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December 1988

Primary Lateral Sclerosis: A Clinical Diagnosis Reemerges

Author Affiliations

From the Department of Neurology (Drs Younger, Lange, Emerson, Brin, and Rowland) and Division of Neuropathology (Dr Hays), Neurological Institute of New York, Columbia Presbyterian Medical Center; Department of Pathology, Cleveland Clinic (Dr Chou); and Department of Neurology, University of Connecticut, Farmington (Dr Thompson).

Arch Neurol. 1988;45(12):1304-1307. doi:10.1001/archneur.1988.00520360022005

• 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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