MovDisordV3, Main, Merge, bibRecord, 004094

Pure Akinesia with Gait Freezing : A Third Clinical Phenotype of Progressive Supranuclear Palsy

Identifieur interne : 004094 ( Main/Merge ); précédent : 004093; suivant : 004095

Pure Akinesia with Gait Freezing : A Third Clinical Phenotype of Progressive Supranuclear Palsy

Auteurs : David R. Williams [Australie, Royaume-Uni] ; Janice L. Holton [Royaume-Uni] ; Kate Strand [Royaume-Uni] ; Tamas Revesz [Royaume-Uni] ; Andrew Lees (neurologue) [Royaume-Uni]

Source :

Movement disorders [ 0885-3185 ] ; 2007.

RBID : Pascal:08-0071411

Descripteurs français

Pascal (Inist)
- Pathologie du système nerveux, Akinésie, Congélation, Phénotype.
Wicri :
- topic : Congélation.

English descriptors

KwdEn :
- Akinesia, Freezing, Nervous system diseases, Phenotype.

Abstract

The clinical syndrome of pure akinesia has most often been associated with progressive supranuclear palsy (PSP) and is characterized by difficulty initiating gait and "freezing" during walking, writing and speaking. Similar syndromes have been described under the rubrics of primary progressive freezing gait and primary gait ignition failure. We investigated the specificity of the clinical syndrome of pure akinesia with gait freezing (PAGF) for PSP-tau pathology. Among 749 patients archived at the QSBB, only 7 fulfilled proposed diagnostic criteria of: gradual onset of freezing of gait or speech; absent limb rigidity and tremor; no sustained response to levodopa; and no dementia or ophthalmoplegia in the first 5 years of disease. In these cases detailed pathological examination was performed. PSP was the pathological diagnosis in six patients, and Parkinson's disease (PD) in the seventh. As defined, this syndrome had a positive predictive value of 86% for PSP-tau pathology. In the cases with PSP there were no additional features of coexistent vascular or PD and the median PSP-tau score was 3, reflecting relative mild tau load. The clinical syndrome of PAGF appears to have a high specificity for PSP-tau pathology. This relatively uncommon presentation of PSP-tau pathology has less severe tau accumulation than in the more common, "classic" PSP clinical phenotype: Richardson's disease.

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

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to stream PascalFrancis, to step Curation: 001869
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Links to Exploration step

Pascal:08-0071411

Le document en format XML

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<s2>London</s2>
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<placeName><settlement type="city">Londres</settlement>
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<orgName>National Hospital for Neurology and Neurosurgery</orgName>
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Pure Akinesia with Gait Freezing : A Third Clinical Phenotype of Progressive Supranuclear Palsy</title>
<author><name sortKey="Williams, David R" sort="Williams, David R" uniqKey="Williams D" first="David R." last="Williams">David R. Williams</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Faculty of Medicine (Neurosciences), Monash University (Alfred Hospital Campus)</s1>
<s2>Melbourne</s2>
<s3>AUS</s3>
<sZ>1 aut.</sZ>
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<country>Australie</country>
<wicri:noRegion>Melbourne</wicri:noRegion>
</affiliation>
<affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Reta Lila Weston Institute of Neurological Studies</s1>
<s2>London</s2>
<s3>GBR</s3>
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<region type="country">Angleterre</region>
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<s2>London</s2>
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</placeName>
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<affiliation wicri:level="3"><inist:fA14 i1="04"><s1>Department of Molecular Neuroscience, Institute of Neurology, UCL</s1>
<s2>London</s2>
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<author><name sortKey="Holton, Janice L" sort="Holton, Janice L" uniqKey="Holton J" first="Janice L." last="Holton">Janice L. Holton</name>
<affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Reta Lila Weston Institute of Neurological Studies</s1>
<s2>London</s2>
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<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
</affiliation>
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<s2>London</s2>
<s3>GBR</s3>
<sZ>1 aut.</sZ>
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<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
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</inist:fA14>
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<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
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<s2>London</s2>
<s3>GBR</s3>
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<sZ>4 aut.</sZ>
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<region type="country">Angleterre</region>
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<author><name sortKey="Strand, Kate" sort="Strand, Kate" uniqKey="Strand K" first="Kate" last="Strand">Kate Strand</name>
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<author><name sortKey="Revesz, Tamas" sort="Revesz, Tamas" uniqKey="Revesz T" first="Tamas" last="Revesz">Tamas Revesz</name>
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<s2>London</s2>
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<author><name sortKey="Lees, Andrew J" sort="Lees, Andrew J" uniqKey="Lees A" first="Andrew J." last="Lees">Andrew Lees (neurologue)</name>
<affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Reta Lila Weston Institute of Neurological Studies</s1>
<s2>London</s2>
<s3>GBR</s3>
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<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
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<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
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<orgName>National Hospital for Neurology and Neurosurgery</orgName>
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<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
<orgName>National Hospital for Neurology and Neurosurgery</orgName>
</affiliation>
<affiliation wicri:level="3"><inist:fA14 i1="04"><s1>Department of Molecular Neuroscience, Institute of Neurology, UCL</s1>
<s2>London</s2>
<s3>GBR</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
</inist:fA14>
<country>Royaume-Uni</country>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
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<region type="région" nuts="1">Grand Londres</region>
</placeName>
<orgName>National Hospital for Neurology and Neurosurgery</orgName>
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<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="2007">2007</date>
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<seriesStmt><title level="j" type="main">Movement disorders</title>
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<idno type="ISSN">0885-3185</idno>
</seriesStmt>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Akinesia</term>
<term>Freezing</term>
<term>Nervous system diseases</term>
<term>Phenotype</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr"><term>Pathologie du   système nerveux</term>
<term>Akinésie</term>
<term>Congélation</term>
<term>Phénotype</term>
</keywords>
<keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Congélation</term>
</keywords>
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</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">The clinical syndrome of pure akinesia has most often been associated with progressive supranuclear palsy (PSP) and is characterized by difficulty initiating gait and "freezing" during walking, writing and speaking. Similar syndromes have been described under the rubrics of primary progressive freezing gait and primary gait ignition failure. We investigated the specificity of the clinical syndrome of pure akinesia with gait freezing (PAGF) for PSP-tau pathology. Among 749 patients archived at the QSBB, only 7 fulfilled proposed diagnostic criteria of: gradual onset of freezing of gait or speech; absent limb rigidity and tremor; no sustained response to levodopa; and no dementia or ophthalmoplegia in the first 5 years of disease. In these cases detailed pathological examination was performed. PSP was the pathological diagnosis in six patients, and Parkinson's disease (PD) in the seventh. As defined, this syndrome had a positive predictive value of 86% for PSP-tau pathology. In the cases with PSP there were no additional features of coexistent vascular or PD and the median PSP-tau score was 3, reflecting relative mild tau load. The clinical syndrome of PAGF appears to have a high specificity for PSP-tau pathology. This relatively uncommon presentation of PSP-tau pathology has less severe tau accumulation than in the more common, "classic" PSP clinical phenotype: Richardson's disease.</div>
</front>
</TEI>
<affiliations><list><country><li>Australie</li>
<li>Royaume-Uni</li>
</country>
<region><li>Angleterre</li>
<li>Grand Londres</li>
</region>
<settlement><li>Londres</li>
</settlement>
<orgName><li>National Hospital for Neurology and Neurosurgery</li>
</orgName>
</list>
<tree><country name="Australie"><noRegion><name sortKey="Williams, David R" sort="Williams, David R" uniqKey="Williams D" first="David R." last="Williams">David R. Williams</name>
</noRegion>
</country>
<country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Williams, David R" sort="Williams, David R" uniqKey="Williams D" first="David R." last="Williams">David R. Williams</name>
</region>
<name sortKey="Holton, Janice L" sort="Holton, Janice L" uniqKey="Holton J" first="Janice L." last="Holton">Janice L. Holton</name>
<name sortKey="Holton, Janice L" sort="Holton, Janice L" uniqKey="Holton J" first="Janice L." last="Holton">Janice L. Holton</name>
<name sortKey="Holton, Janice L" sort="Holton, Janice L" uniqKey="Holton J" first="Janice L." last="Holton">Janice L. Holton</name>
<name sortKey="Lees, Andrew J" sort="Lees, Andrew J" uniqKey="Lees A" first="Andrew J." last="Lees">Andrew Lees (neurologue)</name>
<name sortKey="Lees, Andrew J" sort="Lees, Andrew J" uniqKey="Lees A" first="Andrew J." last="Lees">Andrew Lees (neurologue)</name>
<name sortKey="Lees, Andrew J" sort="Lees, Andrew J" uniqKey="Lees A" first="Andrew J." last="Lees">Andrew Lees (neurologue)</name>
<name sortKey="Revesz, Tamas" sort="Revesz, Tamas" uniqKey="Revesz T" first="Tamas" last="Revesz">Tamas Revesz</name>
<name sortKey="Revesz, Tamas" sort="Revesz, Tamas" uniqKey="Revesz T" first="Tamas" last="Revesz">Tamas Revesz</name>
<name sortKey="Strand, Kate" sort="Strand, Kate" uniqKey="Strand K" first="Kate" last="Strand">Kate Strand</name>
<name sortKey="Strand, Kate" sort="Strand, Kate" uniqKey="Strand K" first="Kate" last="Strand">Kate Strand</name>
<name sortKey="Williams, David R" sort="Williams, David R" uniqKey="Williams D" first="David R." last="Williams">David R. Williams</name>
<name sortKey="Williams, David R" sort="Williams, David R" uniqKey="Williams D" first="David R." last="Williams">David R. Williams</name>
</country>
</tree>
</affiliations>
</record>

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	Movement Disorders (revue)
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Movement Disorders (revue)

Pure Akinesia with Gait Freezing : A Third Clinical Phenotype of Progressive Supranuclear Palsy

Pure Akinesia with Gait Freezing : A Third Clinical Phenotype of Progressive Supranuclear Palsy

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