MovDisordV3, PascalFrancis, Curation, bibRecord, 000751

Progressive supranuclear palsy diagnosis and confounding features: Report on 16 autopsied cases

Identifieur interne : 000751 ( PascalFrancis/Curation ); précédent : 000750; suivant : 000752

Progressive supranuclear palsy diagnosis and confounding features: Report on 16 autopsied cases

Auteurs : Sam Birdi [Canada] ; Ali H. Rajput [Canada] ; Mark Fenton [Canada] ; Jeffery R. Donat [Canada] ; Bohdan Rozdilsky [Canada] ; Christopher Robinson [Canada] ; Rob Macaulay [Canada] ; David George [Canada]

Source :

Movement disorders [ 0885-3185 ] ; 2002.

RBID : Pascal:03-0040684

Descripteurs français

Pascal (Inist)
- Ophtalmoplégie supranucléaire, Progressif, Lévodopa, Autopsie, Diagnostic, Symptomatologie, Homme.
Wicri :
- topic : Homme.

English descriptors

KwdEn :
- Autopsy, Diagnosis, Human, Levodopa, Progressive, Supranuclear ophthalmoplegia, Symptomatology.

Abstract

We evaluated 16 (15 men, 1 woman) autopsy-verified progressive supranuclear palsy (PSP) cases during 31 years (1969-2000) for clinical diagnosis and the course of the disease. The onset was gait difficulty or postural instability in 9 (56.3%), general motor slowing in 3 (18.8%), and tremor in 2. One case had onset with cognitive decline and I as hemidystonia. Four cases had supranuclear ophthalmoplegia (SNO) at the first assessment and were diagnosed as PSP. By last assessment, PSP diagnosis was made in 4 additional cases, but in 8 (50%) who never manifested ophthalmoplegia (mean 9.8 years after onset), PSP diagnosis was not made. Other manifestations included bulbar symptoms in 13 (81.3%), and cognitive impairment in 10 (62.5%) during the course of illness. Fifteen cases received levodopa, amantadine, anticholinergics, dopamine agonists, and selegiline in different combinations with symptomatic benefit in 9 of 15 (60%). Five had some improvement on levodopa alone and 3 showed more improvement when a dopamine agonist was added to levodopa. In general, the benefit was minimal and occurred only early in the course of illness. The mean age at onset was 63.7 (range, 53-85) years. Mean duration at PSP diagnosis was 4.8 (range, 2-11) years. Mean survival was 8.6 (range, 3-24) years and mean age at death was 72.3 (range, 60-89) years. When the different diagnostic criteria recommended in the literature were used, the accuracy of clinical diagnosis did not improve substantially.

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A08	`01`	`1`	`ENG`	`@1 Progressive supranuclear palsy diagnosis and confounding features: Report on 16 autopsied cases`
A11	`01`	`1`		`@1 BIRDI (Sam)`
A11	`02`	`1`		`@1 RAJPUT (Ali H.)`
A11	`03`	`1`		`@1 FENTON (Mark)`
A11	`04`	`1`		`@1 DONAT (Jeffery R.)`
A11	`05`	`1`		`@1 ROZDILSKY (Bohdan)`
A11	`06`	`1`		`@1 ROBINSON (Christopher)`
A11	`07`	`1`		`@1 MACAULAY (Rob)`
A11	`08`	`1`		`@1 GEORGE (David)`
A14	`01`			`@1 Division of Neurology, University of Saskatchewan @2 Saskatoon, Saskatchewan @3 CAN @Z 1 aut. @Z 2 aut. @Z 3 aut. @Z 4 aut.`
A14	`02`			`@1 Department of Pathology, University of Saskatchewan @2 Saskatoon, Saskatchewan @3 CAN @Z 5 aut. @Z 6 aut.`
A14	`03`			`@1 Department of Pathology, University of Dalhousie @2 Halifax, Nova Scotia @3 CAN @Z 7 aut.`
A14	`04`			`@1 Department of Pathology, University of Calgary @2 Calgary, Alberta @3 CAN @Z 8 aut.`
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C01	`01`		`ENG`	@0 We evaluated 16 (15 men, 1 woman) autopsy-verified progressive supranuclear palsy (PSP) cases during 31 years (1969-2000) for clinical diagnosis and the course of the disease. The onset was gait difficulty or postural instability in 9 (56.3%), general motor slowing in 3 (18.8%), and tremor in 2. One case had onset with cognitive decline and I as hemidystonia. Four cases had supranuclear ophthalmoplegia (SNO) at the first assessment and were diagnosed as PSP. By last assessment, PSP diagnosis was made in 4 additional cases, but in 8 (50%) who never manifested ophthalmoplegia (mean 9.8 years after onset), PSP diagnosis was not made. Other manifestations included bulbar symptoms in 13 (81.3%), and cognitive impairment in 10 (62.5%) during the course of illness. Fifteen cases received levodopa, amantadine, anticholinergics, dopamine agonists, and selegiline in different combinations with symptomatic benefit in 9 of 15 (60%). Five had some improvement on levodopa alone and 3 showed more improvement when a dopamine agonist was added to levodopa. In general, the benefit was minimal and occurred only early in the course of illness. The mean age at onset was 63.7 (range, 53-85) years. Mean duration at PSP diagnosis was 4.8 (range, 2-11) years. Mean survival was 8.6 (range, 3-24) years and mean age at death was 72.3 (range, 60-89) years. When the different diagnostic criteria recommended in the literature were used, the accuracy of clinical diagnosis did not improve substantially.
C02	`01`	`X`		`@0 002B17G`
C02	`02`	`X`		`@0 002B09M`
C03	`01`	`X`	`FRE`	`@0 Ophtalmoplégie supranucléaire @5 01`
C03	`01`	`X`	`ENG`	`@0 Supranuclear ophthalmoplegia @5 01`
C03	`01`	`X`	`SPA`	`@0 Oftalmoplejía supranuclear @5 01`
C03	`02`	`X`	`FRE`	`@0 Progressif @5 02`
C03	`02`	`X`	`ENG`	`@0 Progressive @5 02`
C03	`02`	`X`	`SPA`	`@0 Progresivo @5 02`
C03	`03`	`X`	`FRE`	`@0 Lévodopa @2 NK @2 FR @5 04`
C03	`03`	`X`	`ENG`	`@0 Levodopa @2 NK @2 FR @5 04`
C03	`03`	`X`	`SPA`	`@0 Levodopa @2 NK @2 FR @5 04`
C03	`04`	`X`	`FRE`	`@0 Autopsie @5 16`
C03	`04`	`X`	`ENG`	`@0 Autopsy @5 16`
C03	`04`	`X`	`SPA`	`@0 Autopsia @5 16`
C03	`05`	`X`	`FRE`	`@0 Diagnostic @5 17`
C03	`05`	`X`	`ENG`	`@0 Diagnosis @5 17`
C03	`05`	`X`	`SPA`	`@0 Diagnóstico @5 17`
C03	`06`	`X`	`FRE`	`@0 Symptomatologie @5 18`
C03	`06`	`X`	`ENG`	`@0 Symptomatology @5 18`
C03	`06`	`X`	`SPA`	`@0 Sintomatología @5 18`
C03	`07`	`X`	`FRE`	`@0 Homme @5 20`
C03	`07`	`X`	`ENG`	`@0 Human @5 20`
C03	`07`	`X`	`SPA`	`@0 Hombre @5 20`
C07	`01`	`X`	`FRE`	`@0 Oeil pathologie @5 37`
C07	`01`	`X`	`ENG`	`@0 Eye disease @5 37`
C07	`01`	`X`	`SPA`	`@0 Ojo patología @5 37`
C07	`02`	`X`	`FRE`	`@0 Oculomotricité syndrome @5 38`
C07	`02`	`X`	`ENG`	`@0 Oculomotor syndrome @5 38`
C07	`02`	`X`	`SPA`	`@0 Oculomotricidad síndrome @5 38`
C07	`03`	`X`	`FRE`	`@0 Système nerveux pathologie @5 39`
C07	`03`	`X`	`ENG`	`@0 Nervous system diseases @5 39`
C07	`03`	`X`	`SPA`	`@0 Sistema nervioso patología @5 39`
C07	`04`	`X`	`FRE`	`@0 Système nerveux central pathologie @5 40`
C07	`04`	`X`	`ENG`	`@0 Central nervous system disease @5 40`
C07	`04`	`X`	`SPA`	`@0 Sistema nervosio central patología @5 40`
C07	`05`	`X`	`FRE`	`@0 Tronc cérébral syndrome @5 41`
C07	`05`	`X`	`ENG`	`@0 Brain stem syndrome @5 41`
C07	`05`	`X`	`SPA`	`@0 Tallo encefalico sindrome @5 41`
C07	`06`	`X`	`FRE`	`@0 Encéphale pathologie @5 42`
C07	`06`	`X`	`ENG`	`@0 Cerebral disorder @5 42`
C07	`06`	`X`	`SPA`	`@0 Encéfalo patología @5 42`
C07	`07`	`X`	`FRE`	`@0 Maladie dégénérative @5 43`
C07	`07`	`X`	`ENG`	`@0 Degenerative disease @5 43`
C07	`07`	`X`	`SPA`	`@0 Enfermedad degenerativa @5 43`
N21				`@1 020`
N82				`@1 PSI`

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Pascal:03-0040684

Le document en format XML

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<front><div type="abstract" xml:lang="en">We evaluated 16 (15 men, 1 woman) autopsy-verified progressive supranuclear palsy (PSP) cases during 31 years (1969-2000) for clinical diagnosis and the course of the disease. The onset was gait difficulty or postural instability in 9 (56.3%), general motor slowing in 3 (18.8%), and tremor in 2. One case had onset with cognitive decline and I as hemidystonia. Four cases had supranuclear ophthalmoplegia (SNO) at the first assessment and were diagnosed as PSP. By last assessment, PSP diagnosis was made in 4 additional cases, but in 8 (50%) who never manifested ophthalmoplegia (mean 9.8 years after onset), PSP diagnosis was not made. Other manifestations included bulbar symptoms in 13 (81.3%), and cognitive impairment in 10 (62.5%) during the course of illness. Fifteen cases received levodopa, amantadine, anticholinergics, dopamine agonists, and selegiline in different combinations with symptomatic benefit in 9 of 15 (60%). Five had some improvement on levodopa alone and 3 showed more improvement when a dopamine agonist was added to levodopa. In general, the benefit was minimal and occurred only early in the course of illness. The mean age at onset was 63.7 (range, 53-85) years. Mean duration at PSP diagnosis was 4.8 (range, 2-11) years. Mean survival was 8.6 (range, 3-24) years and mean age at death was 72.3 (range, 60-89) years. When the different diagnostic criteria recommended in the literature were used, the accuracy of clinical diagnosis did not improve substantially.</div>
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<fA11 i1="01" i2="1"><s1>BIRDI (Sam)</s1>
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<fA11 i1="02" i2="1"><s1>RAJPUT (Ali H.)</s1>
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<fA11 i1="03" i2="1"><s1>FENTON (Mark)</s1>
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<fA11 i1="04" i2="1"><s1>DONAT (Jeffery R.)</s1>
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<fA14 i1="01"><s1>Division of Neurology, University of Saskatchewan</s1>
<s2>Saskatoon, Saskatchewan</s2>
<s3>CAN</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
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</fA14>
<fA14 i1="02"><s1>Department of Pathology, University of Saskatchewan</s1>
<s2>Saskatoon, Saskatchewan</s2>
<s3>CAN</s3>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
</fA14>
<fA14 i1="03"><s1>Department of Pathology, University of Dalhousie</s1>
<s2>Halifax, Nova Scotia</s2>
<s3>CAN</s3>
<sZ>7 aut.</sZ>
</fA14>
<fA14 i1="04"><s1>Department of Pathology, University of Calgary</s1>
<s2>Calgary, Alberta</s2>
<s3>CAN</s3>
<sZ>8 aut.</sZ>
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<fA66 i1="01"><s0>USA</s0>
</fA66>
<fC01 i1="01" l="ENG"><s0>We evaluated 16 (15 men, 1 woman) autopsy-verified progressive supranuclear palsy (PSP) cases during 31 years (1969-2000) for clinical diagnosis and the course of the disease. The onset was gait difficulty or postural instability in 9 (56.3%), general motor slowing in 3 (18.8%), and tremor in 2. One case had onset with cognitive decline and I as hemidystonia. Four cases had supranuclear ophthalmoplegia (SNO) at the first assessment and were diagnosed as PSP. By last assessment, PSP diagnosis was made in 4 additional cases, but in 8 (50%) who never manifested ophthalmoplegia (mean 9.8 years after onset), PSP diagnosis was not made. Other manifestations included bulbar symptoms in 13 (81.3%), and cognitive impairment in 10 (62.5%) during the course of illness. Fifteen cases received levodopa, amantadine, anticholinergics, dopamine agonists, and selegiline in different combinations with symptomatic benefit in 9 of 15 (60%). Five had some improvement on levodopa alone and 3 showed more improvement when a dopamine agonist was added to levodopa. In general, the benefit was minimal and occurred only early in the course of illness. The mean age at onset was 63.7 (range, 53-85) years. Mean duration at PSP diagnosis was 4.8 (range, 2-11) years. Mean survival was 8.6 (range, 3-24) years and mean age at death was 72.3 (range, 60-89) years. When the different diagnostic criteria recommended in the literature were used, the accuracy of clinical diagnosis did not improve substantially.</s0>
</fC01>
<fC02 i1="01" i2="X"><s0>002B17G</s0>
</fC02>
<fC02 i1="02" i2="X"><s0>002B09M</s0>
</fC02>
<fC03 i1="01" i2="X" l="FRE"><s0>Ophtalmoplégie supranucléaire</s0>
<s5>01</s5>
</fC03>
<fC03 i1="01" i2="X" l="ENG"><s0>Supranuclear ophthalmoplegia</s0>
<s5>01</s5>
</fC03>
<fC03 i1="01" i2="X" l="SPA"><s0>Oftalmoplejía supranuclear</s0>
<s5>01</s5>
</fC03>
<fC03 i1="02" i2="X" l="FRE"><s0>Progressif</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="ENG"><s0>Progressive</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="SPA"><s0>Progresivo</s0>
<s5>02</s5>
</fC03>
<fC03 i1="03" i2="X" l="FRE"><s0>Lévodopa</s0>
<s2>NK</s2>
<s2>FR</s2>
<s5>04</s5>
</fC03>
<fC03 i1="03" i2="X" l="ENG"><s0>Levodopa</s0>
<s2>NK</s2>
<s2>FR</s2>
<s5>04</s5>
</fC03>
<fC03 i1="03" i2="X" l="SPA"><s0>Levodopa</s0>
<s2>NK</s2>
<s2>FR</s2>
<s5>04</s5>
</fC03>
<fC03 i1="04" i2="X" l="FRE"><s0>Autopsie</s0>
<s5>16</s5>
</fC03>
<fC03 i1="04" i2="X" l="ENG"><s0>Autopsy</s0>
<s5>16</s5>
</fC03>
<fC03 i1="04" i2="X" l="SPA"><s0>Autopsia</s0>
<s5>16</s5>
</fC03>
<fC03 i1="05" i2="X" l="FRE"><s0>Diagnostic</s0>
<s5>17</s5>
</fC03>
<fC03 i1="05" i2="X" l="ENG"><s0>Diagnosis</s0>
<s5>17</s5>
</fC03>
<fC03 i1="05" i2="X" l="SPA"><s0>Diagnóstico</s0>
<s5>17</s5>
</fC03>
<fC03 i1="06" i2="X" l="FRE"><s0>Symptomatologie</s0>
<s5>18</s5>
</fC03>
<fC03 i1="06" i2="X" l="ENG"><s0>Symptomatology</s0>
<s5>18</s5>
</fC03>
<fC03 i1="06" i2="X" l="SPA"><s0>Sintomatología</s0>
<s5>18</s5>
</fC03>
<fC03 i1="07" i2="X" l="FRE"><s0>Homme</s0>
<s5>20</s5>
</fC03>
<fC03 i1="07" i2="X" l="ENG"><s0>Human</s0>
<s5>20</s5>
</fC03>
<fC03 i1="07" i2="X" l="SPA"><s0>Hombre</s0>
<s5>20</s5>
</fC03>
<fC07 i1="01" i2="X" l="FRE"><s0>Oeil pathologie</s0>
<s5>37</s5>
</fC07>
<fC07 i1="01" i2="X" l="ENG"><s0>Eye disease</s0>
<s5>37</s5>
</fC07>
<fC07 i1="01" i2="X" l="SPA"><s0>Ojo patología</s0>
<s5>37</s5>
</fC07>
<fC07 i1="02" i2="X" l="FRE"><s0>Oculomotricité syndrome</s0>
<s5>38</s5>
</fC07>
<fC07 i1="02" i2="X" l="ENG"><s0>Oculomotor syndrome</s0>
<s5>38</s5>
</fC07>
<fC07 i1="02" i2="X" l="SPA"><s0>Oculomotricidad síndrome</s0>
<s5>38</s5>
</fC07>
<fC07 i1="03" i2="X" l="FRE"><s0>Système nerveux pathologie</s0>
<s5>39</s5>
</fC07>
<fC07 i1="03" i2="X" l="ENG"><s0>Nervous system diseases</s0>
<s5>39</s5>
</fC07>
<fC07 i1="03" i2="X" l="SPA"><s0>Sistema nervioso patología</s0>
<s5>39</s5>
</fC07>
<fC07 i1="04" i2="X" l="FRE"><s0>Système nerveux central pathologie</s0>
<s5>40</s5>
</fC07>
<fC07 i1="04" i2="X" l="ENG"><s0>Central nervous system disease</s0>
<s5>40</s5>
</fC07>
<fC07 i1="04" i2="X" l="SPA"><s0>Sistema nervosio central patología</s0>
<s5>40</s5>
</fC07>
<fC07 i1="05" i2="X" l="FRE"><s0>Tronc cérébral syndrome</s0>
<s5>41</s5>
</fC07>
<fC07 i1="05" i2="X" l="ENG"><s0>Brain stem syndrome</s0>
<s5>41</s5>
</fC07>
<fC07 i1="05" i2="X" l="SPA"><s0>Tallo encefalico sindrome</s0>
<s5>41</s5>
</fC07>
<fC07 i1="06" i2="X" l="FRE"><s0>Encéphale pathologie</s0>
<s5>42</s5>
</fC07>
<fC07 i1="06" i2="X" l="ENG"><s0>Cerebral disorder</s0>
<s5>42</s5>
</fC07>
<fC07 i1="06" i2="X" l="SPA"><s0>Encéfalo patología</s0>
<s5>42</s5>
</fC07>
<fC07 i1="07" i2="X" l="FRE"><s0>Maladie dégénérative</s0>
<s5>43</s5>
</fC07>
<fC07 i1="07" i2="X" l="ENG"><s0>Degenerative disease</s0>
<s5>43</s5>
</fC07>
<fC07 i1="07" i2="X" l="SPA"><s0>Enfermedad degenerativa</s0>
<s5>43</s5>
</fC07>
<fN21><s1>020</s1>
</fN21>
<fN82><s1>PSI</s1>
</fN82>
</pA>
</standard>
</inist>
</record>

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	Movement Disorders (revue)
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Movement Disorders (revue)

Progressive supranuclear palsy diagnosis and confounding features: Report on 16 autopsied cases

Progressive supranuclear palsy diagnosis and confounding features: Report on 16 autopsied cases

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