title: Pediatric median neuropathy due to pruritus in Alagille syndrome.
abstract: Median entrapment neuropathy or carpal tunnel syndrome is uncommon in children. The majority of cases are related to genetic conditions which result in skeletal dysplasia or altered connective tissue characteristics, direct injury to the median nerve caused by intensive sports or trauma, or hereditary neuropathy with liability to pressure palsies. This report describes a 10-year-old patient with Alagille syndrome who presented with poor fine motor skills because of an entrapment neuropathy of the median nerve at the wrist. This condition was probably caused by intermittent external compression at the wrists due to years of rubbing both wrists and hands to relieve pruritus. To our knowledge, median neuropathy has never been associated with Alagille syndrome, although severe pruritus is considered a major symptom and many patients exhibit widespread scratching and rubbing.
authors.affiliation: Department of Neurology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. n.voermans@neuro.umcn.nl
authors.forename: Nicol C
authors.initials: NC
authors.lastname: Voermans
authors.forename: Nens
authors.initials: N
authors.lastname: van Alfen
authors.forename: Jules J M
authors.initials: JJ
authors.lastname: Tolboom
authors.forename: Alfred C
authors.initials: AC
authors.lastname: Koetsveld
authors.forename: Lilian T L
authors.initials: LT
authors.lastname: Sie
mesh_terms: D016738:Alagille Syndrome / Q000150:complications*; D002349:Carpal Tunnel Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D002648:Child; D006801:Humans; D008297:Male; D011537:Pruritus / Q000209:etiology / Q000523:psychology*; D016728:Self-Injurious Behavior / Q000150:complications*