Headaches

With respect to imaging studies, recent research has suggested that ultrasound with color Doppler techniques may be useful in diagnosing GCA.^20,21 Other imaging studies may be needed based on the entire clinical presentation, specifically if involvement of other vascular territories is suspected.

Historically, the most definitive diagnostic test is temporal artery biopsy. It should be emphasized that the diagnosis of GCA is a clinical one in which biopsy, laboratory, and other clinical findings all play a supportive role in the diagnosis.

Treatment consists of high-dose corticosteroids, usually prednisone 60 to 80 mg/d initially. Treatment should not be withheld pending temporal artery biopsy as the findings remain positive for weeks. There is ongoing research into low-dose aspirin as well as other immune-modulating drugs. Ophthalmologic consultation should be sought for patients with visual symptoms and surgical follow-up is necessary for biopsy.²²

Idiopathic Intracranial Hypertension

Idiopathic intracranial hypertension, also known as pseudotumor cerebri or benign IIH, is a disease of abnormal elevated intracranial pressure (ICP) that typically affects overweight women of child-bearing age. The etiology is unclear. Patients present with nonspecific headaches and possible visual complaints that can range from blurring to sudden visual loss. Although IIH is rare in healthy men, those who do develop the condition have a higher risk of permanent vision loss.

The most important clinical finding is papilledema, which should be sought on all patients with a new headache as IIH may lead to progressive blindness.²³ A detailed history and physical examination should be performed as well as imaging to investigate for intracranial masses and alternate etiologies of increased ICP. Once other causes are excluded, the diagnosis is established by measuring an elevated opening pressure during lumbar puncture performed with the patient in the lateral position.

Pharmacologic therapy is aimed at decreasing ICP. Acetazolamide is a mainstay of medical therapy. Surgical treatments such as optic nerve sheath fenestration and cerebrospinal fluid diversion procedures may be performed to limit visual loss. Ophthalmologic consultation should always be obtained.²⁴

Indomethacin-Responsive Headache Syndromes

While indomethacin may be effective for many types of headaches, the hemicrania syndromes of paroxysmal hemicrania and hemicrania continua are defined by an absolute, invariable response to indomethacin therapy. In IRHS, headaches are typically unilateral, moderate to severe, last longer than other headache syndromes, and may occur in conjunction with autonomic symptoms.

Paroxysmal hemicrania consists of 5- to 30-minute episodes with pain-free periods. This syndrome, however, may progress to hemicrania continua, which is characterized by longer lasting, chronic headaches with less prominent autonomic symptoms. When considering either of these syndromes, a careful history and physical examination should be performed and other etiologies considered.^24-26

Cluster Headache

Cluster headache is characterized clinically by unilateral paroxysmal pain, usually involving the upper half of the face and head. Episodes typically last between 15 to 180 minutes and can recur multiple times per day. To make the diagnosis, at least one local autonomic symptom must be present, such as forehead sweating or redness, conjunctival injection, lacrimation, or nasal congestion or drainage. Other ocular symptoms, such as miosis, ptosis, or lid swelling, may occur. Cluster headache is more common in men, and patients typically are restless or agitated. Laboratory studies and imaging are not typically necessary unless evaluation for other problems is warranted. Abortive treatment should include 100% oxygen by non-rebreather facemask, as well as a 5-HT1 receptor agonist, including metoclopramide, the triptan drugs, or ergotamine alkaloids. Corticosteroids may be effective in terminating a cluster headache cycle, and multiple medications have been used for prophylactic management. There are multiple surgical options available for refractory symptoms. Prevention should focus on a headache diary to identify triggers. Use of alcohol and tobacco products have also been shown to worsen symptoms.²⁴

Conclusion

As patients present to the ED with a wide variety of headaches, EPs should include dangerous and secondary causes highest in the differential diagnosis. However, with a careful history and physical examination, other headache syndromes may be diagnosed with implications that can improve immediate and follow-up treatment, and, in some cases, prevent serious complications—particularly blindness and, though rare, death.

Dr Tadros is an associate professor, department of emergency medicine, West Virginia University, Morgantown. Dr Minardi is an associate professor, department of emergency medicine and medical education, West Virginia University, Morgantown.