Positron emission tomography in pallido-ponto-nigral degeneration (PPND) family (frontotemporal dementia with parkinsonism linked to chromosome 17 and point mutation in tau gene)
Identifieur interne : 003188 ( Main/Exploration ); précédent : 003187; suivant : 003189Positron emission tomography in pallido-ponto-nigral degeneration (PPND) family (frontotemporal dementia with parkinsonism linked to chromosome 17 and point mutation in tau gene)
Auteurs : P. K Pal [Canada] ; Z. K Wszolek [États-Unis] ; A. Kishore [Canada] ; R. De La Fuente-Fernandez [Canada] ; V. Sossi [Canada] ; R. J Uitti [États-Unis] ; T. Dobko [Canada] ; A. J Stoessl [Canada]Source :
- Parkinsonism and Related Disorders [ 1353-8020 ] ; 2001.
English descriptors
- KwdEn :
Abstract
Pallido-ponto-nigral degeneration (PPND) is a rapidly progressive disorder characterized by frontotemporal dementia with parkinsonism unresponsive to levodopa therapy. In this study, we have further characterized the regional abnormalities of cerebral function using PET with 6-[18F]fluoro-l-dopa (FD), [11C] raclopride (RAC), and 2-deoxy-2-fluoro-[18F]-d-glucose (FDG). FD and RAC scans were performed in 3 patients—2 new patients and a previously reported asymptomatic at-risk individual who became symptomatic 2years after the first FD scan. Cerebral glucose metabolism was studied by FDG in 2 other patients. In keeping with previous reports, there was a severe reduction of FD uptake, which affected both caudate and putamen to a similar degree in all 3 patients. RAC scans showed normal to elevated striatal D2-receptor binding in all patients. Cerebral glucose metabolism was globally reduced (>2 SD below control mean) in one patient, with maximal involvement of frontal regions, and to a lesser degree in the other patient. Our study showed severe presynaptic dopaminergic dysfunction with intact striatal D2 receptors in PPND patients, implying that the dopa unresponsiveness is probably a result of pathology downstream to the striatum. The pattern of presynaptic dysfunction contrasts with that seen in idiopathic parkinsonism, where the putamen is affected more than the caudate nucleus. The pattern of glucose hypometabolism correlates well with the presence of frontotemporal dementia.
Url:
- https://api-v5.istex.fr/document/3FB389364DA5C4001DB4DE9E05A9C9407DCA10D6/fulltext/pdf
- https://api-v5.istex.fr/document/B74520A47E7B4854295F1B35B50C93925C1BBA6B/fulltext/pdf
DOI: 10.1016/S1353-8020(00)00026-2
Affiliations:
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Le document en format XML
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K" last="Pal">P. K Pal</name><affiliation wicri:level="1"><country>Canada</country><wicri:regionArea>Neurodegenerative Disorders Centre, Vancouver Hospital and Health Sciences Centre, Vancouver, BC</wicri:regionArea><wicri:noRegion>BC</wicri:noRegion></affiliation></author><author><name sortKey="Wszolek, Z K" sort="Wszolek, Z K" uniqKey="Wszolek Z" first="Z. K" last="Wszolek">Z. K Wszolek</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Neurology, Mayo Clinic, Jacksonville, FL</wicri:regionArea><placeName><region type="state">Floride</region></placeName></affiliation></author><author><name sortKey="Kishore, A" sort="Kishore, A" uniqKey="Kishore A" first="A" last="Kishore">A. Kishore</name><affiliation wicri:level="1"><country>Canada</country><wicri:regionArea>Neurodegenerative Disorders Centre, Vancouver Hospital and Health Sciences Centre, Vancouver, BC</wicri:regionArea><wicri:noRegion>BC</wicri:noRegion></affiliation></author><author><name sortKey="De La Fuente Fernandez, R" sort="De La Fuente Fernandez, R" uniqKey="De La Fuente Fernandez R" first="R" last="De La Fuente-Fernandez">R. De La Fuente-Fernandez</name><affiliation wicri:level="1"><country>Canada</country><wicri:regionArea>Neurodegenerative Disorders Centre, Vancouver Hospital and Health Sciences Centre, Vancouver, BC</wicri:regionArea><wicri:noRegion>BC</wicri:noRegion></affiliation></author><author><name sortKey="Sossi, V" sort="Sossi, V" uniqKey="Sossi V" first="V" last="Sossi">V. Sossi</name><affiliation wicri:level="1"><country>Canada</country><wicri:regionArea>Neurodegenerative Disorders Centre, Vancouver Hospital and Health Sciences Centre, Vancouver, BC</wicri:regionArea><wicri:noRegion>BC</wicri:noRegion></affiliation></author><author><name sortKey="Uitti, R J" sort="Uitti, R J" uniqKey="Uitti R" first="R. J" last="Uitti">R. J Uitti</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Neurology, Mayo Clinic, Jacksonville, FL</wicri:regionArea><placeName><region type="state">Floride</region></placeName></affiliation></author><author><name sortKey="Dobko, T" sort="Dobko, T" uniqKey="Dobko T" first="T" last="Dobko">T. 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J Stoessl</name><affiliation wicri:level="1"><country wicri:rule="url">Canada</country></affiliation><affiliation wicri:level="1"><country>Canada</country><wicri:regionArea>Neurodegenerative Disorders Centre, Vancouver Hospital and Health Sciences Centre, Vancouver, BC</wicri:regionArea><wicri:noRegion>BC</wicri:noRegion></affiliation></author></analytic><monogr></monogr><series><title level="j">Parkinsonism and Related Disorders</title><title level="j" type="abbrev">PRD</title><idno type="ISSN">1353-8020</idno><imprint><publisher>ELSEVIER</publisher><date type="published" when="2001">2001</date><biblScope unit="volume">7</biblScope><biblScope unit="issue">2</biblScope><biblScope unit="page" from="81">81</biblScope><biblScope unit="page" to="88">88</biblScope></imprint><idno type="ISSN">1353-8020</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">1353-8020</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Dopamine D2 receptors</term><term>Familial</term><term>Fluorodeoxyglucose</term><term>Fluorodopa</term><term>Frontotemporal dementia</term><term>Genetic</term><term>PET</term><term>Pallido-ponto-nigral degeneration</term><term>Parkinsonism</term><term>Positron emission tomography</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Pallido-ponto-nigral degeneration (PPND) is a rapidly progressive disorder characterized by frontotemporal dementia with parkinsonism unresponsive to levodopa therapy. In this study, we have further characterized the regional abnormalities of cerebral function using PET with 6-[18F]fluoro-l-dopa (FD), [11C] raclopride (RAC), and 2-deoxy-2-fluoro-[18F]-d-glucose (FDG). FD and RAC scans were performed in 3 patients—2 new patients and a previously reported asymptomatic at-risk individual who became symptomatic 2years after the first FD scan. Cerebral glucose metabolism was studied by FDG in 2 other patients. In keeping with previous reports, there was a severe reduction of FD uptake, which affected both caudate and putamen to a similar degree in all 3 patients. RAC scans showed normal to elevated striatal D2-receptor binding in all patients. Cerebral glucose metabolism was globally reduced (>2 SD below control mean) in one patient, with maximal involvement of frontal regions, and to a lesser degree in the other patient. Our study showed severe presynaptic dopaminergic dysfunction with intact striatal D2 receptors in PPND patients, implying that the dopa unresponsiveness is probably a result of pathology downstream to the striatum. The pattern of presynaptic dysfunction contrasts with that seen in idiopathic parkinsonism, where the putamen is affected more than the caudate nucleus. The pattern of glucose hypometabolism correlates well with the presence of frontotemporal dementia.</div></front></TEI><affiliations><list><country><li>Canada</li><li>États-Unis</li></country><region><li>Floride</li></region></list><tree><country name="Canada"><noRegion><name sortKey="Pal, P K" sort="Pal, P K" uniqKey="Pal P" first="P. K" last="Pal">P. K Pal</name></noRegion><name sortKey="De La Fuente Fernandez, R" sort="De La Fuente Fernandez, R" uniqKey="De La Fuente Fernandez R" first="R" last="De La Fuente-Fernandez">R. De La Fuente-Fernandez</name><name sortKey="Dobko, T" sort="Dobko, T" uniqKey="Dobko T" first="T" last="Dobko">T. Dobko</name><name sortKey="Kishore, A" sort="Kishore, A" uniqKey="Kishore A" first="A" last="Kishore">A. Kishore</name><name sortKey="Sossi, V" sort="Sossi, V" uniqKey="Sossi V" first="V" last="Sossi">V. Sossi</name><name sortKey="Stoessl, A J" sort="Stoessl, A J" uniqKey="Stoessl A" first="A. J" last="Stoessl">A. J Stoessl</name><name sortKey="Stoessl, A J" sort="Stoessl, A J" uniqKey="Stoessl A" first="A. J" last="Stoessl">A. J Stoessl</name></country><country name="États-Unis"><region name="Floride"><name sortKey="Wszolek, Z K" sort="Wszolek, Z K" uniqKey="Wszolek Z" first="Z. K" last="Wszolek">Z. K Wszolek</name></region><name sortKey="Uitti, R J" sort="Uitti, R J" uniqKey="Uitti R" first="R. J" last="Uitti">R. J Uitti</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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