Hereditary neuropathy with liability to pressure palsy in patients under 30 years old: Neurophysiological data and proposed electrodiagnostic criteria

• Published in: Muscle & Nerve
• Main 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
• Format: Article in Journal/Newspaper
• Language: English
• Published: Wiley 2017
• Subjects: Physiology (medical); Cellular and Molecular Neuroscience; Neurology (clinical); Physiology; DML
• Online Access: http://dx.doi.org/10.1002/mus.25666; https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1002%2Fmus.25666; https://onlinelibrary.wiley.com/doi/pdf/10.1002/mus.25666

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