Frontotemporal dementia with ubiquitinated cytoplasmic and intranuclear inclusions
Identifieur interne : 003321 ( Main/Exploration ); précédent : 003320; suivant : 003322Frontotemporal dementia with ubiquitinated cytoplasmic and intranuclear inclusions
Auteurs : John Woulfe [Canada] ; Andrew Kertesz [Canada] ; David G. Munoz [Canada, Espagne]Source :
- Acta neuropathologica [ 0001-6322 ] ; 2001.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Homme.
English descriptors
- KwdEn :
Abstract
Dementia of motor neuron disease type (DMND) is a variety of frontotemporal dementia (FTD) which is pathologically defined by characteristic neuronal ubiquitinated, tau- and synuclein-negative intracytoplasmic inclusions. Many cases with this pathology, however, do not have motor neuron disease. In the present study, we document the presence of ubiquitinated neuronal intranuclear inclusions in a sub-population of cases of neuropathologically verified DMND. Immunohistochemical localization of ubiquitin was performed on sections of post-mortem brain from 12 patients with DMND as well as from cases with other neurodegenerative diseases including amyotrophic lateral sclerosis, Parkinson's disease, dementia with Lewy bodies, corticobasal degeneration, progressive supranuclear palsy, and multiple system atrophy. All of the cases of DMND showed ubiquitinated, tau-negative intracytoplasmic inclusions in dentate granule cells and cortical neurons. Of these 12 cases of DMND, 3 also showed neuronal ubiquitinated intranuclear inclusions. In 1 of these cases, CAG repeat expansions in the genes known to harbor these mutations were excluded. Cases with intranuclear inclusions displayed striatal atrophy and reduced brain weight relative to non-inclusion-bearing cases. In addition, patients with intranuclear inclusions tended to have a younger age of onset, a prolonged duration of disease, absence of motor neuron symptoms, and a family history of dementia. Intranuclear inclusions were not identified in the control cases with other neurodegenerative diseases. Ubiquitinated neuronal intranuclear inclusions have not been reported previously in DMND. The presence of ubiquitinated intranuclear inclusions along with striatal atrophy in a subset of cases of DMND may signify the existence of a neuropathologically distinct subset of this unique form of FTD.
Affiliations:
Links toward previous steps (curation, corpus...)
- to stream PascalFrancis, to step Corpus: 000C15
- to stream PascalFrancis, to step Curation: 000108
- to stream PascalFrancis, to step Checkpoint: 000B02
- to stream Main, to step Merge: 003809
- to stream Main, to step Curation: 003321
Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en" level="a">Frontotemporal dementia with ubiquitinated cytoplasmic and intranuclear inclusions</title><author><name sortKey="Woulfe, John" sort="Woulfe, John" uniqKey="Woulfe J" first="John" last="Woulfe">John Woulfe</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Pathology, Ottawa Hospital, Civic Campus, 1053 Carling Avenue</s1><s2>Ottawa, Ontario, K1Y 4E9</s2><s3>CAN</s3><sZ>1 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>Ottawa, Ontario, K1Y 4E9</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Pathology and Molecular Medicine, McMaster University and The Hamilton Regional Laboratory Medicine Program, Hamilton Health Sciences Corporation</s1><s2>Hamilton, Ontario</s2><s3>CAN</s3><sZ>1 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>Hamilton, Ontario</wicri:noRegion></affiliation></author><author><name sortKey="Kertesz, Andrew" sort="Kertesz, Andrew" uniqKey="Kertesz A" first="Andrew" last="Kertesz">Andrew Kertesz</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>St. Joseph's Health Care, Department of Neurology, 268 Grosvenor St.</s1><s2>London, Ontario</s2><s3>CAN</s3><sZ>2 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>London, Ontario</wicri:noRegion></affiliation></author><author><name sortKey="Munoz, David G" sort="Munoz, David G" uniqKey="Munoz D" first="David G." last="Munoz">David G. Munoz</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Departments of Pathology and Clinical Neurological Sciences, University of Western Ontario</s1><s2>London, Ontario</s2><s3>CAN</s3><sZ>3 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>London, Ontario</wicri:noRegion></affiliation><affiliation wicri:level="3"><inist:fA14 i1="05"><s1>Servicio de Neurologia, "Doce de Octubre" Hospital</s1><s2>Madrid</s2><s3>ESP</s3><sZ>3 aut.</sZ></inist:fA14><country>Espagne</country><placeName><settlement type="city">Madrid</settlement><region nuts="2" type="region">Communauté de Madrid</region></placeName></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">INIST</idno><idno type="inist">01-0408687</idno><date when="2001">2001</date><idno type="stanalyst">PASCAL 01-0408687 INIST</idno><idno type="RBID">Pascal:01-0408687</idno><idno type="wicri:Area/PascalFrancis/Corpus">000C15</idno><idno type="wicri:Area/PascalFrancis/Curation">000108</idno><idno type="wicri:Area/PascalFrancis/Checkpoint">000B02</idno><idno type="wicri:explorRef" wicri:stream="PascalFrancis" wicri:step="Checkpoint">000B02</idno><idno type="wicri:doubleKey">0001-6322:2001:Woulfe J:frontotemporal:dementia:with</idno><idno type="wicri:Area/Main/Merge">003809</idno><idno type="wicri:Area/Main/Curation">003321</idno><idno type="wicri:Area/Main/Exploration">003321</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Frontotemporal dementia with ubiquitinated cytoplasmic and intranuclear inclusions</title><author><name sortKey="Woulfe, John" sort="Woulfe, John" uniqKey="Woulfe J" first="John" last="Woulfe">John Woulfe</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Pathology, Ottawa Hospital, Civic Campus, 1053 Carling Avenue</s1><s2>Ottawa, Ontario, K1Y 4E9</s2><s3>CAN</s3><sZ>1 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>Ottawa, Ontario, K1Y 4E9</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Pathology and Molecular Medicine, McMaster University and The Hamilton Regional Laboratory Medicine Program, Hamilton Health Sciences Corporation</s1><s2>Hamilton, Ontario</s2><s3>CAN</s3><sZ>1 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>Hamilton, Ontario</wicri:noRegion></affiliation></author><author><name sortKey="Kertesz, Andrew" sort="Kertesz, Andrew" uniqKey="Kertesz A" first="Andrew" last="Kertesz">Andrew Kertesz</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>St. Joseph's Health Care, Department of Neurology, 268 Grosvenor St.</s1><s2>London, Ontario</s2><s3>CAN</s3><sZ>2 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>London, Ontario</wicri:noRegion></affiliation></author><author><name sortKey="Munoz, David G" sort="Munoz, David G" uniqKey="Munoz D" first="David G." last="Munoz">David G. Munoz</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Departments of Pathology and Clinical Neurological Sciences, University of Western Ontario</s1><s2>London, Ontario</s2><s3>CAN</s3><sZ>3 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>London, Ontario</wicri:noRegion></affiliation><affiliation wicri:level="3"><inist:fA14 i1="05"><s1>Servicio de Neurologia, "Doce de Octubre" Hospital</s1><s2>Madrid</s2><s3>ESP</s3><sZ>3 aut.</sZ></inist:fA14><country>Espagne</country><placeName><settlement type="city">Madrid</settlement><region nuts="2" type="region">Communauté de Madrid</region></placeName></affiliation></author></analytic><series><title level="j" type="main">Acta neuropathologica</title><title level="j" type="abbreviated">Acta neuropathol.</title><idno type="ISSN">0001-6322</idno><imprint><date when="2001">2001</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Acta neuropathologica</title><title level="j" type="abbreviated">Acta neuropathol.</title><idno type="ISSN">0001-6322</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Case study</term><term>Cell nucleus</term><term>Dementia</term><term>Electron microscopy</term><term>Frontal lobe</term><term>Human</term><term>Immunohistochemistry</term><term>Inclusion</term><term>Pathophysiology</term><term>Temporal lobe</term><term>Ubiquitin</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Démence</term><term>Lobe frontal</term><term>Lobe temporal</term><term>Immunohistochimie</term><term>Microscopie électronique</term><term>Ubiquitine</term><term>Inclusion</term><term>Noyau cellulaire</term><term>Etude cas</term><term>Physiopathologie</term><term>Homme</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Homme</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Dementia of motor neuron disease type (DMND) is a variety of frontotemporal dementia (FTD) which is pathologically defined by characteristic neuronal ubiquitinated, tau- and synuclein-negative intracytoplasmic inclusions. Many cases with this pathology, however, do not have motor neuron disease. In the present study, we document the presence of ubiquitinated neuronal intranuclear inclusions in a sub-population of cases of neuropathologically verified DMND. Immunohistochemical localization of ubiquitin was performed on sections of post-mortem brain from 12 patients with DMND as well as from cases with other neurodegenerative diseases including amyotrophic lateral sclerosis, Parkinson's disease, dementia with Lewy bodies, corticobasal degeneration, progressive supranuclear palsy, and multiple system atrophy. All of the cases of DMND showed ubiquitinated, tau-negative intracytoplasmic inclusions in dentate granule cells and cortical neurons. Of these 12 cases of DMND, 3 also showed neuronal ubiquitinated intranuclear inclusions. In 1 of these cases, CAG repeat expansions in the genes known to harbor these mutations were excluded. Cases with intranuclear inclusions displayed striatal atrophy and reduced brain weight relative to non-inclusion-bearing cases. In addition, patients with intranuclear inclusions tended to have a younger age of onset, a prolonged duration of disease, absence of motor neuron symptoms, and a family history of dementia. Intranuclear inclusions were not identified in the control cases with other neurodegenerative diseases. Ubiquitinated neuronal intranuclear inclusions have not been reported previously in DMND. The presence of ubiquitinated intranuclear inclusions along with striatal atrophy in a subset of cases of DMND may signify the existence of a neuropathologically distinct subset of this unique form of FTD.</div></front></TEI><affiliations><list><country><li>Canada</li><li>Espagne</li></country><region><li>Communauté de Madrid</li></region><settlement><li>Madrid</li></settlement></list><tree><country name="Canada"><noRegion><name sortKey="Woulfe, John" sort="Woulfe, John" uniqKey="Woulfe J" first="John" last="Woulfe">John Woulfe</name></noRegion><name sortKey="Kertesz, Andrew" sort="Kertesz, Andrew" uniqKey="Kertesz A" first="Andrew" last="Kertesz">Andrew Kertesz</name><name sortKey="Munoz, David G" sort="Munoz, David G" uniqKey="Munoz D" first="David G." last="Munoz">David G. Munoz</name><name sortKey="Woulfe, John" sort="Woulfe, John" uniqKey="Woulfe J" first="John" last="Woulfe">John Woulfe</name></country><country name="Espagne"><region name="Communauté de Madrid"><name sortKey="Munoz, David G" sort="Munoz, David G" uniqKey="Munoz D" first="David G." last="Munoz">David G. Munoz</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Canada/explor/ParkinsonCanadaV1/Data/Main/Exploration
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 003321 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 003321 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien
|wiki= Wicri/Canada
|area= ParkinsonCanadaV1
|flux= Main
|étape= Exploration
|type= RBID
|clé= Pascal:01-0408687
|texte= Frontotemporal dementia with ubiquitinated cytoplasmic and intranuclear inclusions
}}
| This area was generated with Dilib version V0.6.29. |  |