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From the Department of Neurology (Drs. Collins, Mendell, Periquet, Sahenk, and Kissel), The Ohio State University College of Medicine, Columbus; Department of Neurology (Dr. Amato), Brigham and Women’s Hospital, Boston, MA; Division of Neurology (Dr. Jackson), Department of Medicine, University of Texas Health Science Center at San Antonio; Department of Neurology (Dr. Gronseth), Wilford Hall Medical Center, Lackland AFB, TX; and Department of Neurology (Dr. Barohn), University of Texas Southwestern Medical Center at Dallas.
Address correspondence and reprint requests to Michael P. Collins, Department of Neurology, The Ohio State University College of Medicine, 4th Floor Means Hall, 1654 Upham Drive, Columbus, OH 43210; e-mail: collins-6{at}medctr.osu.edu
OBJECTIVE: To determine the sensitivity and specificity of superficial peroneal nerve (SPN)/peroneus brevis muscle (PBM) biopsy in a cohort of patients with suspected peripheral nerve vasculitis.
BACKGROUND: In patients with suspected vasculitic neuropathy, combined nerve and muscle biopsies have been advocated as a way to increase the diagnostic yield, but the sensitivity and specificity of this approach have not been evaluated. Pathologic predictors of biopsy-proven peripheral nerve vasculitis have also not been analyzed in a systematic fashion.
METHODS: The clinical, laboratory, and pathologic data for all patients undergoing SPN/PBM biopsy for possible vasculitis from 1986 through 1996 were analyzed. Biopsies were classified as positive, negative, or suspicious for vasculitis. Patients were then divided into vasculitis and nonvasculitis cohorts by final clinical diagnosis.
RESULTS: Of 70 SPN/PBM biopsies, 22 (30%) showed definite vasculitis; nerve was diagnostic in 90% (n = 20) and muscle in 50% (n = 11). Nerve biopsy had a higher yield than muscle in patients with nonsystemic vasculitic neuropathy (p = 0.0047) but not in those with systemic vasculitis. The estimated sensitivity of a positive SPN/PBM biopsy for vasculitis was 60%. Considering biopsies either positive or suspicious for vasculitis increased the sensitivity to 86% with a corresponding specificity of 85%. Pathologic features associated with necrotizing vasculitis were muscle fiber necrosis/regeneration (relative risk 18.1; 95% CI 3.4 to 96.1), predominant axonal nerve pathology (>8.8; >1.0 to 77.4), Wallerian-like degeneration (5.6; 1.4 to 21.9), and asymmetric nerve fiber loss (4.6; 1.4 to 15.9).
CONCLUSIONS: These findings establish the yield, sensitivity, and specificity of SPN/PBM biopsy for diagnosing vasculitic neuropathy and validate the use of suggestive pathologic features for diagnosing cases lacking definite necrotizing vascular changes.
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