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NEUROLOGY 1996;47:956-962
© 1996 American Academy of Neurology

Heat-sensitive myotonia in proximal myotonic myopathy

Howard W. Sander, MD, George P. Tavoulareas, DO and Sudhansu Chokroverty, MD

From the Department of Neurology and the Division of Clinical Neurophysiology, Saint Vincents Hospital and Medical Center of New York, New York Medical College (Drs. Sander, Tavoulareas, and Chokroverty); The Neurology Service, Veterans Affairs Medical Center, Lyons, NJ; and the Department of Neurology, Robert Wood Johnson Medical School, Piscataway, NJ (Dr. Chokroverty).
Presented in part at the 48th annual meeting of the American Academy of Neurology, San Francisco, CA, March 1996.
Received December 5, 1995. Accepted in final form February 20, 1996.
Address correspondence and reprint requests to Dr. Howard W. Sander, Department of Neurology, Saint Vincents Hospital and Medical Center of New York, Cronin 466, 153 West 11th St., New York, NY 10011.

Two siblings with proximal myotonic myopathy (PROMM), a hereditary disorder, had predominantly proximal weakness, pain, and gait impairment aggravated by warm temperatures.EMG of the deltoid showed sparse abnormal spontaneous activity at room temperature and with cooling. Warming induced frequent myotonic discharges and fibrillations. Profuse myotonia recorded at room temperature in the first dorsal interosseous abated following cooling. Repetitive stimulation did not reveal a decrement recording from distal muscles, but recording from the deltoid, in the one patient tested, revealed a significant decrement that did not improve with edrophonium. Myopathic motor units were recorded only in distal musculature. The myotonia of PROMM is provoked by heat and diminished by cold and may have a different physiologic basis than traditional myotonic syndromes.

NEUROLOGY 1996;47: 956-962.




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[Abstract] [Full Text] [PDF]


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