s take a look at three severe conditions, often associated with headache, that you should be aware of:

  1. Giant cell arteritis
  2. Tumors
  3. Idiopathic intracranial hypertension or pseudotumor cerebri

Giant cell arteritis

Giant cell arteritis is an example of one such condition. It is a serious inflammatory disorder affecting the extracranial carotid artery. It is most often seen in individuals over the age of 60, although it can be seen in individuals as early as age 50.

The headache of giant cell arteritis is often located over the temples. It is dull and achy and can be bilateral or unilateral. Fatigue or discomfort in jaw muscles (i.e., jaw claudication) with chewing hard or chewy items is a classic finding, along with weight loss and myalgia.

Figure 1. Clinical features and pain characteristics of giant cell arteritis include dull and achy pain located over one or both temples along with jaw claudication, weight loss, myalgia, thickened temporal arteries and elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels.

On examination, you’ll find temporal arteries that are often thickened and have either a reduced pulse or are pulseless. Labs will reveal elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels. Temporal artery biopsy is diagnostic.

When the disease affects the ophthalmic artery, blindness can result if not treated. This is the region involved in about 50% of those affected.

Treatment of giant cell arteritis

Giant cell arteritis is treated with high dose steroids. Prompt treatment can prevent blindness.

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Tumors

Patients with headaches often worry that they may have a tumor, but tumors are actually a rare cause of severe headaches. Usually a tumor will present with other focal neurological complaints or signs.

There are three major clues that a headache may actually be related to a tumor:

  1. Worsening of the headache with exertion or a change in position
  2. Increased pain at night which awakens a patient from sleep
  3. A drastic change in an established headache pattern

The headache pain from a tumor is generally nondescript holocranial pain, and is often described as dull, achy, and intermittent.

In a patient with a known cancer, a new headache warrants imaging with magnetic resonance imaging (MRI), both with and without contrast.

Figure 2. Features and characteristics of headache due to tumor include nondescript holocranial, dull, achy, intermittent pain. Often a tumor will present with other focal neurological complaints or signs.

Treatment of tumor-associated headaches

Steroids can decrease the swelling of surrounding brain tissues, which can help to ameliorate headache and other neurologic dysfunction until the tumor can be treated with more specific measures such as surgery or radiation.

Idiopathic intracranial hypertension or pseudotumor cerebri

Idiopathic intracranial hypertension is also called pseudotumor cerebri. It involves diffusely increased intracranial pressure from generalized edema with headache characteristics similar to those seen with tumors. Papilledema is typical on fundoscopic examination, and many patients have diplopia. Untreated, it can lead to visual impairment.

Women of childbearing age are affected more frequently, and most are obese.

Figure 3. Clinical features and pain characteristics of idiopathic intracranial hypertension, or pseudotumor cerebri, include holocranial pain with papilledema, diplopia, and visual impairment that most often affects obese women of childbearing years.

Treatment of idiopathic intracranial hypertension or pseudotumor cerebri

Treatment is multifaceted, and includes acetazolamide (an oral carbonic anhydrase inhibitor), loop diuretics, weight reduction, serial lumbar punctures, surgical correction with optic nerve fenestration surgery, and lumbar-peritoneal or ventriculoperitoneal shunts.

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Recommended reading

  • Chou, DE. 2018. Secondary headache syndromes. Continuum (Minneap Minn). 24: 1179–1191. PMID: 30074555
  • Ferguson, LW and Gerwin, R. 2005. Clinical Mastery in the Treatment of Myofascial Pain. Baltimore: Lippincott Williams & Wilkins.
  • Fernández-de-las-Peñas, C, Arendt-Nielsen, L, and Gerwin, R. 2010. Tension-Type and Cervicogenic Headache—Pathophysiology, Diagnosis and Management. Sudbury: Jones and Bartlett Publishers.
  • Goadsby, PJ and Silberstein, SD. 1997. Headache. Boston: Butterworth-Heinemann.
  • Goadsby, PJ, Silberstein, SD, and Dodick, DW. 2005. Chronic Daily Headache for Clinicians. Hamilton: BC Decker.
  • Green, MW. 2012. Secondary headaches. Continuum (Minneap Minn). 18: 783–795. PMID: 22868541
  • Silberstein, SD, Lipton, RB, and Goadsby, PJ. 2002. Headache in Clinical Practice. 2nd edition. London: Martin Dunitz.

About the author

Robert Coni, DO EdS
Neurohospitalist, Medical Director and Coordinator at the Grand Strand Medical Center, and Clinical Assistant Professor at the University of South Carolina.
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