This early report identifies myopathic involvement of the heart muscle in progressive muscular dystrophy, explaining the sudden cardiovascular deterioration observed in these patients.
May explain sudden cardiac deaths in progressive muscular dystrophy; challenges prior doubts and leaves open need for targeted cardiac studies.
While observing the clinical course of several cases of progressive muscular dystrophy, my attention was called to the manner in which patients who had remained in statu quo for fairly long periods of time, would, without warning, suddenly develop in rapid succession hypostatic pneumonia, pulmonary edema, hydropericardium and hydrothorax, culminating in death. Cardiovascular disturbance seemed best to explain these phenomena, and in two cases reported by Goodhart and myself,¹lesions were found in the heart muscle, essentially of the same character as those found in the skeletal muscles. In remarkably few cases of pseudohypertrophic muscular atrophy has a study of the heart muscle been made. Oppenheim,²referring to the few cases recorded,³without giving reason for his opinion, says that he considers myopathic involvement of the heart muscle doubtful. Marinesco,³in his splendid monograph on the disease of muscles, devotes a few paragraphs to the changes
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JOSEPH H. GLOBUS (1923) studied this question.
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