Pure akinesia with gait freezing: A third clinical phenotype of progressive supranuclear palsy
Identifieur interne : 002C37 ( Main/Curation ); précédent : 002C36; suivant : 002C38Pure 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 ] ; 2007-11-15.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Congélation.
English descriptors
- KwdEn :
- Adult, Aged, Akinesia, Brain (metabolism), Brain (pathology), Fatal Outcome, Female, Freezing, Gait Disorders, Neurologic (classification), Gait Disorders, Neurologic (pathology), Gait Disorders, Neurologic (physiopathology), Humans, Immunohistochemistry, Lewy Bodies (pathology), Male, Middle Aged, Movement Disorders (classification), Movement Disorders (pathology), Movement Disorders (physiopathology), Nervous system diseases, Phenotype, Retrospective Studies, Supranuclear Palsy, Progressive (classification), Supranuclear Palsy, Progressive (pathology), Supranuclear Palsy, Progressive (physiopathology), alpha-Synuclein (metabolism), gait ignition failure, primary progressive freezing gait, progressive supranuclear palsy (PSP), pure akinesia with gait freezing.
- MESH :
- chemical , metabolism : alpha-Synuclein.
- classification : Gait Disorders, Neurologic, Movement Disorders, Supranuclear Palsy, Progressive.
- metabolism : Brain.
- pathology : Brain, Gait Disorders, Neurologic, Lewy Bodies, Movement Disorders, Supranuclear Palsy, Progressive.
- physiopathology : Gait Disorders, Neurologic, Movement Disorders, Supranuclear Palsy, Progressive.
- Adult, Aged, Fatal Outcome, Female, Humans, Immunohistochemistry, Male, Middle Aged, Phenotype, Retrospective Studies.
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. © 2007 Movement Disorder Society
Url:
DOI: 10.1002/mds.21698
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<term>Aged</term>
<term>Akinesia</term>
<term>Brain (metabolism)</term>
<term>Brain (pathology)</term>
<term>Fatal Outcome</term>
<term>Female</term>
<term>Freezing</term>
<term>Gait Disorders, Neurologic (classification)</term>
<term>Gait Disorders, Neurologic (pathology)</term>
<term>Gait Disorders, Neurologic (physiopathology)</term>
<term>Humans</term>
<term>Immunohistochemistry</term>
<term>Lewy Bodies (pathology)</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Movement Disorders (classification)</term>
<term>Movement Disorders (pathology)</term>
<term>Movement Disorders (physiopathology)</term>
<term>Nervous system diseases</term>
<term>Phenotype</term>
<term>Retrospective Studies</term>
<term>Supranuclear Palsy, Progressive (classification)</term>
<term>Supranuclear Palsy, Progressive (pathology)</term>
<term>Supranuclear Palsy, Progressive (physiopathology)</term>
<term>alpha-Synuclein (metabolism)</term>
<term>gait ignition failure</term>
<term>primary progressive freezing gait</term>
<term>progressive supranuclear palsy (PSP)</term>
<term>pure akinesia with gait freezing</term>
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<term>Movement Disorders</term>
<term>Supranuclear Palsy, Progressive</term>
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<term>Movement Disorders</term>
<term>Supranuclear Palsy, Progressive</term>
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<term>Movement Disorders</term>
<term>Supranuclear Palsy, Progressive</term>
</keywords>
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<term>Aged</term>
<term>Fatal Outcome</term>
<term>Female</term>
<term>Humans</term>
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<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. © 2007 Movement Disorder Society</div>
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<region type="région" nuts="1">Grand Londres</region>
</placeName>
</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>
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</placeName>
</affiliation>
</author>
<author><name sortKey="Revesz, Tamas" sort="Revesz, Tamas" uniqKey="Revesz T" first="Tamas" last="Revesz">Tamas Revesz</name>
<affiliation wicri:level="3"><inist:fA14 i1="03"><s1>Queen Square Brain Bank for Neurological Disorders</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>
</placeName>
</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>
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<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>
</placeName>
</affiliation>
</author>
<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>
<sZ>1 aut.</sZ>
<sZ>2 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>
</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>
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<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>
</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>
</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>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">INIST</idno>
<idno type="inist">08-0071411</idno>
<date when="2007">2007</date>
<idno type="stanalyst">PASCAL 08-0071411 INIST</idno>
<idno type="RBID">Pascal:08-0071411</idno>
<idno type="wicri:Area/PascalFrancis/Corpus">001450</idno>
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<idno type="wicri:Area/PascalFrancis/Checkpoint">001504</idno>
<idno type="wicri:doubleKey">0885-3185:2007:Williams D:pure:akinesia:with</idno>
<idno type="wicri:Area/Main/Merge">004094</idno>
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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>
</inist:fA14>
<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>
<sZ>1 aut.</sZ>
<sZ>2 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>
</placeName>
</affiliation>
<affiliation wicri:level="3"><inist:fA14 i1="03"><s1>Queen Square Brain Bank for Neurological Disorders</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>
</placeName>
</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>
</placeName>
</affiliation>
</author>
<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>
<s3>GBR</s3>
<sZ>1 aut.</sZ>
<sZ>2 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>
</placeName>
</affiliation>
<affiliation wicri:level="3"><inist:fA14 i1="03"><s1>Queen Square Brain Bank for Neurological Disorders</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>
</placeName>
</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>
</placeName>
</affiliation>
</author>
<author><name sortKey="Strand, Kate" sort="Strand, Kate" uniqKey="Strand K" first="Kate" last="Strand">Kate Strand</name>
<affiliation wicri:level="3"><inist:fA14 i1="03"><s1>Queen Square Brain Bank for Neurological Disorders</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>
</placeName>
</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>
</placeName>
</affiliation>
</author>
<author><name sortKey="Revesz, Tamas" sort="Revesz, Tamas" uniqKey="Revesz T" first="Tamas" last="Revesz">Tamas Revesz</name>
<affiliation wicri:level="3"><inist:fA14 i1="03"><s1>Queen Square Brain Bank for Neurological Disorders</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>
</placeName>
</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>
</placeName>
</affiliation>
</author>
<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>
<sZ>1 aut.</sZ>
<sZ>2 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>
</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="03"><s1>Queen Square Brain Bank for Neurological Disorders</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>
</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>
</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>
</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="2007">2007</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>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>
</textClass>
</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>
</INIST>
<ISTEX><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">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>
</author>
<author><name sortKey="Holton, Janice L" sort="Holton, Janice L" uniqKey="Holton J" first="Janice L." last="Holton">Janice L. Holton</name>
</author>
<author><name sortKey="Strand, Kate" sort="Strand, Kate" uniqKey="Strand K" first="Kate" last="Strand">Kate Strand</name>
</author>
<author><name sortKey="Revesz, Tamas" sort="Revesz, Tamas" uniqKey="Revesz T" first="Tamas" last="Revesz">Tamas Revesz</name>
</author>
<author><name sortKey="Lees, Andrew J" sort="Lees, Andrew J" uniqKey="Lees A" first="Andrew J." last="Lees">Andrew Lees (neurologue)</name>
<affiliation><country>Royaume-Uni</country>
<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>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">ISTEX</idno>
<idno type="RBID">ISTEX:F95BAE189F1D08DBF9647093EB25277703D69730</idno>
<date when="2007" year="2007">2007</date>
<idno type="doi">10.1002/mds.21698</idno>
<idno type="url">https://api.istex.fr/document/F95BAE189F1D08DBF9647093EB25277703D69730/fulltext/pdf</idno>
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<idno type="wicri:source">PubMed</idno>
<idno type="RBID">pubmed:17712855</idno>
<idno type="wicri:Area/PubMed/Corpus">002546</idno>
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<idno type="wicri:Area/Main/Merge">003B59</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">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"><country xml:lang="fr">Australie</country>
<wicri:regionArea>Faculty of Medicine (Neurosciences), Monash University (Alfred Hospital Campus), Melbourne</wicri:regionArea>
<wicri:noRegion>Melbourne</wicri:noRegion>
</affiliation>
<affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Reta Lila Weston Institute of Neurological Studies, London</wicri:regionArea>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
</affiliation>
<affiliation wicri:level="3"><country>Royaume-Uni</country>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
<wicri:orgArea>Queen Square Brain Bank for Neurological Disorders</wicri:orgArea>
</affiliation>
<affiliation wicri:level="3"><country>Royaume-Uni</country>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
<wicri:orgArea>Department of Molecular Neuroscience, Institute of Neurology, UCL</wicri:orgArea>
</affiliation>
</author>
<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"><country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Reta Lila Weston Institute of Neurological Studies, London</wicri:regionArea>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
</affiliation>
<affiliation wicri:level="3"><country>Royaume-Uni</country>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
<wicri:orgArea>Queen Square Brain Bank for Neurological Disorders</wicri:orgArea>
</affiliation>
<affiliation wicri:level="3"><country>Royaume-Uni</country>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
<wicri:orgArea>Department of Molecular Neuroscience, Institute of Neurology, UCL</wicri:orgArea>
</affiliation>
</author>
<author><name sortKey="Strand, Kate" sort="Strand, Kate" uniqKey="Strand K" first="Kate" last="Strand">Kate Strand</name>
<affiliation wicri:level="3"><country>Royaume-Uni</country>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
<wicri:orgArea>Queen Square Brain Bank for Neurological Disorders</wicri:orgArea>
</affiliation>
<affiliation wicri:level="3"><country>Royaume-Uni</country>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
<wicri:orgArea>Department of Molecular Neuroscience, Institute of Neurology, UCL</wicri:orgArea>
</affiliation>
</author>
<author><name sortKey="Revesz, Tamas" sort="Revesz, Tamas" uniqKey="Revesz T" first="Tamas" last="Revesz">Tamas Revesz</name>
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<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
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</placeName>
<wicri:orgArea>Queen Square Brain Bank for Neurological Disorders</wicri:orgArea>
</affiliation>
<affiliation wicri:level="3"><country>Royaume-Uni</country>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
<wicri:orgArea>Department of Molecular Neuroscience, Institute of Neurology, UCL</wicri:orgArea>
</affiliation>
</author>
<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"><country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Reta Lila Weston Institute of Neurological Studies, London</wicri:regionArea>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
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<placeName><settlement type="city">Londres</settlement>
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</placeName>
<orgName>National Hospital for Neurology and Neurosurgery</orgName>
</affiliation>
<affiliation wicri:level="3"><country>Royaume-Uni</country>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
<wicri:orgArea>Queen Square Brain Bank for Neurological Disorders</wicri:orgArea>
<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
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</placeName>
<orgName>National Hospital for Neurology and Neurosurgery</orgName>
</affiliation>
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<placeName><settlement type="city">Londres</settlement>
<region type="country">Angleterre</region>
<region type="région" nuts="1">Grand Londres</region>
</placeName>
<wicri:orgArea>Department of Molecular Neuroscience, Institute of Neurology, UCL</wicri:orgArea>
<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>
</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="2007-11-15">2007-11-15</date>
<biblScope unit="vol">22</biblScope>
<biblScope unit="issue">15</biblScope>
<biblScope unit="page" from="2235">2235</biblScope>
<biblScope unit="page" to="2241">2241</biblScope>
</imprint>
<idno type="ISSN">0885-3185</idno>
</series>
<idno type="istex">F95BAE189F1D08DBF9647093EB25277703D69730</idno>
<idno type="DOI">10.1002/mds.21698</idno>
<idno type="ArticleID">MDS21698</idno>
</biblStruct>
</sourceDesc>
<seriesStmt><idno type="ISSN">0885-3185</idno>
</seriesStmt>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term>
<term>Aged</term>
<term>Brain (metabolism)</term>
<term>Brain (pathology)</term>
<term>Fatal Outcome</term>
<term>Female</term>
<term>Gait Disorders, Neurologic (classification)</term>
<term>Gait Disorders, Neurologic (pathology)</term>
<term>Gait Disorders, Neurologic (physiopathology)</term>
<term>Humans</term>
<term>Immunohistochemistry</term>
<term>Lewy Bodies (pathology)</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Movement Disorders (classification)</term>
<term>Movement Disorders (pathology)</term>
<term>Movement Disorders (physiopathology)</term>
<term>Phenotype</term>
<term>Retrospective Studies</term>
<term>Supranuclear Palsy, Progressive (classification)</term>
<term>Supranuclear Palsy, Progressive (pathology)</term>
<term>Supranuclear Palsy, Progressive (physiopathology)</term>
<term>alpha-Synuclein (metabolism)</term>
<term>gait ignition failure</term>
<term>primary progressive freezing gait</term>
<term>progressive supranuclear palsy (PSP)</term>
<term>pure akinesia with gait freezing</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>alpha-Synuclein</term>
</keywords>
<keywords scheme="MESH" qualifier="classification" xml:lang="en"><term>Gait Disorders, Neurologic</term>
<term>Movement Disorders</term>
<term>Supranuclear Palsy, Progressive</term>
</keywords>
<keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Brain</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain</term>
<term>Gait Disorders, Neurologic</term>
<term>Lewy Bodies</term>
<term>Movement Disorders</term>
<term>Supranuclear Palsy, Progressive</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Gait Disorders, Neurologic</term>
<term>Movement Disorders</term>
<term>Supranuclear Palsy, Progressive</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adult</term>
<term>Aged</term>
<term>Fatal Outcome</term>
<term>Female</term>
<term>Humans</term>
<term>Immunohistochemistry</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Phenotype</term>
<term>Retrospective Studies</term>
</keywords>
</textClass>
<langUsage><language ident="en">en</language>
</langUsage>
</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. © 2007 Movement Disorder Society</div>
</front>
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