Primary lateral sclerosis as a phenotypic manifestation of familial ALS

F. Brugman, J. H. J. Wokke, J. M. B. Vianney de Jong, H. Franssen, C. G. Faber, L. H. van den Berg

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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 that PLS can be linked pathophysiologically to ALS
Original languageEnglish
Pages (from-to)1778-1779
Issue number10
Publication statusPublished - 2005

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