Hereditary hypertrophic neuropathy in the Trembler mouse: electrophysiological studies

A study was undertaken to examine whether the electrophysiological findings in experimental acute demyelination apply also to a naturally occurring chronic demyelinating neuropathy. Detailed electrophysiological studies were conducted in the peripheral nerves of Trembler mice who suffer from a dominantly inherited, naturally occurring chronic hypertrophic neuropathy. Motor conduction velocities were studied on the sciatic, median and tail nerves of control and Trembler mice. In another series of experiments the refractory period of the sciatic tibial nerve trunk, its ability to conduct rapid trains of stimuli and the effect of temperature on conduction were studied. It was found that the amplitude of muscle action potential and the motor conduction velocity in the Trembler mice were below the control range. The peripheral nerves of the Trembler mice did not also conduct trains of impulses and underwent conduction block at stimulus frequencies and temperatures that were well within the physiological range. These findings are similar to the observations made in experimental acute demyelinating neuropathies. (Ramamurthi - Madras)