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.