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Hereditary neuropathy with liability to pressure palsy

The electrophysiology fits the name

From the Department of Neurology (Drs. Li, Shy, and Lewis, and K. Krajewski) and Center for Molecular Medicine and Genetics (K. Krajewski and Dr. Shy), Wayne State University School of Medicine, Detroit, MI.

Address correspondence and reprint requests to Dr. Jun Li, Department of Neurology, Wayne State University, 4201 St. Antoine, UHC-8D, Detroit, MI 48201; e-mail: junli{at}med.wayne.edu

Background: Studies of patients with hereditary neuropathy with liability to pressure palsies (HNPP) have shown accentuated distal slowing along with nonuniform conduction abnormalities at segments liable to compression, suggesting a distal myelinopathy as an underlying pathophysiological mechanism.

Methods: We evaluated 12 patients with HNPP by standard nerve conduction studies and by conduction to more proximal muscles in the arm and leg. Three CMT1A patients and six healthy subjects also were evaluated as controls.

Results: Median and peroneal motor nerves in all HNPP patients showed prolonged distal motor latencies (DML) (mean ± SE, 5.9 ± 0.41 and 8.63 ± 0.58 milliseconds), but the ulnar and tibial DML were minimally prolonged or normal (mean ± SE, 3.87 ± 0.16 and 5.66 ± 0.24 milliseconds). DML to forearm flexor (median and ulnar nerves) or anterior tibial muscles (peroneal nerve) were also normal.

Conclusion: Accentuated distal slowing is found primarily in median and peroneal nerve segments liable to pressure palsies or repetitive trauma. However, the ulnar and tibial nerves, which are less liable to compression, have minimal changes. In addition, distal latencies to more proximal muscles in the arm and leg do not have distal slowing. These findings do not support a distal myelinopathy as a determinant of the conduction abnormalities in HNPP.

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