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Primary Lateral Sclerosis A Clinical Diagnosis Reemerges

David S. Younger, MD; Samuel Chou, MD; Arthur P. Hays, MD; Dale J. Lange, MD; Ronald Emerson, MD; Mitchell Brin, MD; Hartwell Thompson Jr, MD; Lewis P. Rowland, MD
Arch Neurol. 1988;45(12):1304-1307. doi:10.1001/archneur.1988.00520360022005.
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• Adults with slowly progressive non-inherited 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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