• Sophie Ng Wing Tin, Violaine Planté-Bordeneuve, Hayet Salhi, Colette Goujon, Thibaud Damy, and Jean-Pascal Lefaucheur.
• Université Paris Est Créteil, Faculté de Médecine, EA 4391, Créteil, France; Assistance Publique-Hôpitaux de Paris, Hôpital Avicenne, Service de Physiologie, Explorations Fonctionnelles et Médecine du Sport, Bobigny, France.
• J Pain. 2015 Nov 1; 16 (11): 1106-14.

UnlabelledFamilial amyloid polyneuropathy (FAP) caused by transthyretin (TTR) mutation is a small-fiber predominant polyneuropathy, exposing patients with TTR-FAP to development of neuropathic pain. However, the painful nature of TTR-FAP has never been specifically addressed. In this study, we compared 2 groups of 16 patients with either painless or painful TTR-FAP with regard to various clinical and neurophysiologic variables, including laser evoked potential (LEP) recording and quantitative sensory testing. The 2 groups of patients did not differ on any clinical or neurophysiologic variable. Patients with painful TTR-FAP complained of ongoing burning pain sensations, pain aggravation at rest, paroxysmal pain (electric shock and stabbing sensations), or provoked pain (mostly dynamic mechanical allodynia). However, the symptomatic presentation of painful TTR-FAP evolved with the course of the disease. The duration of the disease and the severity of small-fiber lesions (increase in thermal thresholds and reduction in LEP amplitude) correlated negatively with the intensity of ongoing burning sensations and positively with the intensity of paroxysmal pain. In addition, small-fiber preservation correlated positively with cold allodynia and pain aggravation at rest and negatively with dynamic mechanical allodynia. Peripheral sensitization of small-diameter nociceptive axons might occur in early TTR-FAP and be responsible for the burning sensation and cold allodynia. As polyneuropathy and small-fiber loss progress, paroxysmal pain and dynamic mechanical allodynia may develop as a result of central sensitization generated by abnormal activities affecting relatively spared large-diameter sensory fibers.PerspectivePain in TTR-FAP includes several mechanisms varying with the course of the disease and the involvement of the different types of nerve fibers.Copyright © 2015 American Pain Society. Published by Elsevier Inc. All rights reserved.

37 keywords...

hide…