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.

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

ALS: pitfalls in the diagnosis
Baek and Desai
PN 2007;7:74-81.
FULL TEXT  

The first ALS2 missense mutation associated with JPLS reveals new aspects of alsin biological function
Panzeri et al.
Brain 2006;129:1710-1719.
ABSTRACT | FULL TEXT  

The natural history of primary lateral sclerosis
Gordon et al.
Neurology 2006;66:647-653.
ABSTRACT | FULL TEXT  

Blink reflex in primary lateral sclerosis
Podikoglou et al.
J. Neurol. Neurosurg. Psychiatry 2004;75:1064-1065.
ABSTRACT | FULL TEXT  

Primary lateral sclerosis and Pierre Marie
Pearce
J. Neurol. Neurosurg. Psychiatry 2004;75:897-897.
FULL TEXT  

Primary lateral sclerosis: A heterogeneous disorder composed of different subtypes?
Zhai et al.
Neurology 2003;60:1258-1265.
ABSTRACT | FULL TEXT  

Infantile ascending hereditary spastic paralysis (IAHSP): Clinical features in 11 families
Lesca et al.
Neurology 2003;60:674-682.
ABSTRACT | FULL TEXT  

Serial MRI findings in a case of primary lateral sclerosis
Smith
Neurology 2002;58:647-649.
ABSTRACT | FULL TEXT  

Primary lateral sclerosis: clinical, neurophysiological, and magnetic resonance findings
Kuipers-Upmeijer et al.
J. Neurol. Neurosurg. Psychiatry 2001;71:615-620.
ABSTRACT | FULL TEXT  

Does primary lateral sclerosis exist?: A study of 20 patients and a review of the literature
Le Forestier et al.
Brain 2001;124:1989-1999.
ABSTRACT | FULL TEXT  

Atypical Dementia and Spastic Paraplegia in a Patient with Primary Lateral Sclerosis and Numerous Neocortical Beta Amyloid Plaques: New Disorder or Alzheimer's Disease Variant?
Engel and Grunnet
J Geriatr Psychiatry Neurol 2000;13:60-64.
ABSTRACT  

Infantile Onset of Hereditary Ascending Spastic Paralysis With Bulbar Involvement
Lerman-Sagie et al.
J Child Neurol 1996;11:54-57.