Progressive supranuclear palsy diagnosis and confounding features: report on 16 autopsied cases.
Identifieur interne : 000937 ( Ncbi/Checkpoint ); précédent : 000936; suivant : 000938Progressive supranuclear palsy diagnosis and confounding features: report on 16 autopsied cases.
Auteurs : Sam Birdi [Canada] ; Ali H. Rajput ; Mark Fenton ; Jeffery R. Donat ; Bohdan Rozdilsky ; Christopher Robinson ; Rob Macaulay ; David GeorgeSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 2002.
English descriptors
- KwdEn :
- Aged, Aged, 80 and over, Brain (pathology), Diagnosis, Differential, Dopamine Agonists (therapeutic use), Drug Therapy, Combination, Female, Humans, Levodopa (therapeutic use), Magnetic Resonance Imaging, Male, Middle Aged, Neurologic Examination (drug effects), Prognosis, Supranuclear Palsy, Progressive (diagnosis), Supranuclear Palsy, Progressive (drug therapy), Supranuclear Palsy, Progressive (pathology), Treatment Outcome.
- MESH :
- chemical , therapeutic use : Dopamine Agonists, Levodopa.
- diagnosis : Supranuclear Palsy, Progressive.
- drug effects : Neurologic Examination.
- drug therapy : Supranuclear Palsy, Progressive.
- pathology : Brain, Supranuclear Palsy, Progressive.
- Aged, Aged, 80 and over, Diagnosis, Differential, Drug Therapy, Combination, Female, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Prognosis, Treatment Outcome.
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 1 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.
DOI: 10.1002/mds.10211
PubMed: 12465065
Affiliations:
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Progressive supranuclear palsy diagnosis and confounding features: report on 16 autopsied cases.</title><author><name sortKey="Birdi, Sam" sort="Birdi, Sam" uniqKey="Birdi S" first="Sam" last="Birdi">Sam Birdi</name><affiliation wicri:level="1"><nlm:affiliation>Division of Neurology, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.</nlm:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>Division of Neurology, University of Saskatchewan, Saskatoon, Saskatchewan</wicri:regionArea><wicri:noRegion>Saskatchewan</wicri:noRegion></affiliation></author><author><name sortKey="Rajput, Ali H" sort="Rajput, Ali H" uniqKey="Rajput A" first="Ali H" last="Rajput">Ali H. Rajput</name></author><author><name sortKey="Fenton, Mark" sort="Fenton, Mark" uniqKey="Fenton M" first="Mark" last="Fenton">Mark Fenton</name></author><author><name sortKey="Donat, Jeffery R" sort="Donat, Jeffery R" uniqKey="Donat J" first="Jeffery R" last="Donat">Jeffery R. Donat</name></author><author><name sortKey="Rozdilsky, Bohdan" sort="Rozdilsky, Bohdan" uniqKey="Rozdilsky B" first="Bohdan" last="Rozdilsky">Bohdan Rozdilsky</name></author><author><name sortKey="Robinson, Christopher" sort="Robinson, Christopher" uniqKey="Robinson C" first="Christopher" last="Robinson">Christopher Robinson</name></author><author><name sortKey="Macaulay, Rob" sort="Macaulay, Rob" uniqKey="Macaulay R" first="Rob" last="Macaulay">Rob Macaulay</name></author><author><name sortKey="George, David" sort="George, David" uniqKey="George D" first="David" last="George">David George</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2002">2002</date><idno type="RBID">pubmed:12465065</idno><idno type="pmid">12465065</idno><idno type="doi">10.1002/mds.10211</idno><idno type="wicri:Area/PubMed/Corpus">003914</idno><idno type="wicri:Area/PubMed/Curation">003914</idno><idno type="wicri:Area/PubMed/Checkpoint">003964</idno><idno type="wicri:Area/Ncbi/Merge">000937</idno><idno type="wicri:Area/Ncbi/Curation">000937</idno><idno type="wicri:Area/Ncbi/Checkpoint">000937</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Progressive supranuclear palsy diagnosis and confounding features: report on 16 autopsied cases.</title><author><name sortKey="Birdi, Sam" sort="Birdi, Sam" uniqKey="Birdi S" first="Sam" last="Birdi">Sam Birdi</name><affiliation wicri:level="1"><nlm:affiliation>Division of Neurology, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.</nlm:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>Division of Neurology, University of Saskatchewan, Saskatoon, Saskatchewan</wicri:regionArea><wicri:noRegion>Saskatchewan</wicri:noRegion></affiliation></author><author><name sortKey="Rajput, Ali H" sort="Rajput, Ali H" uniqKey="Rajput A" first="Ali H" last="Rajput">Ali H. Rajput</name></author><author><name sortKey="Fenton, Mark" sort="Fenton, Mark" uniqKey="Fenton M" first="Mark" last="Fenton">Mark Fenton</name></author><author><name sortKey="Donat, Jeffery R" sort="Donat, Jeffery R" uniqKey="Donat J" first="Jeffery R" last="Donat">Jeffery R. Donat</name></author><author><name sortKey="Rozdilsky, Bohdan" sort="Rozdilsky, Bohdan" uniqKey="Rozdilsky B" first="Bohdan" last="Rozdilsky">Bohdan Rozdilsky</name></author><author><name sortKey="Robinson, Christopher" sort="Robinson, Christopher" uniqKey="Robinson C" first="Christopher" last="Robinson">Christopher Robinson</name></author><author><name sortKey="Macaulay, Rob" sort="Macaulay, Rob" uniqKey="Macaulay R" first="Rob" last="Macaulay">Rob Macaulay</name></author><author><name sortKey="George, David" sort="George, David" uniqKey="George D" first="David" last="George">David George</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="2002" type="published">2002</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Aged</term><term>Aged, 80 and over</term><term>Brain (pathology)</term><term>Diagnosis, Differential</term><term>Dopamine Agonists (therapeutic use)</term><term>Drug Therapy, Combination</term><term>Female</term><term>Humans</term><term>Levodopa (therapeutic use)</term><term>Magnetic Resonance Imaging</term><term>Male</term><term>Middle Aged</term><term>Neurologic Examination (drug effects)</term><term>Prognosis</term><term>Supranuclear Palsy, Progressive (diagnosis)</term><term>Supranuclear Palsy, Progressive (drug therapy)</term><term>Supranuclear Palsy, Progressive (pathology)</term><term>Treatment Outcome</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Dopamine Agonists</term><term>Levodopa</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Supranuclear Palsy, Progressive</term></keywords><keywords scheme="MESH" qualifier="drug effects" xml:lang="en"><term>Neurologic Examination</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Supranuclear Palsy, Progressive</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain</term><term>Supranuclear Palsy, Progressive</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Aged</term><term>Aged, 80 and over</term><term>Diagnosis, Differential</term><term>Drug Therapy, Combination</term><term>Female</term><term>Humans</term><term>Magnetic Resonance Imaging</term><term>Male</term><term>Middle Aged</term><term>Prognosis</term><term>Treatment Outcome</term></keywords></textClass></profileDesc></teiHeader><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 1 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></front></TEI><affiliations><list><country><li>Canada</li></country></list><tree><noCountry><name sortKey="Donat, Jeffery R" sort="Donat, Jeffery R" uniqKey="Donat J" first="Jeffery R" last="Donat">Jeffery R. Donat</name><name sortKey="Fenton, Mark" sort="Fenton, Mark" uniqKey="Fenton M" first="Mark" last="Fenton">Mark Fenton</name><name sortKey="George, David" sort="George, David" uniqKey="George D" first="David" last="George">David George</name><name sortKey="Macaulay, Rob" sort="Macaulay, Rob" uniqKey="Macaulay R" first="Rob" last="Macaulay">Rob Macaulay</name><name sortKey="Rajput, Ali H" sort="Rajput, Ali H" uniqKey="Rajput A" first="Ali H" last="Rajput">Ali H. Rajput</name><name sortKey="Robinson, Christopher" sort="Robinson, Christopher" uniqKey="Robinson C" first="Christopher" last="Robinson">Christopher Robinson</name><name sortKey="Rozdilsky, Bohdan" sort="Rozdilsky, Bohdan" uniqKey="Rozdilsky B" first="Bohdan" last="Rozdilsky">Bohdan Rozdilsky</name></noCountry><country name="Canada"><noRegion><name sortKey="Birdi, Sam" sort="Birdi, Sam" uniqKey="Birdi S" first="Sam" last="Birdi">Sam Birdi</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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