Pharmaceutical treatment can be inadequate, non-effective, or intolerable for many people suffering from a neuronal channelopathy. Development of novel treatment options, particularly those with the potential to be curative is warranted. Gene therapy approaches can permit cell-specific modification of neuronal and circuit excitability and have been investigated experimentally as a therapy for n...

Mendelian heritable pain disorders have provided insights into human pain mechanisms and suggested new analgesic drug targets. Interestingly, many of the heritable monogenic pain disorders have been mapped to mutations in genes encoding ion channels. Studies in transgenic mice have also implicated many ion channels in damage sensing and pain modulation. It seems likely that aberrant peripheral ...

Objective: To assess neuromuscular transmission (NMT) in patients with Meniere's disease (MD), in which the precise etiology is unknown and a channelopathy hypothesis has been proposed Methods: Single fiber electromyography (SFEMG) during voluntary contraction of the extansor digitorum communis muscle, nerve conduction studies of upper and lower extremities and concentric needle electromyograph...

OBJECTIVE The role of electrophysiology study in Brugada syndrome (BS) sudden cardiac death risk stratification remains controversial and seems to depend on the phenotypic expression of the channelopathy. Ajmaline has a key role in the diagnosis of BS. We observed that programmed electrical stimulation (PES) of the right ventricular outflow tract (RVOT), only when type 1 BS ECG is unmasked by a...

Ion channelopathy is a proven cause of inherited human epilepsy, and may play a role in acquired epileptic syndromes as well. Of the many ion channel causes of epilepsy, the h-channel is a potential new addition. H-channels are voltage-gated ion channels with unique biophysical properties. The h-channel exerts a significant modulatory influence on neuronal excitability, and is a target of antie...