Channelopathies of the Nervous System

Recent advances in molecular biology and genetics have changed the face of all medical specialties, with the neurology sector in particular garnering exciting insight into the pathophysiology of many conditions. The authors have compiled a concise collection of material from expert contributors concerning ion channel disorders, or channelopathies, that affect the nervous system.

Channels for sodium, potassium, and calcium transmission have been identified in the membranes of most cells, but only recently has dysfunction of ion transport been correlated with specific clinical conditions. Indeed, as the book's foreword states, the concept of channelopathy did not exist just a few years ago.

Prototype channelopathies with neurologic manifestations include blockage of sodium transport by tetrodotoxin, and antibody-- mediated ion channel blockade in myasthenia gravis. Although first described in several neuromuscular disorders, channelopathies are now recognized as potentially responsible for many other forms of neurologic illness.

The first three sections of the book cover the basic science of ion channels, methods to assess ion channel function, and ion channel gene expression as related to normal development and disease states. Following the introduction, specific neuromuscular diseases are discussed, including the myotonias, the periodic paralyses, malignant hyperthermia, myasthenia gravis, and syndrome. In addition, central nervous system disorders such as periodic/progressive ataxias, epilepsies, and ciguatera poisoning are discussed. Interesting speculation about migraine as a channelopathy is presented, as well as data supporting several other potential channel disorder-related conditions. Increased knowledge of channelopathies will result in better understanding of diseases and novel, more effective therapeutic options.

The authors state that one principal aim of the book is to widen the appreciation of the role of current and potential channel disorders in neurology. I feel they have achieved this aim, and while there are the expected multi-author variances in style, on the whole, the book is well organized and written.

Although the authors maintain that the work was geared specifically for the clinical neurologist and neuroscientist, I primarily recommend it to academic neurologists, neuromuscular disease specialists, and other disciplines with an interest in the channelopathies.