Giant cell arteritis presenting with dizziness and a normal erythrocyte sedimentation.

Abstract

To the Editor: Giant cell arteritis (GCA) or temporal arteritis, the most common form of systemic vasculitis in adults, has a mean age of onset of 70. The most common manifestations are headache, visual symptoms, jaw claudication, and scalp tenderness, but about 40% of patients present with atypical manifestation. Familiarity with unusual manifestations of GCA facilitates early diagnosis and treatment, thereby reducing the risk of vision loss. In most cases, the erythrocyte sedimentation rate (ESR) is markedly elevated. In the present study, a patient presented with a normal ESR and complained of dizziness as her initial symptom. An 84-year-old white woman presented to her primary care physician complaining of dizziness of 8 months duration associated with headaches, difficulty hearing, and a spinning sensation. She also complained of weakness and fatigue. There was no history of changes in vision or jaw claudication. The patient stated that her symptoms started after a minimal stroke sustained 8 months before. The stroke was associated with left-sided weakness that resolved completely. There was no scalp tenderness or other abnormalities. The temporal arteries were normal. The ESR was 6 mm/h (normal range 0–30), and the hemoglobin was 12.7 g/dL (normal range 11.7–15.5). Aspartate aminotransferase (serum glutamic oxaloacetic transaminase) and alanine aminotransferase (serum glutamate pyruvate transaminase) levels were within the normal limits. The alkaline phosphatase level was elevated at 346 βμ/L (normal range 20–120). The patient was referred to an ear-nose-throat specialist to be evaluated for dizziness and associated symptoms. The impression was nonotological-origin vertigo (dizziness) and a high-frequency sensorineural hearing loss with associated tinnitus, and the patient was started on therapy. In follow-up visits, the patient complained of painful chewing in addition to sustained dizziness and headaches. The patient was found to have severe osteoporosis, confirmed using a 3-dimensional quantitative computerized tomographic bone mineral densitometry, so that the side effects of corticosteroid therapy, especially in her condition, were explained to her. Thereafter, she was started on prednisone 40 mg once a day along with vitamin D and calcium supplements. The patient refused to use calcitonin nasal spray and oral bisphosphonate. The right and left temporal arteries were biopsied 2 weeks later. The biopsies revealed normal right temporal artery and pathologic changes consistent with GCA in the left temporal artery. After starting the patient on prednisone, the symptoms improved dramatically, but corticosteroid therapy (40 mg/d) was discontinued after 3 weeks when she developed a compression fracture. As a result, the symptoms of dizziness, jaw claudication, and headache returned. Then she was restarted on a low-dose prednisone of 20 mg/d, which resulted in gradual resolving of the symptoms again. This patient presented with dizziness (described as spinning sensation), which is an atypical manifestation of GCA. Dizziness/vertigo as a main complaint in GCA is rare. Compromise of the arteries supplying the otic region can lead to tinnitus, hearing loss, and vertigo.1 As a result of this case, the differential diagnosis of dizziness in the elderly should include GCA even when typical symptoms of GCA and an elevated ESR are lacking. In this case, when the patient was started on prednisone, dizziness and other symptoms were resolved dramatically. All the symptoms started immediately after she had a stroke. Neurological problems, including neuropathies, transient ischemic attacks, and strokes, occur in 30% of patients with GCA.2 Because, in this 84-year-old patient, the history of dizziness, headache, and tinnitus started after the stroke, GCA should have been strongly considered in the differential diagnosis. This patient did not complain of any other symptoms. The physical examination did not show scalp tenderness, proximal muscle weakness, or any abnormality of the temporal arteries. The patient's ESR was 6 mm/h. In patients with GCA, ESR is usually above 50 mm/h, with an average of 80 to 100 mm/h using the Westergren method. There have been different studies that have reported a few cases of biopsy-proven GCA with low or normal ESR.3-9 In most of these cases, the patients had a history of receiving corticosteroid therapy for different reasons, such as polymyalgia rheumatica.3 One review found that about 4% to 13% of patients with clinical polymyalgia rheumatica or GCA have a normal ESR.2, 10 Other studies5 reported GCA with a normal ESR in 10% to 30% of patients. A normal ESR may occur with typical and atypical presentation of GCA. The diagnosis of GCA was strongly suspected when the patient developed jaw claudication almost 11 months after the onset of dizziness.