The clinical spectrum of primary lateral sclerosis

Abstract

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.

Keywords:

• Epidemiology
• prognostic
• survival

Declaration of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.