The phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases.
Identifieur interne : 000386 ( Main/Exploration ); précédent : 000385; suivant : 000387The phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases.
Auteurs : Gesine Respondek [Allemagne] ; Maria Stamelou ; Carolin Kurz ; Leslie W. Ferguson ; Alexander Rajput ; Wan Zheng Chiu ; John C. Van Swieten ; Claire Troakes ; Safa Al Sarraj ; Ellen Gelpi ; Carles Gaig ; Eduardo Tolosa ; Wolfgang H. Oertel ; Armin Giese ; Sigrun Roeber ; Thomas Arzberger ; Stefan Wagenpfeil ; Günter U. HöglingerSource :
- Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2014.
English descriptors
- KwdEn :
- MESH :
- pathology : Supranuclear Palsy, Progressive.
- physiopathology : Brain.
- therapy : Supranuclear Palsy, Progressive.
- Adult, Aged, Aged, 80 and over, Autopsy, Disease Progression, Female, Humans, Male, Middle Aged, Phenotype, Prognosis, Retrospective Studies, Treatment Outcome.
Abstract
The phenotypic variability of progressive supranuclear palsy (PSP) may account for its frequent misdiagnosis, in particular in early stages of the disease. However, large multicenter studies to define the frequency and natural history of PSP phenotypes are missing. In a cohort of 100 autopsy-confirmed patients we studied the phenotypic spectrum of PSP by retrospective chart review. Patients were derived from five brain banks with expertise in neurodegenerative disorders with referrals from multiple academic hospitals. The clinical characteristics of the 100 cases showed remarkable heterogeneity. Most strikingly, only 24% of cases presented as Richardson's Syndrome (RS), and more than half of the cases either showed overlapping features of several predescribed phenotypes, or features not fitting proposed classification criteria for PSP phenotypes. Classification of patients according to predominant clinical features in the first 2 years of the disease course allowed a more comprehensive description of the phenotypic spectrum. These predominance types differed significantly with regard to survival time and frequency of cognitive deficits. In summary, the phenotypic spectrum of PSP may be broader and more variable than previously described in single-center studies. Thus, too strict clinical criteria defining distinct phenotypes may not reflect this variability. A more pragmatic clinical approach using predominance types could potentially be more helpful in the early recognition of and for making prognostic predictions for these patients. Given the limitations arising from the retrospective nature of this analysis, a systematic validation in a prospective cohort study is imperative.
DOI: 10.1002/mds.26054
PubMed: 25370486
Affiliations:
Links toward previous steps (curation, corpus...)
- to stream PubMed, to step Corpus: 000356
- to stream PubMed, to step Curation: 000356
- to stream PubMed, to step Checkpoint: 000388
- to stream Ncbi, to step Merge: 004148
- to stream Ncbi, to step Curation: 004148
- to stream Ncbi, to step Checkpoint: 004148
- to stream Main, to step Merge: 000386
- to stream Main, to step Curation: 000386
Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">The phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases.</title><author><name sortKey="Respondek, Gesine" sort="Respondek, Gesine" uniqKey="Respondek G" first="Gesine" last="Respondek">Gesine Respondek</name><affiliation wicri:level="4"><nlm:affiliation>Department of Neurology, Technische Universität München, Munich, Germany; German Center for Neurodegenerative Diseases (DZNE), Munich, Germany; Department of Neurology, Philipps Universität, Marburg, Germany.</nlm:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Technische Universität München, Munich, Germany; German Center for Neurodegenerative Diseases (DZNE), Munich, Germany; Department of Neurology, Philipps Universität, Marburg</wicri:regionArea><placeName><region type="land" nuts="1">Hesse (Land)</region><region type="district" nuts="2">District de Giessen</region><settlement type="city">Marbourg</settlement></placeName><orgName type="university">Université technique de Munich</orgName></affiliation></author><author><name sortKey="Stamelou, Maria" sort="Stamelou, Maria" uniqKey="Stamelou M" first="Maria" last="Stamelou">Maria Stamelou</name></author><author><name sortKey="Kurz, Carolin" sort="Kurz, Carolin" uniqKey="Kurz C" first="Carolin" last="Kurz">Carolin Kurz</name></author><author><name sortKey="Ferguson, Leslie W" sort="Ferguson, Leslie W" uniqKey="Ferguson L" first="Leslie W" last="Ferguson">Leslie W. Ferguson</name></author><author><name sortKey="Rajput, Alexander" sort="Rajput, Alexander" uniqKey="Rajput A" first="Alexander" last="Rajput">Alexander Rajput</name></author><author><name sortKey="Chiu, Wan Zheng" sort="Chiu, Wan Zheng" uniqKey="Chiu W" first="Wan Zheng" last="Chiu">Wan Zheng Chiu</name></author><author><name sortKey="Van Swieten, John C" sort="Van Swieten, John C" uniqKey="Van Swieten J" first="John C" last="Van Swieten">John C. Van Swieten</name></author><author><name sortKey="Troakes, Claire" sort="Troakes, Claire" uniqKey="Troakes C" first="Claire" last="Troakes">Claire Troakes</name></author><author><name sortKey="Al Sarraj, Safa" sort="Al Sarraj, Safa" uniqKey="Al Sarraj S" first="Safa" last="Al Sarraj">Safa Al Sarraj</name></author><author><name sortKey="Gelpi, Ellen" sort="Gelpi, Ellen" uniqKey="Gelpi E" first="Ellen" last="Gelpi">Ellen Gelpi</name></author><author><name sortKey="Gaig, Carles" sort="Gaig, Carles" uniqKey="Gaig C" first="Carles" last="Gaig">Carles Gaig</name></author><author><name sortKey="Tolosa, Eduardo" sort="Tolosa, Eduardo" uniqKey="Tolosa E" first="Eduardo" last="Tolosa">Eduardo Tolosa</name></author><author><name sortKey="Oertel, Wolfgang H" sort="Oertel, Wolfgang H" uniqKey="Oertel W" first="Wolfgang H" last="Oertel">Wolfgang H. Oertel</name></author><author><name sortKey="Giese, Armin" sort="Giese, Armin" uniqKey="Giese A" first="Armin" last="Giese">Armin Giese</name></author><author><name sortKey="Roeber, Sigrun" sort="Roeber, Sigrun" uniqKey="Roeber S" first="Sigrun" last="Roeber">Sigrun Roeber</name></author><author><name sortKey="Arzberger, Thomas" sort="Arzberger, Thomas" uniqKey="Arzberger T" first="Thomas" last="Arzberger">Thomas Arzberger</name></author><author><name sortKey="Wagenpfeil, Stefan" sort="Wagenpfeil, Stefan" uniqKey="Wagenpfeil S" first="Stefan" last="Wagenpfeil">Stefan Wagenpfeil</name></author><author><name sortKey="Hoglinger, Gunter U" sort="Hoglinger, Gunter U" uniqKey="Hoglinger G" first="Günter U" last="Höglinger">Günter U. Höglinger</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2014">2014</date><idno type="RBID">pubmed:25370486</idno><idno type="pmid">25370486</idno><idno type="doi">10.1002/mds.26054</idno><idno type="wicri:Area/PubMed/Corpus">000356</idno><idno type="wicri:Area/PubMed/Curation">000356</idno><idno type="wicri:Area/PubMed/Checkpoint">000388</idno><idno type="wicri:Area/Ncbi/Merge">004148</idno><idno type="wicri:Area/Ncbi/Curation">004148</idno><idno type="wicri:Area/Ncbi/Checkpoint">004148</idno><idno type="wicri:Area/Main/Merge">000386</idno><idno type="wicri:Area/Main/Curation">000386</idno><idno type="wicri:Area/Main/Exploration">000386</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">The phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases.</title><author><name sortKey="Respondek, Gesine" sort="Respondek, Gesine" uniqKey="Respondek G" first="Gesine" last="Respondek">Gesine Respondek</name><affiliation wicri:level="4"><nlm:affiliation>Department of Neurology, Technische Universität München, Munich, Germany; German Center for Neurodegenerative Diseases (DZNE), Munich, Germany; Department of Neurology, Philipps Universität, Marburg, Germany.</nlm:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Technische Universität München, Munich, Germany; German Center for Neurodegenerative Diseases (DZNE), Munich, Germany; Department of Neurology, Philipps Universität, Marburg</wicri:regionArea><placeName><region type="land" nuts="1">Hesse (Land)</region><region type="district" nuts="2">District de Giessen</region><settlement type="city">Marbourg</settlement></placeName><orgName type="university">Université technique de Munich</orgName></affiliation></author><author><name sortKey="Stamelou, Maria" sort="Stamelou, Maria" uniqKey="Stamelou M" first="Maria" last="Stamelou">Maria Stamelou</name></author><author><name sortKey="Kurz, Carolin" sort="Kurz, Carolin" uniqKey="Kurz C" first="Carolin" last="Kurz">Carolin Kurz</name></author><author><name sortKey="Ferguson, Leslie W" sort="Ferguson, Leslie W" uniqKey="Ferguson L" first="Leslie W" last="Ferguson">Leslie W. Ferguson</name></author><author><name sortKey="Rajput, Alexander" sort="Rajput, Alexander" uniqKey="Rajput A" first="Alexander" last="Rajput">Alexander Rajput</name></author><author><name sortKey="Chiu, Wan Zheng" sort="Chiu, Wan Zheng" uniqKey="Chiu W" first="Wan Zheng" last="Chiu">Wan Zheng Chiu</name></author><author><name sortKey="Van Swieten, John C" sort="Van Swieten, John C" uniqKey="Van Swieten J" first="John C" last="Van Swieten">John C. Van Swieten</name></author><author><name sortKey="Troakes, Claire" sort="Troakes, Claire" uniqKey="Troakes C" first="Claire" last="Troakes">Claire Troakes</name></author><author><name sortKey="Al Sarraj, Safa" sort="Al Sarraj, Safa" uniqKey="Al Sarraj S" first="Safa" last="Al Sarraj">Safa Al Sarraj</name></author><author><name sortKey="Gelpi, Ellen" sort="Gelpi, Ellen" uniqKey="Gelpi E" first="Ellen" last="Gelpi">Ellen Gelpi</name></author><author><name sortKey="Gaig, Carles" sort="Gaig, Carles" uniqKey="Gaig C" first="Carles" last="Gaig">Carles Gaig</name></author><author><name sortKey="Tolosa, Eduardo" sort="Tolosa, Eduardo" uniqKey="Tolosa E" first="Eduardo" last="Tolosa">Eduardo Tolosa</name></author><author><name sortKey="Oertel, Wolfgang H" sort="Oertel, Wolfgang H" uniqKey="Oertel W" first="Wolfgang H" last="Oertel">Wolfgang H. Oertel</name></author><author><name sortKey="Giese, Armin" sort="Giese, Armin" uniqKey="Giese A" first="Armin" last="Giese">Armin Giese</name></author><author><name sortKey="Roeber, Sigrun" sort="Roeber, Sigrun" uniqKey="Roeber S" first="Sigrun" last="Roeber">Sigrun Roeber</name></author><author><name sortKey="Arzberger, Thomas" sort="Arzberger, Thomas" uniqKey="Arzberger T" first="Thomas" last="Arzberger">Thomas Arzberger</name></author><author><name sortKey="Wagenpfeil, Stefan" sort="Wagenpfeil, Stefan" uniqKey="Wagenpfeil S" first="Stefan" last="Wagenpfeil">Stefan Wagenpfeil</name></author><author><name sortKey="Hoglinger, Gunter U" sort="Hoglinger, Gunter U" uniqKey="Hoglinger G" first="Günter U" last="Höglinger">Günter U. Höglinger</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="eISSN">1531-8257</idno><imprint><date when="2014" type="published">2014</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Aged</term><term>Aged, 80 and over</term><term>Autopsy</term><term>Brain (physiopathology)</term><term>Disease Progression</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Phenotype</term><term>Prognosis</term><term>Retrospective Studies</term><term>Supranuclear Palsy, Progressive (pathology)</term><term>Supranuclear Palsy, Progressive (therapy)</term><term>Treatment Outcome</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Supranuclear Palsy, Progressive</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Brain</term></keywords><keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Supranuclear Palsy, Progressive</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Aged</term><term>Aged, 80 and over</term><term>Autopsy</term><term>Disease Progression</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Phenotype</term><term>Prognosis</term><term>Retrospective Studies</term><term>Treatment Outcome</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">The phenotypic variability of progressive supranuclear palsy (PSP) may account for its frequent misdiagnosis, in particular in early stages of the disease. However, large multicenter studies to define the frequency and natural history of PSP phenotypes are missing. In a cohort of 100 autopsy-confirmed patients we studied the phenotypic spectrum of PSP by retrospective chart review. Patients were derived from five brain banks with expertise in neurodegenerative disorders with referrals from multiple academic hospitals. The clinical characteristics of the 100 cases showed remarkable heterogeneity. Most strikingly, only 24% of cases presented as Richardson's Syndrome (RS), and more than half of the cases either showed overlapping features of several predescribed phenotypes, or features not fitting proposed classification criteria for PSP phenotypes. Classification of patients according to predominant clinical features in the first 2 years of the disease course allowed a more comprehensive description of the phenotypic spectrum. These predominance types differed significantly with regard to survival time and frequency of cognitive deficits. In summary, the phenotypic spectrum of PSP may be broader and more variable than previously described in single-center studies. Thus, too strict clinical criteria defining distinct phenotypes may not reflect this variability. A more pragmatic clinical approach using predominance types could potentially be more helpful in the early recognition of and for making prognostic predictions for these patients. Given the limitations arising from the retrospective nature of this analysis, a systematic validation in a prospective cohort study is imperative.</div></front></TEI><affiliations><list><country><li>Allemagne</li></country><region><li>District de Giessen</li><li>Hesse (Land)</li></region><settlement><li>Marbourg</li></settlement><orgName><li>Université technique de Munich</li></orgName></list><tree><noCountry><name sortKey="Al Sarraj, Safa" sort="Al Sarraj, Safa" uniqKey="Al Sarraj S" first="Safa" last="Al Sarraj">Safa Al Sarraj</name><name sortKey="Arzberger, Thomas" sort="Arzberger, Thomas" uniqKey="Arzberger T" first="Thomas" last="Arzberger">Thomas Arzberger</name><name sortKey="Chiu, Wan Zheng" sort="Chiu, Wan Zheng" uniqKey="Chiu W" first="Wan Zheng" last="Chiu">Wan Zheng Chiu</name><name sortKey="Ferguson, Leslie W" sort="Ferguson, Leslie W" uniqKey="Ferguson L" first="Leslie W" last="Ferguson">Leslie W. Ferguson</name><name sortKey="Gaig, Carles" sort="Gaig, Carles" uniqKey="Gaig C" first="Carles" last="Gaig">Carles Gaig</name><name sortKey="Gelpi, Ellen" sort="Gelpi, Ellen" uniqKey="Gelpi E" first="Ellen" last="Gelpi">Ellen Gelpi</name><name sortKey="Giese, Armin" sort="Giese, Armin" uniqKey="Giese A" first="Armin" last="Giese">Armin Giese</name><name sortKey="Hoglinger, Gunter U" sort="Hoglinger, Gunter U" uniqKey="Hoglinger G" first="Günter U" last="Höglinger">Günter U. Höglinger</name><name sortKey="Kurz, Carolin" sort="Kurz, Carolin" uniqKey="Kurz C" first="Carolin" last="Kurz">Carolin Kurz</name><name sortKey="Oertel, Wolfgang H" sort="Oertel, Wolfgang H" uniqKey="Oertel W" first="Wolfgang H" last="Oertel">Wolfgang H. Oertel</name><name sortKey="Rajput, Alexander" sort="Rajput, Alexander" uniqKey="Rajput A" first="Alexander" last="Rajput">Alexander Rajput</name><name sortKey="Roeber, Sigrun" sort="Roeber, Sigrun" uniqKey="Roeber S" first="Sigrun" last="Roeber">Sigrun Roeber</name><name sortKey="Stamelou, Maria" sort="Stamelou, Maria" uniqKey="Stamelou M" first="Maria" last="Stamelou">Maria Stamelou</name><name sortKey="Tolosa, Eduardo" sort="Tolosa, Eduardo" uniqKey="Tolosa E" first="Eduardo" last="Tolosa">Eduardo Tolosa</name><name sortKey="Troakes, Claire" sort="Troakes, Claire" uniqKey="Troakes C" first="Claire" last="Troakes">Claire Troakes</name><name sortKey="Van Swieten, John C" sort="Van Swieten, John C" uniqKey="Van Swieten J" first="John C" last="Van Swieten">John C. Van Swieten</name><name sortKey="Wagenpfeil, Stefan" sort="Wagenpfeil, Stefan" uniqKey="Wagenpfeil S" first="Stefan" last="Wagenpfeil">Stefan Wagenpfeil</name></noCountry><country name="Allemagne"><region name="Hesse (Land)"><name sortKey="Respondek, Gesine" sort="Respondek, Gesine" uniqKey="Respondek G" first="Gesine" last="Respondek">Gesine Respondek</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Santé/explor/MovDisordV3/Data/Main/Exploration
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 000386 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 000386 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien
|wiki= Wicri/Santé
|area= MovDisordV3
|flux= Main
|étape= Exploration
|type= RBID
|clé= pubmed:25370486
|texte= The phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases.
}}
Pour générer des pages wiki
HfdIndexSelect -h $EXPLOR_AREA/Data/Main/Exploration/RBID.i -Sk "pubmed:25370486" \
| HfdSelect -Kh $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd \
| NlmPubMed2Wicri -a MovDisordV3
| This area was generated with Dilib version V0.6.23. |  |