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  • Hereditary neuropathy with ...
    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

    Muscle & nerve, February 2018, 2018-02-00, 20180201, Letnik: 57, Številka: 2
    Journal Article

    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