A CASE-CONTROL AND NERVE BIOPSY STUDY OF CREST MULTIPLE MONONEUROPATHY

From 536 patients with the CREST syndrome (calcinosis, Raynaud's pheno menon, esophageal dysmotility, sclerodactyly, and telangiectasis), sev en were identified as having peripheral neuropathy not attributable to another cause. Peripheral neuropathy developed 0 to 25 years after th eir first symptoms of scleroderma. Unexplained neuropathy in CREST pat ients (seven patients) was more frequent than in control subjects (two patients) matched for age, sex, time of evaluation, and geographic re ferral region. Multiple mononeuropathy occurred significantly more fre quently in the CREST group (six patients) than in the control group (0 patients). Four sural nerve biopsy specimens from the CREST patients demonstrated multifocal fiber loss and perivascular inflammation; one was diagnostic for necrotizing vasculitis and two others were highly s uggestive for necrotizing vasculitis. The density of myelinated fibers in three nerves from CREST patients was significantly decreased, wher eas the index of dispersion (a measure of multifocal fiber loss) was i ncreased, and the frequency of axonal degeneration was significantly i ncreased. Based on these clinical and pathologic findings, we conclude that in the CREST syndrome multiple mononeuropathy, although occurrin g infrequently, occurs more frequently than by chance and necrotizing vasculitis is the cause of this multiple mononeuropathy.