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Establishing a Mouse Model of a Pure Small Fiber Neuropathy with the Ultrapotent Agonist of Transient Receptor Potential Vanilloid Type 1
Published on: February 13, 2018
Peripheral neuropathy in scleroderma
Ann N Poncelet1, M Kari Connolly
1Department of Neurology, Box 0114, University of California, San Francisco, San Francisco, California 94143, USA. ponce@itsa.ucsf.edu
Abstract:
Because patients with scleroderma report neuropathic symptoms including numbness, paresthesias, and dysesthesias, we assessed peripheral nerve function in such patients. Fourteen scleroderma patients underwent complete neurologic examination, nerve conduction studies (NCS) and quantitative sensory testing (QST). Neurologic examination revealed reduced vibration (7) or pinprick (4) sensation in the upper or lower extremities, focal atrophy or proximal weakness (2), and decreased deep tendon reflexes (2). NCS showed reduced sensory nerve action potentials (1) and carpal tunnel syndrome (1). QST of the upper and lower extremity revealed increased cold or vibration detection thresholds in 8 of 14 patients. Our findings suggest that peripheral neuropathy occurs in patients with scleroderma at a higher frequency than previously appreciated. These findings cannot be ascribed to compression neuropathies, but rather involve large and small fibers in a non-length-dependent fashion. Larger, prospective studies using the more sensitive QST as well as pathologic studies of nerve, including cutaneous innervation, are needed to further assess the characteristics and etiology of the neuropathy.
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