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To the Editor.—
Early manifestations of stiff-man syndrome are likely to be seen by neurologists. The ultimate fate of such patients has received little attention in the literature. However, all six of the reported autopsies, and two others I have obtained, ascribe death to respiratory failure.In the past 26 years, I have followed up four cases to death (one I called chronic tetanus) and am watching the clinical status decline in six more. It has been my experience, and this is supported by the literature, that the management of the ultimate miseries of these patients is transferred to respiratory specialists or psychiatrists or both. My occasional continuing involvement has been in the role of a stubbornly curious, but not very helpful, scorekeeper.The literature of pulmonary function studies acknowledges shortcomings in the measurement of defects of "total respiratory apparatus" when chest-wall mobility and lack of muscular relaxation interfere with
Campbell JR. Stiff-man Syndrome and Emphysema. Arch Neurol. 1979;36(6):387–388. doi:10.1001/archneur.1979.00500420097020
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