By Robert Kunkel, MD, Consultant, Center for Headache and Pain Neurological Institute, Cleveland Clinic, Cleveland, OH
I first saw George on March 12, 2008. He was 75 years old and had had a headache since December 12, 2007, when he slipped on ice and fell. He said he didn’t hit his head, but he had left-sided neck and head discomfort afterward, which had persisted. He had high blood pressure, which was well controlled, but no other significant medical problems.
George described the head pain as “sharp” and constant in character. It was strictly on the left side and involved the left neck and upper shoulder area, the back of the head and spread forward above the left ear into the temple. It was not aggravated by neck motion, coughing or straining. He had a CT scan of the brain in January of 2008, which was negative, and a CT scan of his neck showed only mild degenerative arthritic changes. He was referred for physical therapy, which helped the neck pain but had no effect on his headache.
In February, his primary care physician diagnosed him with occipital neuritis and he was put on a dose of prednisone (a cortisone) that was tapered over the course of seven days. His head pain was completely gone for a few days but recurred when he reduced the dose. In addition to the head discomfort, George reported that he felt tired and had a lack of stamina. While taking the prednisone, he “felt like a new man.”
When I examined him, he had been off of prednisone for over two weeks. The exam was normal, including his blood pressure, except for mild tenderness with pressure over the left lower neck and at the base of the skull. His neck motion was slightly reduced.
On the presumption that this was a form of occipital neuralgia, we injected his occipital nerve with a mixture of an injectable cortisone preparation and a local anesthetic. Following this procedure, he was free of pain for five days.
Because this was a new headache for George, and because of his response to the prednisone, the diagnosis of temporal arteritis was also considered. On lab testing, he had an elevated sedimentation rate (sed rate), which reflects inflammation in the body. His sed rate was 70 while a normal level is between 0 and 20. Because of this elevated sed rate, we had a biopsy of the left temporal artery done one week after his first visit. His headache had returned by then and the biopsy showed active inflammation in the artery wall, which is typical of temporal arteritis. He was started on a daily dose of 60 mg. of prednisone along with extra calcium and vitamin D.
Temporal arteritis, also known as giant cell arteritis, is an inflammation in the wall of medium-sized arteries. The diagnosis is made from the findings seen on a biopsy of the temporal artery (an artery in the scalp of the temple). The inflammatory process is associated with the presence of “giant cells” along with other inflammatory cells seen on microscopic examination. Although this condition can involve arteries in any area of the body, it has a propensity to involve the arteries of the head and neck. It is an auto-immune disorder and the exact cause or trigger of the inflammation is unknown.
The headache associated with temporal arteritis is usually not severe and more often is a dull aching, pressure sensation. The pain, however, may take on any character and, in this case, the pain was described as sharp. It is usually located on one side of the head, but can be more generalized. Since temporal arteritis is an inflammatory condition, systemic symptoms such as tiredness and fatigue are common. If the arteries to the chewing muscles are involved, pain may increase with eating and chewing (this is called “jaw claudication”). This latter symptom is felt to be quite diagnostic of temporal arteritis, but it rarely occurs. Muscle soreness, stiffness and aching are also common symptoms in temporal arteritis.
Temporal arteritis is a condition that occurs in persons over 50 years of age and needs to be diagnosed and treated promptly. It is one of the few headache conditions that require urgent treatment. If not treated promptly, it may result in permanent visual defects or blindness in 20-30% of those afflicted. The visual problems are the result of the lack of adequate blood supply to the eye due to the thickening and narrowing of the involved arteries.
The best screening test is the sed rate blood test. With temporal arteritis, the sed rate is quite high, frequently over 100. George’s value (70) was probably lower due to his prior use of prednisone. There is also often mild anemia (low red blood cell count) present.
If temporal arteritis is suspected, prednisone should be started immediately at a dose of at least 60 mg. a day, because of the high rate of visual loss that may occur without treatment. Arrangements for a biopsy of the temporal artery, which will confirm the diagnosis, should be made as soon as possible after treatment is begun. A few days on prednisone will not alter the inflammation in the artery enough to obscure the pathological changes that are typical of this condition.
To cure the condition and prevent a relapse, prednisone needs to be taken for many months and often for more than a year. Studies have been done with other types of anti-inflammatory drugs, but none of these have been shown to be as effective, so currently prednisone is considered the drug of choice.
Some of the problems associated with the use of prednisone include ulcer disease, high blood pressure, elevated blood sugars, thinning of the skin with easy bruising, poor tissue healing, weight gain and loss of bone density. Since the prednisone may need to be taken for a year or more, managing these complications can be challenging. Ulcer preventive therapy and the use of extra calcium and vitamin D supplements should be used in most persons.
George was put on this protocol and took the prednisone for 17 months. He was followed closely with monthly lab tests, and the prednisone dose was slowly tapered off. While on the prednisone, his blood sugar was mildly elevated at times, he gained 20 pounds and he developed mild acne. The prednisone was stopped completely in August of 2009 and by November of that year he had lost the 20 pounds, and his skin was once again clear. He was headache-free as soon as the prednisone was started and continues to be so without it.
Because George didn’t recall any head or neck discomfort until after he fell, it was initially presumed that he had injured his occipital nerve. In truth, if he had not fallen, the headache would have occurred anyway. As with George, any person over the age of 50 who develops the new onset of a headache should be screened for temporal arteritis. Prompt diagnosis and treatment is essential to prevent permanent visual loss.