Document Detail

Natural history of upper motor neuron-dominant ALS.
MedLine Citation:
PMID:  19929748     Owner:  NLM     Status:  MEDLINE    
A new amyotrophic lateral sclerosis (ALS) category named 'UMN-dominant ALS' and defined as 'due predominantly to UMN signs but with minor electromyogram (EMG) denervation or LMN signs on examination' has been proposed. The clinical and laboratory features of 20 patients with UMN-dominant ALS are described here, their disease course is analysed longitudinally according to their disability progression, and all these parameters are compared with those of typical ALS patients. Ten women and 10 men diagnosed with UMN-dominant ALS were evaluated. Their mean age at disease onset was 58.6 years. At the most recent evaluation, after a mean disease duration of 7.7 years, all patients progressed to a tetrapyramidal syndrome with pseudobulbar features of varying degree. No patient had respiratory problems. Cognitive impairment was observed in eight patients. The differences in disease progression between the UMN-dominant ALS and typical ALS patients proved significant (p <0.02) both with regard to the total ALSFRS-R score at six months and to each single region subscore at 12 months. Our findings suggest that there is both a different pattern of disability and longer survival in UMN-dominant ALS compared to classic ALS patients.
Gianni Sorarù; Mario Ermani; Giancarlo Logroscino; Arianna Palmieri; Carla D' Ascenzo; Valeria Orsetti; Marco Volpe; Valentina Cima; Gabriella Zara; Elena Pegoraro; Corrado Angelini
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases     Volume:  11     ISSN:  1471-180X     ISO Abbreviation:  Amyotroph Lateral Scler     Publication Date:  2010 Oct 
Date Detail:
Created Date:  2010-09-15     Completed Date:  2011-01-13     Revised Date:  2012-04-11    
Medline Journal Info:
Nlm Unique ID:  101283386     Medline TA:  Amyotroph Lateral Scler     Country:  England    
Other Details:
Languages:  eng     Pagination:  424-9     Citation Subset:  IM    
Department of Neurosciences, University of Padova, Italy.
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MeSH Terms
Amyotrophic Lateral Sclerosis / diagnosis,  pathology,  physiopathology*
Disease Progression*
Kaplan-Meier Estimate
Middle Aged
Motor Neuron Disease / diagnosis,  pathology,  physiopathology*
Motor Neurons / physiology*
Grant Support

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