ADYNAMIA EPISODICA HEREDITARIA AND MYOTONIA

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Abstract
Two families are reported with myotonia and attacks of weakness. In one of them symptoms and signs are in agreement with the original description of paramyotonia congenita. A test dose of a potassium salt increases myotonia but does not elicit weakness. The other family fulfills the diagnostic criteria of adynamia episodica hereditaria and also has myotonia of the type seen in Thomsen''s disease. The relationship between different myotonic syndromes is discussed. It is suggested that pure myotonia congenita and adynamia episodica hereditaria without myotonia represent the 2 ends of a chain. The links between them consist of conditions characterized by weakness and myotonia, including paramyotonia congenita von Eulenburg. For patients reacting with weakness and increasing serum potassium to a small dose of potassium the name of adynamic episodica hereditaria should be retained regardless of the presence of myotonia because of the risk that they may be misjudged as cases of periodic paralysis and treated with potassium with serious results.

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