title: Primary lateral sclerosis. A clinical diagnosis reemerges.
abstract: 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.
authors.affiliation: Department of Neurology, Columbia Presbyterian Medical Center, New York 10032-3784.
authors.forename: D S
authors.initials: DS
authors.lastname: Younger
authors.forename: S
authors.initials: S
authors.lastname: Chou
authors.forename: A P
authors.initials: AP
authors.lastname: Hays
authors.forename: D J
authors.initials: DJ
authors.lastname: Lange
authors.forename: R
authors.initials: R
authors.lastname: Emerson
authors.forename: M
authors.initials: M
authors.lastname: Brin
authors.forename: H
authors.initials: H
authors.lastname: Thompson
authors.forename: L P
authors.initials: LP
authors.lastname: Rowland
mesh_terms: D000328:Adult; D000368:Aged; D001344:Autopsy; D003714:Denervation; D004576:Electromyography; D005260:Female; D005684:Gait; D006679:HIV Seropositivity / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D013118:Spinal Cord Diseases / Q000175:diagnosis* / Q000503:physiopathology; D013577:Syndrome