Hereditary neuropathy with liability to pressure palsy in patients under 30 years old: Neurophysiological data and proposed electrodiagnostic criteria. Issue 2 (11th June 2017)

• Record Type: Journal Article
• Authors: Robert‐Varvat, Florence; Jousserand, Guillemette; Bouhour, Françoise; Vial, Christophe; Cintas, Pascal; Echaniz‐Laguna, Andoni; Delmont, Emilien; Clavelou, Pierre; Chauplannaz, Guy; Jomir, Laurent; Pereon, Yann; Leonard‐Louis, Sarah; Manel, Veronique; Antoine, Jean‐Christophe; Lacour, Arnaud; Camdessanche, Jean‐Philippe
• Is Part Of: Muscle & nerve. Volume 57:Issue 2(2018)
• Page Start: 217
• Page End: 221
• Publication Date: 2017-06-11
• Subjects: criteria -- hereditary neuropathy -- hereditary neuropathy with liability to pressure palsy -- neurophysiology -- PMP22 -- young people; Neuromuscular diseases -- Periodicals; Muscles -- Periodicals; Nerves -- Periodicals; 616.74
• DOI: 10.1002/mus.25666
• Languages: English
• ISSNs: 0148-639X

Abstract

ABSTRACT: Introduction : In young patients with mononeuropathy who lack family history and precipitating factors, hereditary neuropathy with liability to pressure palsy (HNPP) may be a possibility. Our objective is to propose neurophysiological criteria for HNPP in patients <30 years of age. Methods : We conducted a national multicenter retrospective clinical and neurophysiological study in patients under 30 with genetically confirmed HNPP. Results : All of the 51 patients included in the study had at least 1 demyelinating pattern in 2 asymptomatic nerves, and 3 abnormalities were found in almost 90%, including slowed motor nerve conduction velocity across the elbow in at least 1 ulnar nerve (97.5%), increased distal motor latency (DML) in at least 1 fibular nerve (95.8%), and increased DML in both median nerves (89%). Age influenced DML slightly only in the fibular nerve. Discussion : Dissemination of nerve involvement in HNPP incites to perform a complete nerve conduction study. including bilateral ulnar, fibular, and median nerves. Muscle Nerve 57 : 217–221, 2018