Abstract
Nerve excitability studies have emerged as a recent novel non-invasive technique that offers complementary information to that provided by more conventional nerve conduction studies, the latter which provide only limited indices of peripheral nerve function. Such novel tools allow for the assessment of peripheral axonal biophysical properties that include ion channels, energy-dependent pumps and membrane potential in health and disease. With improvements in technique and development of protocols, a typical study can be completed in a short period of time and rapid measurement of multiple excitability indices can be achieved that provide insight into different aspects of peripheral nerve function. The advent of automated protocols for the assessment of nerve excitability has promoted their use in previous studies investigating disease pathophysiology such as in metabolic, toxic and demyelinating neuropathies, amyotrophic lateral sclerosis, stroke, spinal cord injury and inherited channelopathies. In more recent years, the use of nerve excitability studies have additionally provided insights into the pathophysiological mechanisms underlying cerebellar disorders that include stroke and familial cerebellar ataxias such as episodic ataxia types 1 and 2. Moreover, this technique may have diagnostic and therapeutic implications that may encompass a broader range of neurodegenerative cerebellar ataxias in years to come. In the foreseeable future, this technique may eventually be incorporated into clinical practice expanding the currently available armamentarium to the neurophysiologist.