1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-22-93-b 10.15844/pedneurbriefs-22-12-7 Neuromuscular Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Hand Involvement in Charcot-Marie-Tooth Disease 1A http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 12 2008 01 02 2016 22 12 93 94 Copyright: © 2008 The Author(s) 2008 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Hand involvement in children with Charcot-Marie-Tooth disease type 1A

Hand strength, function and disease-related symptoms were determined in 84 children, aged 2-16 years, with Charcot-Marie-Tooth disease type 1A (CMT1A) at University of Sydney, Children’s Hospital at Westmead, and Royal Children’s Hospital, Parkville, Australia.

 Charcot-Marie-Tooth Disease Type 1A Hand Weakness Demyelinating Neuropathy

Hand strength, function and disease-related symptoms were determined in 84 children, aged 2-16 years, with Charcot-Marie-Tooth disease type 1A (CMT1A) at University of Sydney, Children’s Hospital at Westmead, and Royal Children’s Hospital, Parkville, Australia. Hand weakness and dysfunction was present from the earliest stages of the disease and tended to worsen with age throughout childhood. Poor handwriting, weakness, pain and sensory symptoms also worsened with age. [1]

COMMENT. CMT1A is a demyelinating neuropathy characterized by progressive muscle weakness and atrophy. The peroneal muscles are involved first, causing a striking stork-like gait, and weakness and atrophy of the upper extremities is initially limited to the intrinsic muscles of the hands. The authors comment that the hand involvement is frequently under-recognized in the early stages.

Burns J Bray P Cross LA North KN Ryan MM Ouvrier RA Hand involvement in children with Charcot-Marie-Tooth disease type 1A Neuromuscul Disord 2008 Dec 18 12 970 3 10.1016/j.nmd.2008.08.004 18993073