Movement Disorders (revue)

Attention, ce site est en cours de développement !
Attention, site généré par des moyens informatiques à partir de corpus bruts.
Les informations ne sont donc pas validées.

Annual Change in Friedreich's Ataxia Evaluated by the Scale for the Assessment and Rating of Ataxia (SARA) is Independent of Disease Severity

Identifieur interne : 000285 ( PascalFrancis/Checkpoint ); précédent : 000284; suivant : 000286

Annual Change in Friedreich's Ataxia Evaluated by the Scale for the Assessment and Rating of Ataxia (SARA) is Independent of Disease Severity

Auteurs : Cecilia Marelli [France] ; Julie Figoni [France] ; Perrine Charles [France] ; Mathieu Anheim [France] ; Maya Tchikviladze [France] ; Carlo-Maria Vincitorio [France] ; Sophie Tezenas Du Montcel [France] ; Alexis Brice [France] ; Jean Louis Golmard [France] ; Alexandra Dürr [France]

Source :

RBID : Pascal:12-0106500

Descripteurs français

English descriptors

Abstract

Background: The objective of the study was to evaluate the sensitivity to change of the Scale for the Assessment and Rating of Ataxia (score, 0-40) in Friedreich's ataxia. Methods: This was a follow-up study in adult patients with genetically confirmed Friedreich's ataxia evaluated at least twice (minimum interval, 6 months). Participants were outpatients at the Center for Neurogenetics of the Pitie-Salpêtrière Hospital in Paris. Results: We included 84 patients; 60% had 3 or more evaluations. The mean score on first assessment was 22.7 ± 9, and the mean follow-up was 1.84 ± 1.10 years. The mean increase was 1.36 ± 2.3 points/ year; this variation was not significantly linked to factors known to influence disease severity such as age at onset, disease duration, GAA expansion length, and wheelchair use. Conclusions: In adult Friedreich's ataxia patients the Scale for the Assessment and Rating of Ataxia can detect annual changes independently of disease severity. In future therapeutic trials no patient stratification is globally required.


Affiliations:


Links toward previous steps (curation, corpus...)


Links to Exploration step

Pascal:12-0106500

Le document en format XML

<record>
<TEI>
<teiHeader>
<fileDesc>
<titleStmt>
<title xml:lang="en" level="a">Annual Change in Friedreich's Ataxia Evaluated by the Scale for the Assessment and Rating of Ataxia (SARA) is Independent of Disease Severity</title>
<author>
<name sortKey="Marelli, Cecilia" sort="Marelli, Cecilia" uniqKey="Marelli C" first="Cecilia" last="Marelli">Cecilia Marelli</name>
<affiliation wicri:level="1">
<inist:fA14 i1="01">
<s1>Université Pierre et Marie Curie-Paris6, Centre de Recherche de l'Institut du Cerveau et de la Moelle epiniere</s1>
<s2>UMR-S975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="02">
<s1>Inserm</s1>
<s2>U975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="03">
<s1>Cnrs</s1>
<s2>UMR 7225, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Figoni, Julie" sort="Figoni, Julie" uniqKey="Figoni J" first="Julie" last="Figoni">Julie Figoni</name>
<affiliation wicri:level="1">
<inist:fA14 i1="05">
<s1>AP-HP, Department of Biostatistics and Medical Informatics and Pitié-Salpêtriére Charles-Foix Clinical Research Unit</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Charles, Perrine" sort="Charles, Perrine" uniqKey="Charles P" first="Perrine" last="Charles">Perrine Charles</name>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="06">
<s1>AP-HP, Pitié-Salpêtrière Hospital, Federation of the Nervous System Diseases</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>3 aut.</sZ>
<sZ>8 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Anheim, Mathieu" sort="Anheim, Mathieu" uniqKey="Anheim M" first="Mathieu" last="Anheim">Mathieu Anheim</name>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Tchikviladze, Maya" sort="Tchikviladze, Maya" uniqKey="Tchikviladze M" first="Maya" last="Tchikviladze">Maya Tchikviladze</name>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Vincitorio, Carlo Maria" sort="Vincitorio, Carlo Maria" uniqKey="Vincitorio C" first="Carlo-Maria" last="Vincitorio">Carlo-Maria Vincitorio</name>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Du Montcel, Sophie Tezenas" sort="Du Montcel, Sophie Tezenas" uniqKey="Du Montcel S" first="Sophie Tezenas" last="Du Montcel">Sophie Tezenas Du Montcel</name>
<affiliation wicri:level="1">
<inist:fA14 i1="05">
<s1>AP-HP, Department of Biostatistics and Medical Informatics and Pitié-Salpêtriére Charles-Foix Clinical Research Unit</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="07">
<s1>Université Pierre et Marie Curie-Paris6, ER4 (formerly EA 3974), Modeling in Clinical Research</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Brice, Alexis" sort="Brice, Alexis" uniqKey="Brice A" first="Alexis" last="Brice">Alexis Brice</name>
<affiliation wicri:level="1">
<inist:fA14 i1="01">
<s1>Université Pierre et Marie Curie-Paris6, Centre de Recherche de l'Institut du Cerveau et de la Moelle epiniere</s1>
<s2>UMR-S975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="02">
<s1>Inserm</s1>
<s2>U975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="03">
<s1>Cnrs</s1>
<s2>UMR 7225, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="06">
<s1>AP-HP, Pitié-Salpêtrière Hospital, Federation of the Nervous System Diseases</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>3 aut.</sZ>
<sZ>8 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Golmard, Jean Louis" sort="Golmard, Jean Louis" uniqKey="Golmard J" first="Jean Louis" last="Golmard">Jean Louis Golmard</name>
<affiliation wicri:level="1">
<inist:fA14 i1="05">
<s1>AP-HP, Department of Biostatistics and Medical Informatics and Pitié-Salpêtriére Charles-Foix Clinical Research Unit</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="07">
<s1>Université Pierre et Marie Curie-Paris6, ER4 (formerly EA 3974), Modeling in Clinical Research</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Dürr">Alexandra Dürr</name>
<affiliation wicri:level="1">
<inist:fA14 i1="01">
<s1>Université Pierre et Marie Curie-Paris6, Centre de Recherche de l'Institut du Cerveau et de la Moelle epiniere</s1>
<s2>UMR-S975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="02">
<s1>Inserm</s1>
<s2>U975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="03">
<s1>Cnrs</s1>
<s2>UMR 7225, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
</titleStmt>
<publicationStmt>
<idno type="wicri:source">INIST</idno>
<idno type="inist">12-0106500</idno>
<date when="2012">2012</date>
<idno type="stanalyst">PASCAL 12-0106500 INIST</idno>
<idno type="RBID">Pascal:12-0106500</idno>
<idno type="wicri:Area/PascalFrancis/Corpus">000298</idno>
<idno type="wicri:Area/PascalFrancis/Curation">002A16</idno>
<idno type="wicri:Area/PascalFrancis/Checkpoint">000285</idno>
</publicationStmt>
<sourceDesc>
<biblStruct>
<analytic>
<title xml:lang="en" level="a">Annual Change in Friedreich's Ataxia Evaluated by the Scale for the Assessment and Rating of Ataxia (SARA) is Independent of Disease Severity</title>
<author>
<name sortKey="Marelli, Cecilia" sort="Marelli, Cecilia" uniqKey="Marelli C" first="Cecilia" last="Marelli">Cecilia Marelli</name>
<affiliation wicri:level="1">
<inist:fA14 i1="01">
<s1>Université Pierre et Marie Curie-Paris6, Centre de Recherche de l'Institut du Cerveau et de la Moelle epiniere</s1>
<s2>UMR-S975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="02">
<s1>Inserm</s1>
<s2>U975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="03">
<s1>Cnrs</s1>
<s2>UMR 7225, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Figoni, Julie" sort="Figoni, Julie" uniqKey="Figoni J" first="Julie" last="Figoni">Julie Figoni</name>
<affiliation wicri:level="1">
<inist:fA14 i1="05">
<s1>AP-HP, Department of Biostatistics and Medical Informatics and Pitié-Salpêtriére Charles-Foix Clinical Research Unit</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Charles, Perrine" sort="Charles, Perrine" uniqKey="Charles P" first="Perrine" last="Charles">Perrine Charles</name>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="06">
<s1>AP-HP, Pitié-Salpêtrière Hospital, Federation of the Nervous System Diseases</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>3 aut.</sZ>
<sZ>8 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Anheim, Mathieu" sort="Anheim, Mathieu" uniqKey="Anheim M" first="Mathieu" last="Anheim">Mathieu Anheim</name>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Tchikviladze, Maya" sort="Tchikviladze, Maya" uniqKey="Tchikviladze M" first="Maya" last="Tchikviladze">Maya Tchikviladze</name>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Vincitorio, Carlo Maria" sort="Vincitorio, Carlo Maria" uniqKey="Vincitorio C" first="Carlo-Maria" last="Vincitorio">Carlo-Maria Vincitorio</name>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Du Montcel, Sophie Tezenas" sort="Du Montcel, Sophie Tezenas" uniqKey="Du Montcel S" first="Sophie Tezenas" last="Du Montcel">Sophie Tezenas Du Montcel</name>
<affiliation wicri:level="1">
<inist:fA14 i1="05">
<s1>AP-HP, Department of Biostatistics and Medical Informatics and Pitié-Salpêtriére Charles-Foix Clinical Research Unit</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="07">
<s1>Université Pierre et Marie Curie-Paris6, ER4 (formerly EA 3974), Modeling in Clinical Research</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Brice, Alexis" sort="Brice, Alexis" uniqKey="Brice A" first="Alexis" last="Brice">Alexis Brice</name>
<affiliation wicri:level="1">
<inist:fA14 i1="01">
<s1>Université Pierre et Marie Curie-Paris6, Centre de Recherche de l'Institut du Cerveau et de la Moelle epiniere</s1>
<s2>UMR-S975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="02">
<s1>Inserm</s1>
<s2>U975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="03">
<s1>Cnrs</s1>
<s2>UMR 7225, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="06">
<s1>AP-HP, Pitié-Salpêtrière Hospital, Federation of the Nervous System Diseases</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>3 aut.</sZ>
<sZ>8 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Golmard, Jean Louis" sort="Golmard, Jean Louis" uniqKey="Golmard J" first="Jean Louis" last="Golmard">Jean Louis Golmard</name>
<affiliation wicri:level="1">
<inist:fA14 i1="05">
<s1>AP-HP, Department of Biostatistics and Medical Informatics and Pitié-Salpêtriére Charles-Foix Clinical Research Unit</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="07">
<s1>Université Pierre et Marie Curie-Paris6, ER4 (formerly EA 3974), Modeling in Clinical Research</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Dürr">Alexandra Dürr</name>
<affiliation wicri:level="1">
<inist:fA14 i1="01">
<s1>Université Pierre et Marie Curie-Paris6, Centre de Recherche de l'Institut du Cerveau et de la Moelle epiniere</s1>
<s2>UMR-S975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="02">
<s1>Inserm</s1>
<s2>U975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="03">
<s1>Cnrs</s1>
<s2>UMR 7225, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
</analytic>
<series>
<title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
<imprint>
<date when="2012">2012</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
<seriesStmt>
<title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
</seriesStmt>
</fileDesc>
<profileDesc>
<textClass>
<keywords scheme="KwdEn" xml:lang="en">
<term>Ataxia</term>
<term>Evaluation scale</term>
<term>Friedreich ataxia</term>
<term>Nervous system diseases</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr">
<term>Hérédodégénérescence spinocérébelleuse de Friedreich</term>
<term>Ataxie</term>
<term>Pathologie du système nerveux</term>
<term>Echelle d'évaluation</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front>
<div type="abstract" xml:lang="en">Background: The objective of the study was to evaluate the sensitivity to change of the Scale for the Assessment and Rating of Ataxia (score, 0-40) in Friedreich's ataxia. Methods: This was a follow-up study in adult patients with genetically confirmed Friedreich's ataxia evaluated at least twice (minimum interval, 6 months). Participants were outpatients at the Center for Neurogenetics of the Pitie-Salpêtrière Hospital in Paris. Results: We included 84 patients; 60% had 3 or more evaluations. The mean score on first assessment was 22.7 ± 9, and the mean follow-up was 1.84 ± 1.10 years. The mean increase was 1.36 ± 2.3 points/ year; this variation was not significantly linked to factors known to influence disease severity such as age at onset, disease duration, GAA expansion length, and wheelchair use. Conclusions: In adult Friedreich's ataxia patients the Scale for the Assessment and Rating of Ataxia can detect annual changes independently of disease severity. In future therapeutic trials no patient stratification is globally required.</div>
</front>
</TEI>
<inist>
<standard h6="B">
<pA>
<fA01 i1="01" i2="1">
<s0>0885-3185</s0>
</fA01>
<fA03 i2="1">
<s0>Mov. disord.</s0>
</fA03>
<fA05>
<s2>27</s2>
</fA05>
<fA06>
<s2>1</s2>
</fA06>
<fA08 i1="01" i2="1" l="ENG">
<s1>Annual Change in Friedreich's Ataxia Evaluated by the Scale for the Assessment and Rating of Ataxia (SARA) is Independent of Disease Severity</s1>
</fA08>
<fA11 i1="01" i2="1">
<s1>MARELLI (Cecilia)</s1>
</fA11>
<fA11 i1="02" i2="1">
<s1>FIGONI (Julie)</s1>
</fA11>
<fA11 i1="03" i2="1">
<s1>CHARLES (Perrine)</s1>
</fA11>
<fA11 i1="04" i2="1">
<s1>ANHEIM (Mathieu)</s1>
</fA11>
<fA11 i1="05" i2="1">
<s1>TCHIKVILADZE (Maya)</s1>
</fA11>
<fA11 i1="06" i2="1">
<s1>VINCITORIO (Carlo-Maria)</s1>
</fA11>
<fA11 i1="07" i2="1">
<s1>DU MONTCEL (Sophie Tezenas)</s1>
</fA11>
<fA11 i1="08" i2="1">
<s1>BRICE (Alexis)</s1>
</fA11>
<fA11 i1="09" i2="1">
<s1>GOLMARD (Jean Louis)</s1>
</fA11>
<fA11 i1="10" i2="1">
<s1>DÜRR (Alexandra)</s1>
</fA11>
<fA14 i1="01">
<s1>Université Pierre et Marie Curie-Paris6, Centre de Recherche de l'Institut du Cerveau et de la Moelle epiniere</s1>
<s2>UMR-S975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</fA14>
<fA14 i1="02">
<s1>Inserm</s1>
<s2>U975, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</fA14>
<fA14 i1="03">
<s1>Cnrs</s1>
<s2>UMR 7225, Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</fA14>
<fA14 i1="04">
<s1>P-HP, Hôpital Pitié-Salpêtriére, Department of Genetics and Cytogenetics</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>10 aut.</sZ>
</fA14>
<fA14 i1="05">
<s1>AP-HP, Department of Biostatistics and Medical Informatics and Pitié-Salpêtriére Charles-Foix Clinical Research Unit</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
</fA14>
<fA14 i1="06">
<s1>AP-HP, Pitié-Salpêtrière Hospital, Federation of the Nervous System Diseases</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>3 aut.</sZ>
<sZ>8 aut.</sZ>
</fA14>
<fA14 i1="07">
<s1>Université Pierre et Marie Curie-Paris6, ER4 (formerly EA 3974), Modeling in Clinical Research</s1>
<s2>Paris</s2>
<s3>FRA</s3>
<sZ>7 aut.</sZ>
<sZ>9 aut.</sZ>
</fA14>
<fA20>
<s1>135-138</s1>
</fA20>
<fA21>
<s1>2012</s1>
</fA21>
<fA23 i1="01">
<s0>ENG</s0>
</fA23>
<fA43 i1="01">
<s1>INIST</s1>
<s2>20953</s2>
<s5>354000508687520210</s5>
</fA43>
<fA44>
<s0>0000</s0>
<s1>© 2012 INIST-CNRS. All rights reserved.</s1>
</fA44>
<fA45>
<s0>15 ref.</s0>
</fA45>
<fA47 i1="01" i2="1">
<s0>12-0106500</s0>
</fA47>
<fA60>
<s1>P</s1>
<s3>CC</s3>
</fA60>
<fA61>
<s0>A</s0>
</fA61>
<fA64 i1="01" i2="1">
<s0>Movement disorders</s0>
</fA64>
<fA66 i1="01">
<s0>USA</s0>
</fA66>
<fC01 i1="01" l="ENG">
<s0>Background: The objective of the study was to evaluate the sensitivity to change of the Scale for the Assessment and Rating of Ataxia (score, 0-40) in Friedreich's ataxia. Methods: This was a follow-up study in adult patients with genetically confirmed Friedreich's ataxia evaluated at least twice (minimum interval, 6 months). Participants were outpatients at the Center for Neurogenetics of the Pitie-Salpêtrière Hospital in Paris. Results: We included 84 patients; 60% had 3 or more evaluations. The mean score on first assessment was 22.7 ± 9, and the mean follow-up was 1.84 ± 1.10 years. The mean increase was 1.36 ± 2.3 points/ year; this variation was not significantly linked to factors known to influence disease severity such as age at onset, disease duration, GAA expansion length, and wheelchair use. Conclusions: In adult Friedreich's ataxia patients the Scale for the Assessment and Rating of Ataxia can detect annual changes independently of disease severity. In future therapeutic trials no patient stratification is globally required.</s0>
</fC01>
<fC02 i1="01" i2="X">
<s0>002B17</s0>
</fC02>
<fC02 i1="02" i2="X">
<s0>002B17G</s0>
</fC02>
<fC03 i1="01" i2="X" l="FRE">
<s0>Hérédodégénérescence spinocérébelleuse de Friedreich</s0>
<s5>01</s5>
</fC03>
<fC03 i1="01" i2="X" l="ENG">
<s0>Friedreich ataxia</s0>
<s5>01</s5>
</fC03>
<fC03 i1="01" i2="X" l="SPA">
<s0>Heredodegeneración espinocerebelosa Friedreich</s0>
<s5>01</s5>
</fC03>
<fC03 i1="02" i2="X" l="FRE">
<s0>Ataxie</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="ENG">
<s0>Ataxia</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="SPA">
<s0>Ataxia</s0>
<s5>02</s5>
</fC03>
<fC03 i1="03" i2="X" l="FRE">
<s0>Pathologie du système nerveux</s0>
<s5>03</s5>
</fC03>
<fC03 i1="03" i2="X" l="ENG">
<s0>Nervous system diseases</s0>
<s5>03</s5>
</fC03>
<fC03 i1="03" i2="X" l="SPA">
<s0>Sistema nervioso patología</s0>
<s5>03</s5>
</fC03>
<fC03 i1="04" i2="X" l="FRE">
<s0>Echelle d'évaluation</s0>
<s5>09</s5>
</fC03>
<fC03 i1="04" i2="X" l="ENG">
<s0>Evaluation scale</s0>
<s5>09</s5>
</fC03>
<fC03 i1="04" i2="X" l="SPA">
<s0>Escala evaluación</s0>
<s5>09</s5>
</fC03>
<fC07 i1="01" i2="X" l="FRE">
<s0>Pathologie de l'encéphale</s0>
<s5>37</s5>
</fC07>
<fC07 i1="01" i2="X" l="ENG">
<s0>Cerebral disorder</s0>
<s5>37</s5>
</fC07>
<fC07 i1="01" i2="X" l="SPA">
<s0>Encéfalo patología</s0>
<s5>37</s5>
</fC07>
<fC07 i1="02" i2="X" l="FRE">
<s0>Maladie dégénérative</s0>
<s5>38</s5>
</fC07>
<fC07 i1="02" i2="X" l="ENG">
<s0>Degenerative disease</s0>
<s5>38</s5>
</fC07>
<fC07 i1="02" i2="X" l="SPA">
<s0>Enfermedad degenerativa</s0>
<s5>38</s5>
</fC07>
<fC07 i1="03" i2="X" l="FRE">
<s0>Maladie héréditaire</s0>
<s5>39</s5>
</fC07>
<fC07 i1="03" i2="X" l="ENG">
<s0>Genetic disease</s0>
<s5>39</s5>
</fC07>
<fC07 i1="03" i2="X" l="SPA">
<s0>Enfermedad hereditaria</s0>
<s5>39</s5>
</fC07>
<fC07 i1="04" i2="X" l="FRE">
<s0>Pathologie de la moelle épinière</s0>
<s5>40</s5>
</fC07>
<fC07 i1="04" i2="X" l="ENG">
<s0>Spinal cord disease</s0>
<s5>40</s5>
</fC07>
<fC07 i1="04" i2="X" l="SPA">
<s0>Médula espinal patología</s0>
<s5>40</s5>
</fC07>
<fC07 i1="05" i2="X" l="FRE">
<s0>Pathologie du système nerveux central</s0>
<s5>41</s5>
</fC07>
<fC07 i1="05" i2="X" l="ENG">
<s0>Central nervous system disease</s0>
<s5>41</s5>
</fC07>
<fC07 i1="05" i2="X" l="SPA">
<s0>Sistema nervosio central patología</s0>
<s5>41</s5>
</fC07>
<fC07 i1="06" i2="X" l="FRE">
<s0>Trouble neurologique</s0>
<s5>43</s5>
</fC07>
<fC07 i1="06" i2="X" l="ENG">
<s0>Neurological disorder</s0>
<s5>43</s5>
</fC07>
<fC07 i1="06" i2="X" l="SPA">
<s0>Trastorno neurológico</s0>
<s5>43</s5>
</fC07>
<fN21>
<s1>079</s1>
</fN21>
<fN44 i1="01">
<s1>OTO</s1>
</fN44>
<fN82>
<s1>OTO</s1>
</fN82>
</pA>
</standard>
</inist>
<affiliations>
<list>
<country>
<li>France</li>
</country>
<settlement>
<li>Paris</li>
</settlement>
</list>
<tree>
<country name="France">
<noRegion>
<name sortKey="Marelli, Cecilia" sort="Marelli, Cecilia" uniqKey="Marelli C" first="Cecilia" last="Marelli">Cecilia Marelli</name>
</noRegion>
<name sortKey="Anheim, Mathieu" sort="Anheim, Mathieu" uniqKey="Anheim M" first="Mathieu" last="Anheim">Mathieu Anheim</name>
<name sortKey="Brice, Alexis" sort="Brice, Alexis" uniqKey="Brice A" first="Alexis" last="Brice">Alexis Brice</name>
<name sortKey="Brice, Alexis" sort="Brice, Alexis" uniqKey="Brice A" first="Alexis" last="Brice">Alexis Brice</name>
<name sortKey="Brice, Alexis" sort="Brice, Alexis" uniqKey="Brice A" first="Alexis" last="Brice">Alexis Brice</name>
<name sortKey="Brice, Alexis" sort="Brice, Alexis" uniqKey="Brice A" first="Alexis" last="Brice">Alexis Brice</name>
<name sortKey="Brice, Alexis" sort="Brice, Alexis" uniqKey="Brice A" first="Alexis" last="Brice">Alexis Brice</name>
<name sortKey="Charles, Perrine" sort="Charles, Perrine" uniqKey="Charles P" first="Perrine" last="Charles">Perrine Charles</name>
<name sortKey="Charles, Perrine" sort="Charles, Perrine" uniqKey="Charles P" first="Perrine" last="Charles">Perrine Charles</name>
<name sortKey="Du Montcel, Sophie Tezenas" sort="Du Montcel, Sophie Tezenas" uniqKey="Du Montcel S" first="Sophie Tezenas" last="Du Montcel">Sophie Tezenas Du Montcel</name>
<name sortKey="Du Montcel, Sophie Tezenas" sort="Du Montcel, Sophie Tezenas" uniqKey="Du Montcel S" first="Sophie Tezenas" last="Du Montcel">Sophie Tezenas Du Montcel</name>
<name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Dürr">Alexandra Dürr</name>
<name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Dürr">Alexandra Dürr</name>
<name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Dürr">Alexandra Dürr</name>
<name sortKey="Durr, Alexandra" sort="Durr, Alexandra" uniqKey="Durr A" first="Alexandra" last="Dürr">Alexandra Dürr</name>
<name sortKey="Figoni, Julie" sort="Figoni, Julie" uniqKey="Figoni J" first="Julie" last="Figoni">Julie Figoni</name>
<name sortKey="Golmard, Jean Louis" sort="Golmard, Jean Louis" uniqKey="Golmard J" first="Jean Louis" last="Golmard">Jean Louis Golmard</name>
<name sortKey="Golmard, Jean Louis" sort="Golmard, Jean Louis" uniqKey="Golmard J" first="Jean Louis" last="Golmard">Jean Louis Golmard</name>
<name sortKey="Marelli, Cecilia" sort="Marelli, Cecilia" uniqKey="Marelli C" first="Cecilia" last="Marelli">Cecilia Marelli</name>
<name sortKey="Marelli, Cecilia" sort="Marelli, Cecilia" uniqKey="Marelli C" first="Cecilia" last="Marelli">Cecilia Marelli</name>
<name sortKey="Marelli, Cecilia" sort="Marelli, Cecilia" uniqKey="Marelli C" first="Cecilia" last="Marelli">Cecilia Marelli</name>
<name sortKey="Tchikviladze, Maya" sort="Tchikviladze, Maya" uniqKey="Tchikviladze M" first="Maya" last="Tchikviladze">Maya Tchikviladze</name>
<name sortKey="Vincitorio, Carlo Maria" sort="Vincitorio, Carlo Maria" uniqKey="Vincitorio C" first="Carlo-Maria" last="Vincitorio">Carlo-Maria Vincitorio</name>
</country>
</tree>
</affiliations>
</record>

Pour manipuler ce document sous Unix (Dilib)

EXPLOR_STEP=$WICRI_ROOT/Wicri/Santé/explor/MovDisordV3/Data/PascalFrancis/Checkpoint
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 000285 | SxmlIndent | more

Ou

HfdSelect -h $EXPLOR_AREA/Data/PascalFrancis/Checkpoint/biblio.hfd -nk 000285 | SxmlIndent | more

Pour mettre un lien sur cette page dans le réseau Wicri

{{Explor lien
   |wiki=    Wicri/Santé
   |area=    MovDisordV3
   |flux=    PascalFrancis
   |étape=   Checkpoint
   |type=    RBID
   |clé=     Pascal:12-0106500
   |texte=   Annual Change in Friedreich's Ataxia Evaluated by the Scale for the Assessment and Rating of Ataxia (SARA) is Independent of Disease Severity
}}

Wicri

This area was generated with Dilib version V0.6.23.
Data generation: Sun Jul 3 12:29:32 2016. Site generation: Wed Feb 14 10:52:30 2024