Primary lateral sclerosis as a phenotypic manifestation of familial ALS.

Abstract:

: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.

journal_name

Neurology

journal_title

Neurology

authors

Brugman F,Wokke JH,Vianney de Jong JM,Franssen H,Faber CG,Van den Berg LH

doi

10.1212/01.WNL.0000162033.47893.F7

subject

Has Abstract

pub_date

2005-05-24 00:00:00

pages

1778-9

issue

10

eissn

0028-3878

issn

1526-632X

pii

64/10/1778

journal_volume

64

pub_type

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