Young‐onset Parkinson's disease revisited—clinical features, natural history, and mortality
Identifieur interne : 001E46 ( Main/Exploration ); précédent : 001E45; suivant : 001E47Young‐onset Parkinson's disease revisited—clinical features, natural history, and mortality
Auteurs : Anette Schrag [Royaume-Uni] ; Yoav Ben-Shlomo [Royaume-Uni] ; Richard Brown [Royaume-Uni] ; C. David Marsden [Royaume-Uni] ; Niall Quinn [Royaume-Uni]Source :
- Movement Disorders [ 0885-3185 ] ; 1998-11.
English descriptors
Abstract
The authors report on clinical features and mortality rates in a group of 149 patients with apparent idiopathic parkinsonism starting before the age of 40 years. Ten had juvenile parkinsonism (JP; onset before age 21 years) and 139 had young‐onset Parkinson's disease (YOPD; onset at age 21 to 40 years). Included were 60 patients originally reported 10 years ago. Fifty percent of the JP group had a positive family history of parkinsonism in a first‐degree relative, and clinical presentation was heterogeneous. Mortality risk was threefold that of the normal population. In the YOPD group, the mortality risk was double that of the normal population. Poor initial response to L‐dopa was a risk factor for early death. In two previously reported patients, the diagnosis had been changed to multiple system atrophy and Machado‐Joseph disease. After a median disease duration of 18 years, cognitive impairment was found in only 19% of YOPD patients (13% of those younger than 60 years and 43% of those 60 years or older). Age was the most important factor for development of dementia, but female sex and positive family history of parkinsonism also had more modest predictive value. After a disease duration of 10 years or less, only 5% of patients were experiencing falls and 30% freezing, but all patients had developed L‐dopa‐related fluctuations and dyskinesias. The authors conclude that the mortality rate in parkinsonism starting before the age of 40 is increased in comparison to the normal population and is similar to the general Parkinson's disease population. Intellectual function and postural reflexes are usually well preserved for many years despite a long history of parkinsonism and the early and frequent occurrence of treatment complications, provided the patients remain biologically and chronologically young.
Url:
DOI: 10.1002/mds.870130605
Affiliations:
Links toward previous steps (curation, corpus...)
Le document en format XML
<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Young‐onset Parkinson's disease revisited—clinical features, natural history, and mortality</title><author><name sortKey="Schrag, Anette" sort="Schrag, Anette" uniqKey="Schrag A" first="Anette" last="Schrag">Anette Schrag</name></author><author><name sortKey="Ben Hlomo, Yoav" sort="Ben Hlomo, Yoav" uniqKey="Ben Hlomo Y" first="Yoav" last="Ben-Shlomo">Yoav Ben-Shlomo</name></author><author><name sortKey="Brown, Richard" sort="Brown, Richard" uniqKey="Brown R" first="Richard" last="Brown">Richard Brown</name></author><author><name sortKey="David Marsden, C" sort="David Marsden, C" uniqKey="David Marsden C" first="C." last="David Marsden">C. David Marsden</name></author><author><name sortKey="Quinn, Niall" sort="Quinn, Niall" uniqKey="Quinn N" first="Niall" last="Quinn">Niall Quinn</name></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:35694F8B000005E1A542932F265CAF44469A6DD4</idno><date when="1998" year="1998">1998</date><idno type="doi">10.1002/mds.870130605</idno><idno type="url">https://api.istex.fr/document/35694F8B000005E1A542932F265CAF44469A6DD4/fulltext/pdf</idno><idno type="wicri:Area/Main/Corpus">001694</idno><idno type="wicri:Area/Main/Curation">001458</idno><idno type="wicri:Area/Main/Exploration">001E46</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Young‐onset Parkinson's disease revisited—clinical features, natural history, and mortality</title><author><name sortKey="Schrag, Anette" sort="Schrag, Anette" uniqKey="Schrag A" first="Anette" last="Schrag">Anette Schrag</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Clinical Neurology, Institute of Neurology, University College, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Ben Hlomo, Yoav" sort="Ben Hlomo, Yoav" uniqKey="Ben Hlomo Y" first="Yoav" last="Ben-Shlomo">Yoav Ben-Shlomo</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Social Medicine, University of Bristol, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Brown, Richard" sort="Brown, Richard" uniqKey="Brown R" first="Richard" last="Brown">Richard Brown</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Human Movement and Balance Unit, Institute of Neurology, University College, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="David Marsden, C" sort="David Marsden, C" uniqKey="David Marsden C" first="C." last="David Marsden">C. David Marsden</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Clinical Neurology, Institute of Neurology, University College, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Quinn, Niall" sort="Quinn, Niall" uniqKey="Quinn N" first="Niall" last="Quinn">Niall Quinn</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Clinical Neurology, Institute of Neurology, University College, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</title><title level="j" type="sub">Official Journal of the Movement Disorder Society</title><title level="j" type="abbrev">Mov. Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="1998-11">1998-11</date><biblScope unit="volume">13</biblScope><biblScope unit="issue">6</biblScope><biblScope unit="page" from="885">885</biblScope><biblScope unit="page" to="894">894</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">35694F8B000005E1A542932F265CAF44469A6DD4</idno><idno type="DOI">10.1002/mds.870130605</idno><idno type="ArticleID">MDS870130605</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Juvenile parkinsonism</term><term>Young‐onset Parkinson's disease</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">The authors report on clinical features and mortality rates in a group of 149 patients with apparent idiopathic parkinsonism starting before the age of 40 years. Ten had juvenile parkinsonism (JP; onset before age 21 years) and 139 had young‐onset Parkinson's disease (YOPD; onset at age 21 to 40 years). Included were 60 patients originally reported 10 years ago. Fifty percent of the JP group had a positive family history of parkinsonism in a first‐degree relative, and clinical presentation was heterogeneous. Mortality risk was threefold that of the normal population. In the YOPD group, the mortality risk was double that of the normal population. Poor initial response to L‐dopa was a risk factor for early death. In two previously reported patients, the diagnosis had been changed to multiple system atrophy and Machado‐Joseph disease. After a median disease duration of 18 years, cognitive impairment was found in only 19% of YOPD patients (13% of those younger than 60 years and 43% of those 60 years or older). Age was the most important factor for development of dementia, but female sex and positive family history of parkinsonism also had more modest predictive value. After a disease duration of 10 years or less, only 5% of patients were experiencing falls and 30% freezing, but all patients had developed L‐dopa‐related fluctuations and dyskinesias. The authors conclude that the mortality rate in parkinsonism starting before the age of 40 is increased in comparison to the normal population and is similar to the general Parkinson's disease population. Intellectual function and postural reflexes are usually well preserved for many years despite a long history of parkinsonism and the early and frequent occurrence of treatment complications, provided the patients remain biologically and chronologically young.</div></front></TEI><affiliations><list><country><li>Royaume-Uni</li></country><region><li>Angleterre</li><li>Grand Londres</li></region><settlement><li>Londres</li></settlement></list><tree><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Schrag, Anette" sort="Schrag, Anette" uniqKey="Schrag A" first="Anette" last="Schrag">Anette Schrag</name></region><name sortKey="Ben Hlomo, Yoav" sort="Ben Hlomo, Yoav" uniqKey="Ben Hlomo Y" first="Yoav" last="Ben-Shlomo">Yoav Ben-Shlomo</name><name sortKey="Brown, Richard" sort="Brown, Richard" uniqKey="Brown R" first="Richard" last="Brown">Richard Brown</name><name sortKey="David Marsden, C" sort="David Marsden, C" uniqKey="David Marsden C" first="C." last="David Marsden">C. David Marsden</name><name sortKey="Quinn, Niall" sort="Quinn, Niall" uniqKey="Quinn N" first="Niall" last="Quinn">Niall Quinn</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Sante/explor/ParkinsonV1/Data/Main/Exploration
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 001E46 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 001E46 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien
|wiki= Wicri/Sante
|area= ParkinsonV1
|flux= Main
|étape= Exploration
|type= RBID
|clé= ISTEX:35694F8B000005E1A542932F265CAF44469A6DD4
|texte= Young‐onset Parkinson's disease revisited—clinical features, natural history, and mortality
}}
| This area was generated with Dilib version V0.6.23. |  |