Source:http://linkedlifedata.com/resource/pubmed/id/21702734
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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
4
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pubmed:dateCreated |
2011-6-27
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pubmed:abstractText |
The aim of our study was to analyse the natural history and clinical features of upper motor neuron- dominant (UMN-D) ALS. We studied a large series of sporadic ALS patients admitted in a single referral centre over a 23-year period. UMN-D phenotype was compared with other ALS forms, including classic ALS, flail arm and progressive muscular atrophy. Seven hundred and thirty-four sporadic ALS patients were included of which 163 had UMN-D ALS. The mean age of onset in UMN-D ALS (52 years) was 10 years lower than in classic ALS (61.4 years, p < 0.0001); sex ratio by age groups significantly differed with respect to other phenotypes. The pattern of spread of lower motor neuron signs in UMN-D was characterized by early involvement of upper limb muscles and late impairment of respiratory muscles. Duration of the disease was longer in the UMN-D group (56 months) than in classic ALS (33 months, p < 0.001). The UMN-D phenotype was a strong independent predictor of long survival. In summary, UMN-D ALS showed significant differences in age of onset, sex ratio, pattern of spreading and prognosis with respect to other ALS forms, most probably reflecting biological differences.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:status |
MEDLINE
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pubmed:month |
Jul
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pubmed:issn |
1471-180X
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pubmed:author |
pubmed-author:BisogniGiuliaG,
pubmed-author:ConteAmeliaA,
pubmed-author:GrandeAlessandra DelAD,
pubmed-author:LattanteSerenaS,
pubmed-author:LuigettiMarcoM,
pubmed-author:MadiaFrancescaF,
pubmed-author:MarangiGiuseppeG,
pubmed-author:MeleoEmilianaE,
pubmed-author:MonacoMauro LoML,
pubmed-author:SabatelliMarioM,
pubmed-author:ZollinoMarcellaM
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pubmed:issnType |
Electronic
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pubmed:volume |
12
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
278-82
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pubmed:meshHeading |
pubmed-meshheading:21702734-Adult,
pubmed-meshheading:21702734-Age of Onset,
pubmed-meshheading:21702734-Aged,
pubmed-meshheading:21702734-Aged, 80 and over,
pubmed-meshheading:21702734-Amyotrophic Lateral Sclerosis,
pubmed-meshheading:21702734-Disease Progression,
pubmed-meshheading:21702734-Female,
pubmed-meshheading:21702734-Follow-Up Studies,
pubmed-meshheading:21702734-Humans,
pubmed-meshheading:21702734-Male,
pubmed-meshheading:21702734-Middle Aged,
pubmed-meshheading:21702734-Motor Neurons,
pubmed-meshheading:21702734-Phenotype,
pubmed-meshheading:21702734-Survival Rate,
pubmed-meshheading:21702734-Young Adult
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pubmed:year |
2011
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pubmed:articleTitle |
Uncovering amyotrophic lateral sclerosis phenotypes: clinical features and long-term follow-up of upper motor neuron-dominant ALS.
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pubmed:affiliation |
Istituto di Neurologia, Università Cattolica del Sacro, Rome, Italy. msabatelli@rm.unicatt.it
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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