Older Onset Essential Tremor: More Rapid Progression and More Degenerative Pathology
Identifieur interne : 002724 ( Ncbi/Curation ); précédent : 002723; suivant : 002725Older Onset Essential Tremor: More Rapid Progression and More Degenerative Pathology
Auteurs : Elan D. Louis [États-Unis] ; Phyllis L. Faust [États-Unis] ; Jean-Paul G. Vonsattel [États-Unis] ; Lawrence S. Honig [États-Unis] ; Claire Henchcliffe [États-Unis] ; Rajesh Pahwa [États-Unis] ; Kelly E. Lyons [États-Unis] ; Eileen Rios [États-Unis] ; Cordelia Erickson-Davis [États-Unis] ; Carol B. Moskowitz [États-Unis] ; Arlene Lawton [États-Unis]Source :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 2009.
English descriptors
- KwdEn :
- Adolescent, Adult, Age of Onset, Aged, Aged, 80 and over, Axons (ultrastructure), Biological Specimen Banks, Cross-Sectional Studies, Disease Progression, Essential Tremor (drug therapy), Essential Tremor (epidemiology), Essential Tremor (genetics), Essential Tremor (pathology), Female, Humans, Male, Middle Aged, Purkinje Cells (pathology), Young Adult.
- MESH :
- drug therapy : Essential Tremor.
- epidemiology : Essential Tremor.
- genetics : Essential Tremor.
- pathology : Essential Tremor, Purkinje Cells.
- ultrastructure : Axons.
- Adolescent, Adult, Age of Onset, Aged, Aged, 80 and over, Biological Specimen Banks, Cross-Sectional Studies, Disease Progression, Female, Humans, Male, Middle Aged, Young Adult.
Abstract
There are few data on rate of progression in essential tremor (ET).
To quantify rate of tremor progression in a cross-sectional sample of 348 ET cases in an epidemiological study; characterize the relationship between age of tremor onset and rate of tremor progression in that sample; and characterize the relationship between age of tremor onset, rate of tremor progression, and severity of underlying brain changes in 9 cases from a brain repository.
Rate of tremor progression was defined as tremor severity ÷ duration. The degeneration index = number of torpedoes per section ÷ Purkinje cell linear density.
In the epidemiological study, older age of tremor onset was associated with faster rate of tremor progression (p<0.001). In the brain repository, older age of tremor onset was associated with higher degeneration index (p=0.037), and higher degeneration index was associated with faster rate of tremor progression (p=0.018).
In a large clinical sample, older age of onset was associated with more rapid tremor progression. In a brain bank, older age of onset was associated with more degenerative pathology in the cerebellum. As in several neurodegenerative disorders, in older onset cases, it is possible that the disease advances more rapidly.
Url:
DOI: 10.1002/mds.22570
PubMed: 19526587
PubMed Central: 2736358
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Older Onset Essential Tremor: More Rapid Progression and More Degenerative Pathology</title>
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<affiliation wicri:level="4"><nlm:aff id="A2"> Department of Neurology, Columbia University, New York, NY, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea> Department of Neurology, Columbia University, New York, NY</wicri:regionArea>
<placeName><region type="state">État de New York</region>
<settlement type="city">New York</settlement>
</placeName>
<orgName type="university">Université Columbia</orgName>
</affiliation>
</author>
<author><name sortKey="Lawton, Arlene" sort="Lawton, Arlene" uniqKey="Lawton A" first="Arlene" last="Lawton">Arlene Lawton</name>
<affiliation wicri:level="4"><nlm:aff id="A1"> GH Sergievsky Center, Columbia University, New York, NY, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea> GH Sergievsky Center, Columbia University, New York, NY</wicri:regionArea>
<placeName><region type="state">État de New York</region>
<settlement type="city">New York</settlement>
</placeName>
<orgName type="university">Université Columbia</orgName>
</affiliation>
<affiliation wicri:level="4"><nlm:aff id="A3"> Taub Institute for Research on Alzheimer’s Disease and the Aging Brain, Columbia University, New York, NY, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea> Taub Institute for Research on Alzheimer’s Disease and the Aging Brain, Columbia University, New York, NY</wicri:regionArea>
<placeName><region type="state">État de New York</region>
<settlement type="city">New York</settlement>
</placeName>
<orgName type="university">Université Columbia</orgName>
</affiliation>
</author>
</analytic>
<series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title>
<idno type="ISSN">0885-3185</idno>
<idno type="eISSN">1531-8257</idno>
<imprint><date when="2009">2009</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term>
<term>Adult</term>
<term>Age of Onset</term>
<term>Aged</term>
<term>Aged, 80 and over</term>
<term>Axons (ultrastructure)</term>
<term>Biological Specimen Banks</term>
<term>Cross-Sectional Studies</term>
<term>Disease Progression</term>
<term>Essential Tremor (drug therapy)</term>
<term>Essential Tremor (epidemiology)</term>
<term>Essential Tremor (genetics)</term>
<term>Essential Tremor (pathology)</term>
<term>Female</term>
<term>Humans</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Purkinje Cells (pathology)</term>
<term>Young Adult</term>
</keywords>
<keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Essential Tremor</term>
</keywords>
<keywords scheme="MESH" qualifier="epidemiology" xml:lang="en"><term>Essential Tremor</term>
</keywords>
<keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Essential Tremor</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Essential Tremor</term>
<term>Purkinje Cells</term>
</keywords>
<keywords scheme="MESH" qualifier="ultrastructure" xml:lang="en"><term>Axons</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adolescent</term>
<term>Adult</term>
<term>Age of Onset</term>
<term>Aged</term>
<term>Aged, 80 and over</term>
<term>Biological Specimen Banks</term>
<term>Cross-Sectional Studies</term>
<term>Disease Progression</term>
<term>Female</term>
<term>Humans</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Young Adult</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en"><sec id="S1"><title>Background</title>
<p id="P1">There are few data on rate of progression in essential tremor (ET).</p>
</sec>
<sec id="S2"><title>Objectives</title>
<p id="P2">To quantify rate of tremor progression in a cross-sectional sample of 348 ET cases in an epidemiological study; characterize the relationship between age of tremor onset and rate of tremor progression in that sample; and characterize the relationship between age of tremor onset, rate of tremor progression, and severity of underlying brain changes in 9 cases from a brain repository.</p>
</sec>
<sec sec-type="methods" id="S3"><title>Methods</title>
<p id="P3">Rate of tremor progression was defined as tremor severity ÷ duration. The degeneration index = number of torpedoes per section ÷ Purkinje cell linear density.</p>
</sec>
<sec id="S4"><title>Results</title>
<p id="P4">In the epidemiological study, older age of tremor onset was associated with faster rate of tremor progression (p<0.001). In the brain repository, older age of tremor onset was associated with higher degeneration index (p=0.037), and higher degeneration index was associated with faster rate of tremor progression (p=0.018).</p>
</sec>
<sec id="S5"><title>Conclusions</title>
<p id="P5">In a large clinical sample, older age of onset was associated with more rapid tremor progression. In a brain bank, older age of onset was associated with more degenerative pathology in the cerebellum. As in several neurodegenerative disorders, in older onset cases, it is possible that the disease advances more rapidly.</p>
</sec>
</div>
</front>
</TEI>
</record>
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