Giant-cell arteritis.

PURPOSE OF REVIEW: Giant-cell arteritis frequently poses diagnostic and therapeutic challenges. This article summarizes recent investigations concerning diagnosis and treatment of giant-cell arteritis. RECENT FINDINGS: Efforts to improve diagnostic accuracy have centered on serologic markers and imaging techniques. Although the erythrocyte-sedimentation rate, C-reactive protein, and platelet count continue to be the primary markers, others such as interleukin-6 and fibrinogen can provide additional information. High-resolution magnetic resonance imaging and angiography, fluorine-18-fluorodeoxyglucose positron emission tomography, and color Doppler ultrasonography can show findings relevant to the diagnosis of giant-cell arteritis. Recent inquiries suggest a steroid-sparing benefit from initiation of treatment with intravenous steroids. Adjunctive treatment with methotrexate may also provide this benefit. Aspirin appears to decrease likelihood of stroke or visual loss in giant-cell arteritis without increasing bleeding complications. SUMMARY: An expanded repertoire of imaging techniques and serologic markers may supply information relevant to the diagnosis of challenging cases of giant-cell arteritis. Treatment modifications, including initiation with intravenous steroids, or addition of methotrexate or aspirin to the regimen, may decrease morbidity from disease, corticosteroid treatment or both.

Knee deep in the nerve.

A 62-year-old man presented with a 3-week history of a progressive right vision loss. His right optic disk showed some mild elevation. Automated perimetry revealed a junctional scotoma. Magnetic resonance imaging of the brain showed enlargement and enhancement of the right optic nerve, chiasm, and proximal optic tract. A chiasmal biopsy revealed a lesion consistent with malignant optic glioma of adulthood.

Giant Cell Arteritis.

Patients with a suspected diagnosis of giant cell arteritis (GCA) should be started on high-dose corticosteroid therapy without delay. A temporal artery biopsy should be performed after initiation of therapy to confirm the diagnosis. Patients with acute visual or neurologic symptoms present a neuro-ophthalmic emergency. Therapy should be initiated immediately with high-dose intravenous methylprednisolone sodium succinate and followed by high-dose oral prednisone. Treatment may begin with high-dose oral prednisone in patients without visual or neurologic symptoms. Calcium, vitamin D, and peptic ulcer prophylaxis should accompany steroid therapy, as indicated. The following treatments should be considered for patients with suspected GCA and acute visual or neurologic signs or symptoms: intravenous methylprednisolone sodium succinate (250 mg intravenously every 6 hours) should be given for 3 days, followed by oral prednisone (80 mg per day or 1 mg/kg) for 4 to 6 weeks. Prednisone should then be tapered by 10 mg per day every month. Most patients require 1 year of therapy to avoid relapse. Taper and duration should be modified according to erythrocyte sedimentation rate, C-reactive protein, and signs and symptoms of GCA. Rheumatologic consultation and follow-up is often helpful for these patients. For patients with suspected GCA and no acute visual or neurologic signs or symptoms, therapy may begin directly with oral prednisone (80 mg per day or 1 mg/kg) with same taper and duration based on laboratory values and clinical signs and symptoms.

Fourth nerve palsy, homonymous hemianopia, and hemisensory deficit caused by a proximal posterior cerebral artery aneurysm.

A 21-year-old man developed an ipsilateral fourth nerve palsy, contralateral hemianopia, and contralateral hemisensory deficit as manifestations of a proximal right posterior cerebral artery aneurysm. This unusual constellation of signs reflects the involvement of the structures that run in the ambient cistern. The fourth nerve palsy and homonymous hemianopia are attributed to compression by the aneurysm. The hemisensory loss is ascribed to compromise of thalamoperforate arteries emanating from a thrombosed portion of the aneurysm.

The role of unilateral temporal artery biopsy.

PURPOSE: To examine the role of unilateral temporal artery biopsy (TAB) in suspected giant cell arteritis (GCA). DESIGN: Retrospective interventional case series. PARTICIPANTS: We identified 181 subjects from pathology and diagnostic code databases at the University of Pennsylvania Medical Center who underwent TAB between January 1990 and January 2001. METHODS: The medical records for all subjects who underwent TAB were reviewed. Follow-up information was obtained by telephone or record review for those patients who had negative unilateral TAB. MAIN OUTCOME MEASURES: Follow-up information for patients with unilateral negative TAB was reviewed for potential adverse outcomes caused by missed or delayed diagnoses of GCA. Presenting signs and symptoms and laboratory values were recorded for all subjects. Comparisons of clinical profiles between subsets of subjects were performed using Fisher's exact test, significance level alpha = 0.01. RESULTS: Follow-up information was available for 88 (86%) of 102 subjects who had unilateral negative biopsy samples. One (1%) subjects of 88 had a subsequent positive contralateral TAB; no adverse outcomes occurred for this subject or for any other subjects with unilateral negative TAB. Compared with subjects who had unilateral positive or who underwent bilateral TAB (n = 74), those who had unilateral negative TAB (n = 88) had a significantly lower prevalence of jaw claudication (P = 0.007). Compared with subjects diagnosed with GCA (n = 39), those with unilateral negative TAB (n = 88) had significantly lower frequencies of jaw claudication (P = 0.001), "chalky white" optic disc edema (P = 0.002), and fever (P < 0.0001). Compared with subjects with positive TAB (n = 33), subjects with negative TAB (n = 148) had significantly lower prevalence of jaw claudication (P < 0.0001), "chalky white" disc edema (P = 0.0002), pale disc edema (P = 0.006), or any systemic symptom other than headache (P = 0.0002). ("Chalky white" denotes notably extreme disc pallor). The most common indications for biopsy in subjects with unilateral negative TAB were elevated erythrocyte sedimentation rate (ESR) (74%), headache (69%), visual complaints (58%), and ophthalmic signs (52%). Although ESR was a significant predictor of positive TAB overall (unilateral and bilateral TAB) in logistic regression models accounting simultaneously for subject age (P = 0.04), ESR did not significantly predict unilateral negative status in our patients (P = 0.13). CONCLUSIONS: In this cohort of patients, unilateral TAB was associated with an extremely low frequency (1%) of subsequent positive contralateral TAB and was not associated with adverse visual or neurologic outcomes for any subject. We conclude that in the hands of experienced physicians, a unilateral TAB is sufficient to exclude a diagnosis of GCA in populations for which clinical suspicion is low. Jaw claudication, pale optic disc edema, particularly "chalky white" disc edema, fever, or any systemic symptom other than headache should raise suspicion for a diagnosis of GCA.