Primary lateral sclerosis. A clinical diagnosis reemerges
D. S. Younger, S. Chou, A. P. Hays, D. J. Lange, R. Emerson, M. Brin, H. Thompson Jr and L. P. Rowland
Department of Neurology, Columbia Presbyterian Medical Center, New York 10032-3784.
Adults with slowly progressive noninherited gait disorders may show no
abnormalities on examination other than signs implicating the corticospinal
tracts. That is the syndrome of "primary lateral sclerosis" (PLS), a
clinical diagnosis that has been avoided because it is a diagnosis of
exclusion, proven only at autopsy. Now, modern technology can exclude other
disorders that can cause the syndrome with an accuracy of about 95%. That
serves to eliminate the following: compressive lesions at the foramen
magnum or cervical spinal cord, multiple sclerosis, amyotrophic lateral
sclerosis, Chiari malformation, syringomyelia, biochemical abnormality, and
persistent infection with human immunodeficiency virus or human
T-lymphotrophic virus type I. We studied three autopsy-proved cases of PLS;
six living patients in whom PLS was diagnosed clinically after
comprehensive evaluations that excluded the alternative diagnoses; and two
patients with this syndrome of PLS and antibodies to human immunodeficiency
virus seropositivity that clinically resembled PLS. Primary lateral
sclerosis is now a respectable and permissible diagnosis.
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