PMID- 31862515 OWN - NLM STAT- MEDLINE DCOM- 20210319 LR - 20210319 IS - 1878-5883 (Electronic) IS - 0022-510X (Linking) VI - 409 DP - 2020 Feb 15 TI - Comparison of clinical and electrophysiological features of patients with hereditary neuropathy with liability to pressure palsies with or without pain. PG - 116629 LID - S0022-510X(19)32394-9 [pii] LID - 10.1016/j.jns.2019.116629 [doi] AB - BACKGROUND: Hereditary neuropathy with liability to pressure palsies (HNPP) is a rare neuropathy with a heterogeneous clinical profile. Painless recurrent palsies are the usual presentation, but neuropathic pain could be predominant or inaugural. Browsing the medical literature, we only found two articles reffering to this important clinical feature. Whether there are differences between patients with or without pain is unclear. The main objective of this study was to compare the clinical and electrophysiological features of these patients and to evaluate the impact on their disability. METHODS: All patients diagnosed with HNPP at the Limoges University Hospital Centre were included and separated into two groups according to the presence or absence of neuropathic pain. In each case, the clinical, genetic, electrodiagnostic, therapeutic features and the modified Rankin Scale (mRS) were evaluated. RESULTS: Out of 23 patients, 52% presented with neuropathic pain. There was no difference between groups regarding to clinical and electrophysiological features, except for the amplitude of the ulnar sensory nerve (p < 0,003). The amplitudes of sensory nerve action potentials (SNAPs) seemed to be higher in patients with pain, but were below the lower limit of normal. Patients with pain had a higher mRS than patients without pain (p < 0,007). CONCLUSION: This study supports previous published results and highlights a trend for higher sensory amplitudes in HNPP patients with pain. We found a prevalence of neuropathic pain of 52% in patients with HNPP, underlining the need to systematically assess pain in such patients in order to improve their management. CI - Copyright (c) 2019 Elsevier B.V. All rights reserved. FAU - Lefour, Sophie AU - Lefour S AD - Department of Neurology, University Hospital Centre of Reims, 45 rue Cognacg Jay, 51100 Reims, France. Electronic address: slefour@chu-reims.fr. FAU - Gallouedec, Gael AU - Gallouedec G AD - Department of Neurophysiology, University Hospital Centre of Limoges, 2 Avenue Martin Luther King, 8700 Limoges, France. Electronic address: gael.gallouedec@chu-limoges.fr. FAU - Magy, Laurent AU - Magy L AD - Department of Neurology, University Hospital Centre of Limoges, 2 Avenue Martin Luther King, 8700 Limoges, France. Electronic address: laurent.magy@unilim.fr. LA - eng PT - Comparative Study PT - Journal Article DEP - 20191216 PL - Netherlands TA - J Neurol Sci JT - Journal of the neurological sciences JID - 0375403 RN - Tomaculous neuropathy SB - IM MH - Adolescent MH - Adult MH - Aged MH - Arthrogryposis/*diagnosis/epidemiology/*physiopathology MH - Child MH - Child, Preschool MH - Cohort Studies MH - Electrophysiological Phenomena/*physiology MH - Female MH - Hereditary Sensory and Motor Neuropathy/*diagnosis/epidemiology/*physiopathology MH - Humans MH - Male MH - Middle Aged MH - Pain/*diagnosis/epidemiology/*physiopathology MH - Retrospective Studies MH - Young Adult OTO - NOTNLM OT - Electrophysiology OT - Hereditary neuropathy with liability to pressure palsies OT - Neuropathic pain OT - PMP22 OT - Polyneuropathy OT - Small fibres COIS- Declaration of Competing Interest Authors have no conflict of interest to disclosure. EDAT- 2019/12/22 06:00 MHDA- 2021/03/20 06:00 CRDT- 2019/12/22 06:00 PHST- 2019/04/15 00:00 [received] PHST- 2019/11/24 00:00 [revised] PHST- 2019/12/10 00:00 [accepted] PHST- 2019/12/22 06:00 [pubmed] PHST- 2021/03/20 06:00 [medline] PHST- 2019/12/22 06:00 [entrez] AID - S0022-510X(19)32394-9 [pii] AID - 10.1016/j.jns.2019.116629 [doi] PST - ppublish SO - J Neurol Sci. 2020 Feb 15;409:116629. doi: 10.1016/j.jns.2019.116629. Epub 2019 Dec 16.