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Abstract
Thirteen patients with congenital insensitivity to pain and anhidrosis, carrying a
mutation at the TRK-A gene, were studied. Neurologic examination revealed vestigial
pain sensitivity, suggesting an incomplete involvement of the affected nerves. All
13 patients manifested normal electrophysiologic studies but striking absence of sympathetic
skin responses. We suggest the use of the sympathetic skin response test in the clinical
evaluation of patients suspected of having congenital insensitivity to pain and anhidrosis.