Myopathy of Hypokalemic Periodic Paralysis An Electron Microscopic Study

EREDITARY intermittent flaccid paralysis of skeletal muscle is at present classified into three types on the basis of the changes in the serum potassium during the paralytic attack. The hypokalemic form was the first to be clinically recognized, and is the most common of the three variants, more than 600 cases having been described in the literature by 1959.1 The clinical manifestations of this disease will not be detailed here as many reviews are available.2-5 Worthy of emphasis, however, is that permanent proximal muscle weakness may occur. This feature was first noted by Oppenheim6 in 1891 and was later thought to be a variant of spinal muscular atrophy occurring in conjunction with periodic paralysis.7 However, it has now become apparent that permanent myopathic weakness is not uncommon, and not dependent upon severe or repeated paralytic attacks.

Please continue Here: http://archneur.jamanetwork.com/article.aspx?articleid=568933