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  • Title: Electrodiagnostic evidence of phrenic nerve demyelination in Charcot-Marie-Tooth disease 1A.
    Author: Sagliocco L, Orlandi G, Calabrese R, Pellegrinetti A, Baglini O, Castelli F, Baldinotti F, Sartucci F.
    Journal: Am J Phys Med Rehabil; 2003 Oct; 82(10):754-9. PubMed ID: 14508405.
    Abstract:
    OBJECTIVE: To determine whether neurophysiologic findings correlate to clinical respiratory signs or spirometric abnormalities in patients with hereditary motor and sensory neuropathy type 1 (Charcot-Marie-Tooth disease). DESIGN: A total of 11 patients with hereditary motor and sensory neuropathy type 1A, genetically identified, (age range, 10-58 yr) were included and studied by physical pulmonary examination, chest radiography, respiratory function tests, and bilateral transcutaneous phrenic nerve conduction. RESULTS: No patient complained of respiratory symptoms or revealed abnormal spirometric or maximal respiratory pressure data, despite a phrenic nerve conduction significantly slower (P < 0.0001; median conduction time, 18.6 msec; 95th percentile, 31.97 msec) than that recorded in the control group of healthy subjects (median, 6.05 msec; 95th percentile, 8.82 msec); the amplitudes of compound muscle action potentials were not statistically different from the controls. CONCLUSIONS: Our study confirms a dramatic phrenic nerve involvement in absence of clinical and laboratory evidence of diaphragmatic weakness; further studies and an adequate follow-up are necessary to discover whether the disease progress might encompass respiratory dysfunction at later stages.
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