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Updated: Nov 17, 2025

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
Published on: July 29, 2007
The clinical spectrum of primary lateral sclerosis
Richard J Barohn1, John K Fink2, Terry Heiman-Patterson3
1Department of Neurology, University of Kansas Medical Center, Kansas City, KS, USA.
None:
Primary lateral sclerosis is a distinct entity that has recently been classified as a "restricted phenotype" of ALS. It is characterized by a pattern of isolated upper motor neuron involvement that often begins in the legs and spreads diffusely. Distinction from other conditions requires careful consideration of clinical presentation and time course of disease. Mills' Syndrome is a rare unilateral variant of primary lateral sclerosis. Cognitive and behavioral involvement may occur.
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