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Article
February 1965

Hyperkalemic Periodic Paralysis: A Genetic Study, Clinical Observations, and Report of a New Method of Therapy

Arch Neurol. 1965;12(2):145-154. doi:10.1001/archneur.1965.00460260035004
Abstract

IN 1886, Eulenberg1 described a disease characterized by periodic attacks of myotonia and paralysis, occurring in 26 members (six generations) of one family.1 The disease was called paramyotonia. The following criteria for its diagnosis were proposed by Drager et al2: (1) single autosomal dominant inheritance, (2) stationary (nonprogressive) character, (3) presentation of normal muscle bulk (no atrophy, no hypertrophy), (4) presence of myotonia, particularly in the cold, (5) episodes of flaccid weakness. Since Eulenberg's original account, there have been approximately 38 published communications of similar cases (Table 1).1-22 This disease was distinguished from familial periodic paralysis (later found to be associated with hypokalemia) and from congenital myotonia (Thomsen's disease).

In 1956, Gamstorp5 presented 139 cases of an unusual variety of periodic paralysis in which the potassium levels in the serum during paresis were elevated, rather than depressed; and in fact, muscular weakness was precipitated

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