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June 1966

Paramyotonia Congenita: Association With Cutaneous Cold Sensitivity and Description of Peculiar Sustained Postures After Muscle Contraction

Author Affiliations

From the University of Michigan Medical Center, Department of Neurology, Ann Arbor.

Arch Neurol. 1966;14(6):590-594. doi:10.1001/archneur.1966.00470120022003

MOST investigators now agree that paramyotonia congenita is an hereditary syndrome transmitted as a dominant trait, that it is a specific disorder, unrelated to myotonia congenita or dystrophia myotonnica, and that cold temperatures increase the intensity of the paramyotonic symptoms. In a previous study1 the clinical features of a family with paramyotonia congenita were presented as well as experiments performed in cooperation with an intelligent paramyotonic patient and her father. The conclusions of that study were that the symptoms and signs of paramyotonia congenita resulted from repeated contraction of a muscle rather than from simple exposure of the body to cold (paradoxical myotonia). Although exposure to cold markedly decreased the amount of exertion necessary to produce symptoms, paramyotonic signs could be produced with vigorous exercise after an hour-long exposure in a room at the patient's body temperature, 98.6 F (37.0 C). Moreover, myotonia and weakness did not occur after