MovDisordV3, Main, Merge, bibRecord, 003B59

Pure akinesia with gait freezing: A third clinical phenotype of progressive supranuclear palsy

Identifieur interne : 003B59 ( Main/Merge ); précédent : 003B58; suivant : 003B60

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 ] ; 2007-11-15.

RBID : ISTEX:F95BAE189F1D08DBF9647093EB25277703D69730

English descriptors

KwdEn :
- Adult, Aged, Brain (metabolism), Brain (pathology), Fatal Outcome, Female, 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), 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:

https://api.istex.fr/document/F95BAE189F1D08DBF9647093EB25277703D69730/fulltext/pdf

DOI: 10.1002/mds.21698

Links to Exploration step

ISTEX:F95BAE189F1D08DBF9647093EB25277703D69730

Le document en format XML

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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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<wicri:orgArea>Queen Square Brain Bank for Neurological Disorders</wicri:orgArea>
<placeName><settlement type="city">Londres</settlement>
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<region type="country">Angleterre</region>
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</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>
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<orgName>National Hospital for Neurology and Neurosurgery</orgName>
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<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>
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<pubPlace>Hoboken</pubPlace>
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<term>primary progressive freezing gait</term>
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<term>pure akinesia with gait freezing</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>
</front>
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<PubMed><TEI><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>
<affiliation wicri:level="1"><nlm:affiliation>Faculty of Medicine (Neurosciences), Monash University (Alfred Hospital Campus), Melbourne, Australia. david.williams@med.monash.edu.au</nlm:affiliation>
<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>
</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 J. Lees</name>
</author>
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<date when="2007">2007</date>
<idno type="doi">10.1002/mds.21698</idno>
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<sourceDesc><biblStruct><analytic><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>
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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>
</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 J. Lees</name>
</author>
</analytic>
<series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title>
<idno type="ISSN">0885-3185</idno>
<imprint><date when="2007" type="published">2007</date>
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<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>
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<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>
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</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>
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<term>Humans</term>
<term>Immunohistochemistry</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.</div>
</front>
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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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	Movement Disorders (revue)
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