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. 2020 Nov;21(sup1):3-10.
doi: 10.1080/21678421.2020.1837178.

The clinical spectrum of primary lateral sclerosis

Richard J Barohn  1 , John K Fink  2 , Terry Heiman-Patterson  3 , Edward D Huey  4 , Jennifer Murphy  5 , Jeffrey M Statland  1 , Martin R Turner  6 , Lauren Elman  7
Affiliations

The clinical spectrum of primary lateral sclerosis

Richard J Barohn et al. Amyotroph Lateral Scler Frontotemporal Degener. 2020 Nov.

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.

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