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MYATONIA CONGENITA: A REPORT OF A CASE WITH AUTOPSY

W. T. COUNCILMAN, M.D.; CHARLES HUNTER DUNN, M.D.
Am J Dis Child. 1911;II(5):340-355. doi:10.1001/archpedi.1911.04100110049006.
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Oppenheim1 described in 1900 a remarkable disease in children the chief symptom in which is a hypotonia or even atonia of the muscles, combined with a corresponding diminution of the tendon reflexes. There is limitation of active movement varying in degree. In the most marked cases the extremities lie motionless and apparently paralyzed. On close observation contractions may be brought out in some or many of the muscles, but these are weak and without locomotor effect. In one of his cases the affection involved not only the muscles of the extremities, but those if the neck and trunk also. The condition is always more marked in the lower than in the upper extremities and the muscles supplied by the cranial nerves are not affected. Electrical stimulation shows a quantitative diminution of contractility up to its complete disappearance. There is no reaction of degeneration and no loss of sensibility. The joints

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