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Hereditary neuropathy with liability to pressure palsy in patients under 30 years old: Neurophysiological data and proposed electrodiagnostic criteria
Author(s) -
RobertVarvat Florence,
Jousserand Guillemette,
Bouhour Françoise,
Vial Christophe,
Cintas Pascal,
EchanizLaguna Andoni,
Delmont Emilien,
Clavelou Pierre,
Chauplannaz Guy,
Jomir Laurent,
Pereon Yann,
LeonardLouis Sarah,
Manel Veronique,
Antoine JeanChristophe,
Lacour Arnaud,
Camdessanche JeanPhilippe
Publication year - 2018
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.25666
Subject(s) - medicine , mononeuropathy , ulnar nerve , palsy , nerve conduction velocity , asymptomatic , elbow , electromyography , neurophysiology , motor nerve , median nerve , surgery , anesthesia , peripheral neuropathy , physical medicine and rehabilitation , anatomy , pathology , diabetes mellitus , alternative medicine , endocrinology , psychiatry
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