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From the Neuromuscular Diseases Section (M.S., M.C.D.), National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD; and the Center for Drug Evaluation and Research (W.W.B.), U.S. Food and Drug Administration, Rockville, MD.
Address correspondence and reprint requests to Dr. Marinos C. Dalakas, NDS/NINDS/NIH, Building 10, Room 4N248, 10 Center Drive, Bethesda, MD 20892; e-mail: dalakasm{at}ninds.nih.gov.
Four patients with postpolio syndrome (PPS) developed ALS. Weakness and atrophy started from previously unaffected extremities but, contrary to PPS, spread to all muscles leading to death within 0.4 to 8 (mean 3.9) years. Upper motor neuron signs were absent in the atrophic limbs. Abundant spontaneous activity and group atrophy in newly affected muscles were prominent. ALS can rarely occur in the postpolio population starting de novo rather than as evolution of PPS.
This research was supported by the Intramural Research Program of the NIH, National Institute of Neurologic Disorders and Stroke.
Disclosure: The authors report no conflicts of interest
Received December 23, 2005. Accepted in final form May 10, 2006.
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