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Department of Neurology, the Neurological Institute, Columbia-Presbyterian Medical Center, New York, NY. (Drs. Lange, Trojaborg, Latov, Younger, Uncini, Blake, Hirano, Burns, Lovelace, and Rowland)
Department of Pathology, the Neurological Institute, Columbia-Presbyterian Medical Center, New York, NY. (Dr. Hays)
We studied 169 patients with motor neuron disease. Seventeen showed abnormal amplitude reduction of the compound muscle action potential. Ten had focal loss of both amplitude and area across a specific segment (conduction block). Eight of the 10 had slowing of conduction across that segment. Nine were men and had prominent hand involvement. Six had probable or definite upper motor neuron signs. Five of the 10 showed immunologic abnormalities (elevated GM1 antibody titers or paraproteinemia), and eight had had symptoms for more than 4 years. Seven of the 17 patients showed loss of amplitude without corresponding loss of area and focal slowing of conduction (temporal dispersion). Five of the seven were men, five had prominent hand involvement, and five had definite or probable upper motor neuron signs. Two had immunologic abnormalities, and only one had had symptoms for longer than 4 years. Among 152 patients with no abnormality of conduction, 64% were men, hands were dominantly involved in 34%, upper motor neuron signs were definite or probable in 72%, and 3% had immunologic abnormalities. None had symptoms for more than 4 years. Because there were so many exceptions, we could not define a unique syndrome by criteria involving conduction block, GM1 antibodies, or lack of upper motor neuron signs. The clinical syndrome associated with multifocal conduction block seemed uniform, however, and patients with conduction block had slower progression if there were no upper motor neuron signs.
Address correspondence and reprint requests to Dr. D.J. Lange, Neurological Institute, 710 West 168th Street, New York, NY 10032–2603.
Supported by Center Grants from NINCDS (RR-0645) and the Eleanor and Lou Gehrig MDA/ALS Research Center.
Received April 19, 1991. Accepted for publication in final form August 13, 1991.
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