Multiple measures of nerve excitability have been developed to appraise axonal membrane properties in peripheral nerves and are contributing to better understand neurological disorders. These techniques have been adapted to transgenic mouse models. The purpose of this study was to characterize the excitability properties along disease progression in three mouse models of motor neuron disease (MND): i) the SOD1G93A model of amyotrophic lateral sclerosis ii) the late-onset and slowly progressing model of Kennedy disease (KD) iii) a new potential model of MND: the abnormal rear leg (Arl) mice. As in human studies, the multiple excitability program recorded stimulus-response, strength- duration, current-threshold relationships, threshold electrotonus, and recovery cycle. Each model displayed impaired nerve excitability properties compared to normal mice. In the SOD1G93A, the abnormalities occurred early in disease, before the onset of motor neuron degeneration and did not further deteriorate as the disease progressed. Moreover, changes were different from those reported in patients suggesting that the alterations observed in patients may rely on a process specific to the human disease and not well modeled in mice. In the KD model, the abnormalities were different from the SOD1G93A. They occurred concomitantly with motor neuron degeneration and partially mimicked the changes observed in KD patients. The Arl mice displayed different abnormalities, mostly related to a failure for motor neurons to mature. To conclude, three models were explored. They can be distinguished by the type of abnormalities, the parameters involved and by the timing of occurrence.