Article
Primary lateral sclerosis as a phenotypic manifestation of familial ALS.
Neurology - 24 May 2005
Brugman F, Wokke J H J, Vianney de Jong J M B, Franssen H, Faber C G, Van den Berg L H
Abstract excerpt
Primary lateral sclerosis (PLS) is a diagnosis of exclusion in patients with progressive spinobulbar spasticity and could be part of the clinical spectrum of ALS. Unlike ALS, which is familial in 5 to 10% of the cases, PLS has been described as a sporadic disorder in adults. The authors report two patients with PLS from unrelated SOD1-negative familial ALS families. These observations provide further evidence...
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