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Headache Associated With Temporal Arteritis
As the name suggests, headache associated with temporal arteritis is located primarily in the temples, with secondary pain often located in the frontal and occipital regions.
A disease of the sixth decade and beyond, temporal arteritis affects whites almost exclusively, and there is a 3:1 female gender predominance.
Temporal arteritis is also known as giant cell arteritis because of the finding of giant multinucleated cells that infiltrate arteries containing elastin, including the temporal, ophthalmic, and external carotid arteries. Approximately half of patients with temporal arteritis also have polymyalgia rheumatica.
What are the Symptoms
Headache is seen in most patients with temporal arteritis. The headache is in the temples and is usually continuous. The character of the headache pain associated with temporal arteritis is aching and has a mild to moderate level of intensity.
A patient with temporal arteritis also may complain of soreness of the scalp, making the combing of hair or resting the head on a firm pillow extremely uncomfortable.
Although temporal headache is present in almost all patients with temporal arteritis, the finding of intermittent jaw claudication is pathognomonic for the disease. In an elderly patient, jaw pain while chewing should be considered secondary to temporal arteritis until proved otherwise.
In the presence of strong clinical suspicion that the patient has temporal arteritis, immediate treatment with corticosteroids is indicated (see discussion of treatment). The reason immediate treatment is needed is the potential for sudden painless deterioration of vision in one eye secondary to ischemia of the optic nerve.
In addition to the signs and symptoms mentioned previously, patients with temporal arteritis experience myalgia and morning stiffness.
Muscle weakness associated with inflammatory muscle disease and many other collagen-vascular diseases is absent in temporal arteritis, unless the patient has been treated with prolonged doses of corticosteroids for other systemic disease, such as polymyalgia rheumatica.
The patient also may experience nonspecific systemic symptoms, including malaise, weight loss, night sweats, and depression.
On physical examination, a swollen, indurated, nodular temporal artery is present. Diminished pulses are often noted, as is tenderness to palpation. Scalp tenderness to palpation is often seen.
Funduscopic examination may reveal a pale, edematous optic disc. The patient with temporal arteritis often appears chronically ill, depressed, or both.
How is Headache Associated With Temporal Arteritis diagnosed?
Erythrocyte sedimentation rate should be obtained in all patients suspected to have temporal arteritis. In temporal arteritis, the erythrocyte sedimentation rate is greater than 50 mm/h in more than 90% of patients.
Less than 2% of patients with biopsy-proved temporal arteritis have normal erythrocyte sedimentation rates. Ideally, the blood for the erythrocyte sedimentation rate should be obtained before beginning corticosteroid therapy because the initial level of elevation of this test is useful not only to help diagnose the disease but also as a mechanism to establish the efficacy of therapy.
The erythrocyte sedimentation rate is a nonspecific test, and other diseases that may manifest clinically in a manner similar to temporal arteritis, such as malignancy or infection, also may markedly elevate the erythrocyte sedimentation rate. Confirmation of the clinical diagnosis of temporal arteritis requires ultrasound imaging and/or a temporal artery biopsy.
Ultrasound imaging can provide confirmation by identification of a halo sign surrounding the affected temporal artery. The finding of a positive halo sign strongly supports the diagnosis of temporal arteritis.
Given the simplicity and safety of temporal artery biopsy, it probably should be performed on all patients suspected of having temporal arteritis. The presence of an inflammatory infiltrate with giant cells in the biopsied artery is characteristic of the disease. Edema of the intima and disruption of the internal elastic lamina strengthen the diagnosis.
A small percentage of patients with clinical signs and symptoms strongly suggestive of temporal arteritis who also exhibit a significantly elevated erythrocyte sedimentation rate have a negative temporal artery biopsy result.
As mentioned, in the presence of a strong clinical impression that the patient has temporal arteritis, an immediate blood sample for erythrocyte sedimentation rate testing should be obtained and the patient started on corticosteroids.
Complete blood cell count and automated chemistries, including thyroid testing, are indicated in all patients with suspected temporal arteritis to help rule out other systemic disease that may mimic the clinical presentation of temporal arteritis.
If the diagnosis of temporal arteritis is in doubt, magnetic resonance imaging (MRI) of the brain provides the best information regarding the cranial vault and its contents.
MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease.
More importantly, MRI helps identify bleeding associated with leaking intracranial aneurysms. Magnetic resonance angiography (MRA) may be useful to help identify aneurysms responsible for neurological symptoms. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. If intracranial hemorrhage is suspected, lumbar puncture should be performed, even if blood is not present on MRI or CT.
Case reports of the utility of positron emission tomography (PET)/computerized tomography scans in the diagnosis of giant cell arteritis of the temporal, occipital, and vertebral arteries may offer additional diagnostic options. Intraocular pressure should be measured if glaucoma is suspected.
Differential Diagnosis
Headache associated with temporal arteritis is a clinical diagnosis supported by a combination of clinical history, abnormal findings on physical examination of the temporal artery, normal radiography, MRI findings, an elevated erythrocyte sedimentation rate, and a positive temporal artery biopsy result.
Pain syndromes that may mimic temporal arteritis include tension type headache, brain tumor, other forms of arteritis, trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, migraine headache, cluster headache, and chronic paroxysmal hemicrania.
Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities.
The pain of chronic paroxysmal hemicrania and cluster headache is associated with redness and watering of the ipsilateral eye, nasal congestion, and rhinorrhea during the headache. These findings are absent in all types of sexual headache.
Migraine headache may or may not be associated with painless neurological findings known as aura, but the patient almost always reports some systemic symptoms, such as nausea or photophobia, not typically associated with the headache of temporal arteritis.
Treatment
The mainstay of treatment for temporal arteritis and its associated headaches and other systemic symptoms is the immediate use of corticosteroids. If visual symptoms are present, an initial dose of 80 mg of prednisone is indicated. This dose should be continued until the symptoms of temporal arteritis have completely abated.
At this point, the dose may be decreased by 5 mg/week if the symptoms remain quiescent and the erythrocyte sedimentation rate does not increase.
Cytoprotection of the stomach mucosa should be considered because ulceration and gastrointestinal bleeding are possible. If the patient cannot tolerate corticosteroids, or the maintenance dose of steroids remains so high as to produce adverse effects, azathioprine is a reasonable next choice.
Complications
Failure to recognize, diagnose, and treat temporal arteritis promptly may result in the permanent loss of vision.
Failure to diagnose the headache associated with temporal arteritis correctly may put the patient at risk if an intracranial pathological condition or demyelinating disease, which may mimic the clinical presentation of temporal arteritis, is overlooked. MRI of the brain is indicated in all patients thought to have headaches associated with temporal arteritis.
Failure to diagnose glaucoma, which also may cause intermittent ocular pain, may result in permanent loss of sight.
Clinical Pearls
The diagnosis of headache associated with temporal arteritis is made by obtaining a thorough, targeted headache history.
As mentioned, jaw claudication is pathognomonic for temporal arteritis, and its presence should be sought in all elderly patients presenting with headache.
Failure to recognize, diagnose, and treat temporal arteritis promptly may result in the permanent loss of vision.
