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Primary lateral sclerosis: Predicting functional outcome.
MedLine Citation:
PMID:  22957717     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Our objective was to investigate functional outcome in primary lateral sclerosis (PLS). We followed a group of 24 patients with PLS. Clinical (ALSFRS), respiratory, and neurophysiological (electromyography and transcranial magnetic stimulation) evaluations were performed at entry and regularly over the follow-up period. The time taken for a greater than 10% decrease in ALSFRS compared to the first assessment was defined as the dichotomous outcome; prognostic factors were evaluated using the Cox proportional hazard model. Results demonstrated that the median age at symptom onset was 54 years (range 28-75 years). In 46% symptoms began in the lower limbs, in 21% in the upper limbs and in 33% in bulbar muscles. The median symptom duration at first visit was 3.1 years (range 0.9-11.7 years). At last follow-up the median disease duration was 9.9 years (range 4.2-17.6 years). The median follow-up time was 4.6 years (range 2.1-11 years). We excluded from final analysis three patients with positive family history. Older age at onset (p = 0.019) was related to more rapid functional impairment; gender, forced vital capacity, region of onset and neurophysiological changes did not predict outcome. In conclusion, age is the most critical prognostic factor for functional outcome in PLS.
Authors:
Vânia Almeida; Mamede de Carvalho; Manuel Scotto; Susana Pinto; Anabela Pinto; Benjamim Ohana; Michael Swash
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-9-8
Journal Detail:
Title:  Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases     Volume:  -     ISSN:  1471-180X     ISO Abbreviation:  Amyotroph Lateral Scler     Publication Date:  2012 Sep 
Date Detail:
Created Date:  2012-9-10     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101283386     Medline TA:  Amyotroph Lateral Scler     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Department of Neurosciences, Hospital de Santa Maria-CHLN , Lisbon.
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