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• Vol.45 No.2
• CLINICA NEUROL, 45: 117−120, 2005
• Vol.45 No.2 contents

Brief Clinical Note

Perception of dyspnea due to breath-holding in myotonic dystrophy

Yasuo Murakami, M.D., Yasushi Oya, M.D., Masafumi Ogawa, M.D. and Mitsuru Kawai, M.D.

Department of Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry (NCNP)

Patients with myotonic dystrophy (DM1) rarely complain of dyspnea despite of severe hypoxemia. We studied the perception of dyspnea caused by breath-holding in 9 DM1 patients and 8 healthy control subjects. The patients, as well as the control subjects, complained of dyspnea and showed decrease in SpO₂. In none of the patients but one, however, the bottom SpO₂ became lower than the minimal SpO₂ recorded in 24-hour monitoring. DM1 patients were able to realize hypoxia caused by apnea, although they had not realized hypoxia that already existed. Consequently, the breath-holding test does not uncover a blunted perception of dyspnea in DM1; an afferent system contributable to air hunger sensation in breath-holding is preserved in DM1. Breath-holding test may be useful for a DM1 patient to recognize the significance of sleep apnea.

(CLINICA NEUROL, 45: 117−120, 2005)
key words: myotonic dystrophy, breath-holding, hypoxemia, perception of dyspnea

(Received: 26-Jan-04)