Evaluation and management of upper extremity neuropathies in Charcot-Marie-Tooth disease

The evaluation and treatment of five patients with upper extremity neuropathies secondary to Charcot-Marie-Tooth disease were reviewed with emphasis on age at onset of Charcot-Marie-Tooth disease and upper extremity deformities, clinical findings, signs of associated nerve compression, and outcome of surgical treatment. The onset of the disease generally occurred in the first or second decade of life. The onset of upper extremity symptoms lagged behind by an average of 8 years. All patients had intrinsic minus hands with decreased sensibility. Three of five patients had clinical or electrophysiologic evidence of associated nerve compression syndromes. Treatment with standard tendon transfers, nerve compression releases, soft tissue releases, and joint fusions resulted in subjectively improved function in three of four patients undergoing reconstruction. Release of six compression neuropathies in one patient provided excellent pain relief, but the underlying neuropathy progressed. Pessimism regarding reconstructive surgery in the patient with upper extremity neuropathies secondary to Charcot-Marie-Tooth disease is unwarranted.