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Q J Med 2000; 93: 787-797
© 2000 Association of Physicians

Review

Neurological potassium channelopathies

M. Benatar

From the Beth Israel Deaconess Medical Center, Boston, USA

Potassium channel dysfunction has been implicated in a variety of genetic and acquired neurological disorders that are collectively referred to as the potassium channelopathies. These include acquired neuromyotonia, episodic ataxia type-1, hereditary deafness syndromes, benign familial neonatal convulsions and hypokalaemic periodic paralysis. Insight into potassium channel structure and function is crucial to understanding the pathophysiology of these conditions. This article describes potassium channel structure and function and then outlines what is known about the immunology and genetics of the neurological potassium channelopathies.

Address correspondence to Dr M. Benatar, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA 02215, USA

a The terms ‘neuromyotonia’ and ‘myokymia’ are also used to refer to specific electromyographic patterns and are more accurately referred to as neuromyotonic and myokymic discharges. The former refers to a burst of motor unit action potentials firing at 150–300 Hz for several seconds, often starting and stopping abruptly and waning in amplitude. In contrast, a myokymic discharge represents the spontaneous firing of single motor units as doublet, triplet or multiplet discharges at irregular intervals.


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