[Comparison of electrophysiological findings between CIDP and HMSN-1]

Chronic inflammatory demyelinating polyneuropathy (CIDP) and hereditary motor sensory neuropathy type 1 (HMSN-1) are representative myelinopathies. In order to differentiate changes in acquired and congenital demyelinating neuropathies, we studied electrophysiologically 9 patients with active phase of CIDP (36.0 +/- 17.6 years old; mean +/- SD) and 7 patients with genetically-proven HMSN-1 A (56.0 +/- 13.6 years old). Motor conduction studies demonstrated longitudinal uniformity in HMSN-1, contrariwise focal conduction block or conduction delay in CIDP. The mean median nerve conduction velocity in the forearm segment and the mean CMAP amplitude stimulated at the wrist were not different between CIDP and HMSN-1 group; 31.8 +/- 7.2 m/sec and 5.6 +/- 2.8 mV in CIDP, and 26.7 +/- 9.8 m/sec and 3.2 +/- 2.6 mV in HMSN-1, respectively. Upper extremity polyneuropathy index (PNI), a mean percentage of normal for 6 indices concerning to the velocity and latency over two nerves obtained by motor conduction studies, was equal and around 50% on the average in each group. Conduction blocks were presented in 7 patients with CIDP and only one patient with HMSN-1. No sensory nerve action potential was recorded in 6 out of 9 patients with CIDP, and in 6 out of 7 patients with HMSN-1. Intrafascicular neurography of the median nerve, stimulated at the wrist and recorded from intrafascicularly inserted microelectrode at the elbow, revealed irregular multiphasic waves which signify severe temporal dispersion. Maximum conduction velocity was similarly reduced to 48 m/sec in CIDP and 44 m/sec in HMSN-1 on the average, but in one patient with HMSN-1 it was maintained to 63 m/sec with conspicuous temporal dispersion of the waveform. Amplitude of the compound nerve action potential (CNAP) decreased more (p < 0.01) in HMSN-1 (26 +/- 11 micro V) than in CIDP (72 +/- 25 micro V). Temporal dispersion of CNAP was prominent in HMSN-1 than in CIDP. In conclusion, electrophysiological changes were more homogeneous in the longitudinal distribution but more heterogeneous in the cross-sectional distribution in HMSN-1 than in CIDP.