The nigrostriatal pathway in Creutzfeldt-Jakob disease.
Identifieur interne : 000B78 ( PubMed/Corpus ); précédent : 000B77; suivant : 000B79The nigrostriatal pathway in Creutzfeldt-Jakob disease.
Auteurs : Anne Vital ; Pierre-Olivier Fernagut ; Marie-Hélène Canron ; Julien Joux ; Erwan Bezard ; Marie-Laure Martin-Negrier ; Claude Vital ; François TisonSource :
- Journal of neuropathology and experimental neurology [ 0022-3069 ] ; 2009.
English descriptors
- KwdEn :
- 14-3-3 Proteins (metabolism), Adult, Aged, Aged, 80 and over, Chorea (complications), Chorea (metabolism), Chorea (pathology), Corpus Striatum (metabolism), Corpus Striatum (pathology), Creutzfeldt-Jakob Syndrome (complications), Creutzfeldt-Jakob Syndrome (metabolism), Creutzfeldt-Jakob Syndrome (pathology), Female, Humans, Male, Middle Aged, Myoclonus (complications), Myoclonus (metabolism), Myoclonus (pathology), Neural Pathways (metabolism), Neural Pathways (pathology), Neurons (cytology), Neurons (metabolism), Neurons (pathology), Parkinsonian Disorders (complications), Parkinsonian Disorders (metabolism), Parkinsonian Disorders (pathology), Prions (metabolism), Substantia Nigra (metabolism), Substantia Nigra (pathology), Ubiquitin (metabolism), Young Adult, alpha-Synuclein (metabolism).
- MESH :
- chemical , metabolism : 14-3-3 Proteins, Prions, Ubiquitin, alpha-Synuclein.
- complications : Chorea, Creutzfeldt-Jakob Syndrome, Myoclonus, Parkinsonian Disorders.
- cytology : Neurons.
- metabolism : Chorea, Corpus Striatum, Creutzfeldt-Jakob Syndrome, Myoclonus, Neural Pathways, Neurons, Parkinsonian Disorders, Substantia Nigra.
- pathology : Chorea, Corpus Striatum, Creutzfeldt-Jakob Syndrome, Myoclonus, Neural Pathways, Neurons, Parkinsonian Disorders, Substantia Nigra.
- Adult, Aged, Aged, 80 and over, Female, Humans, Male, Middle Aged, Young Adult.
Abstract
Parkinsonism, chorea, and dystonia are well-known clinical manifestations of Creutzfeldt-Jakob disease (CJD), but lesions of the nigrostriatal pathway have never been thoroughly studied. We performed a detailed neuropathologic study of the nigrostriatal pathway in 15 sporadic CJD and 2 variant CJD cases that included clinical correlations and assessment of neuron subtype loss, distribution of prion protein, alpha-synuclein, ubiquitin, and 14-3-3 aggregation. We found evidence of nigrostriatal pathway damage in these CJD cases. Dopaminergic neurons and striatal outflow neurons were markedly affected in sporadic CJD, whereas cholinergic interneurons were spared. In cases of CJD with chorea or myoclonus, there was less presynaptic dopaminergic loss than in cases of CJD with parkinsonism. The 2 variant CJD cases with parkinsonism or chorea showed severe cholinergic interneuron loss in the caudate and putamen, a pattern that differed from that found in sporadic CJD. alpha-Synuclein, ubiquitin, and 14-3-3 aggregation coexisted with prion protein aggregation, thereby generating mixed pathological features. These findings suggest a possible pathophysiological overlap of abnormal protein aggregation in CJD and Parkinson disease.
DOI: 10.1097/NEN.0b013e3181abdae8
PubMed: 19535991
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pubmed:19535991Le document en format XML
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sortKey="Bezard, Erwan" sort="Bezard, Erwan" uniqKey="Bezard E" first="Erwan" last="Bezard">Erwan Bezard</name></author><author><name sortKey="Martin Negrier, Marie Laure" sort="Martin Negrier, Marie Laure" uniqKey="Martin Negrier M" first="Marie-Laure" last="Martin-Negrier">Marie-Laure Martin-Negrier</name></author><author><name sortKey="Vital, Claude" sort="Vital, Claude" uniqKey="Vital C" first="Claude" last="Vital">Claude Vital</name></author><author><name sortKey="Tison, Francois" sort="Tison, Francois" uniqKey="Tison F" first="François" last="Tison">François Tison</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2009">2009</date><idno type="RBID">pubmed:19535991</idno><idno type="pmid">19535991</idno><idno type="doi">10.1097/NEN.0b013e3181abdae8</idno><idno type="wicri:Area/PubMed/Corpus">000B78</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" 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last="Joux">Julien Joux</name></author><author><name sortKey="Bezard, Erwan" sort="Bezard, Erwan" uniqKey="Bezard E" first="Erwan" last="Bezard">Erwan Bezard</name></author><author><name sortKey="Martin Negrier, Marie Laure" sort="Martin Negrier, Marie Laure" uniqKey="Martin Negrier M" first="Marie-Laure" last="Martin-Negrier">Marie-Laure Martin-Negrier</name></author><author><name sortKey="Vital, Claude" sort="Vital, Claude" uniqKey="Vital C" first="Claude" last="Vital">Claude Vital</name></author><author><name sortKey="Tison, Francois" sort="Tison, Francois" uniqKey="Tison F" first="François" last="Tison">François Tison</name></author></analytic><series><title level="j">Journal of neuropathology and experimental neurology</title><idno type="ISSN">0022-3069</idno><imprint><date when="2009" type="published">2009</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>14-3-3 Proteins (metabolism)</term><term>Adult</term><term>Aged</term><term>Aged, 80 and over</term><term>Chorea (complications)</term><term>Chorea (metabolism)</term><term>Chorea (pathology)</term><term>Corpus Striatum (metabolism)</term><term>Corpus Striatum (pathology)</term><term>Creutzfeldt-Jakob Syndrome (complications)</term><term>Creutzfeldt-Jakob Syndrome (metabolism)</term><term>Creutzfeldt-Jakob Syndrome (pathology)</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Myoclonus (complications)</term><term>Myoclonus (metabolism)</term><term>Myoclonus (pathology)</term><term>Neural Pathways (metabolism)</term><term>Neural Pathways (pathology)</term><term>Neurons (cytology)</term><term>Neurons (metabolism)</term><term>Neurons (pathology)</term><term>Parkinsonian Disorders (complications)</term><term>Parkinsonian Disorders (metabolism)</term><term>Parkinsonian Disorders (pathology)</term><term>Prions (metabolism)</term><term>Substantia Nigra (metabolism)</term><term>Substantia Nigra (pathology)</term><term>Ubiquitin (metabolism)</term><term>Young Adult</term><term>alpha-Synuclein (metabolism)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>14-3-3 Proteins</term><term>Prions</term><term>Ubiquitin</term><term>alpha-Synuclein</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Chorea</term><term>Creutzfeldt-Jakob Syndrome</term><term>Myoclonus</term><term>Parkinsonian Disorders</term></keywords><keywords scheme="MESH" qualifier="cytology" xml:lang="en"><term>Neurons</term></keywords><keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Chorea</term><term>Corpus Striatum</term><term>Creutzfeldt-Jakob Syndrome</term><term>Myoclonus</term><term>Neural Pathways</term><term>Neurons</term><term>Parkinsonian Disorders</term><term>Substantia Nigra</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Chorea</term><term>Corpus Striatum</term><term>Creutzfeldt-Jakob Syndrome</term><term>Myoclonus</term><term>Neural Pathways</term><term>Neurons</term><term>Parkinsonian Disorders</term><term>Substantia Nigra</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Aged</term><term>Aged, 80 and over</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Young Adult</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Parkinsonism, chorea, and dystonia are well-known clinical manifestations of Creutzfeldt-Jakob disease (CJD), but lesions of the nigrostriatal pathway have never been thoroughly studied. We performed a detailed neuropathologic study of the nigrostriatal pathway in 15 sporadic CJD and 2 variant CJD cases that included clinical correlations and assessment of neuron subtype loss, distribution of prion protein, alpha-synuclein, ubiquitin, and 14-3-3 aggregation. We found evidence of nigrostriatal pathway damage in these CJD cases. Dopaminergic neurons and striatal outflow neurons were markedly affected in sporadic CJD, whereas cholinergic interneurons were spared. In cases of CJD with chorea or myoclonus, there was less presynaptic dopaminergic loss than in cases of CJD with parkinsonism. The 2 variant CJD cases with parkinsonism or chorea showed severe cholinergic interneuron loss in the caudate and putamen, a pattern that differed from that found in sporadic CJD. alpha-Synuclein, ubiquitin, and 14-3-3 aggregation coexisted with prion protein aggregation, thereby generating mixed pathological features. These findings suggest a possible pathophysiological overlap of abnormal protein aggregation in CJD and Parkinson disease.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">19535991</PMID><DateCreated><Year>2009</Year><Month>06</Month><Day>30</Day></DateCreated><DateCompleted><Year>2009</Year><Month>08</Month><Day>10</Day></DateCompleted><DateRevised><Year>2009</Year><Month>06</Month><Day>30</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0022-3069</ISSN><JournalIssue CitedMedium="Print"><Volume>68</Volume><Issue>7</Issue><PubDate><Year>2009</Year><Month>Jul</Month></PubDate></JournalIssue><Title>Journal of neuropathology and experimental neurology</Title><ISOAbbreviation>J. Neuropathol. Exp. Neurol.</ISOAbbreviation></Journal><ArticleTitle>The nigrostriatal pathway in Creutzfeldt-Jakob disease.</ArticleTitle><Pagination><MedlinePgn>809-15</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1097/NEN.0b013e3181abdae8</ELocationID><Abstract><AbstractText>Parkinsonism, chorea, and dystonia are well-known clinical manifestations of Creutzfeldt-Jakob disease (CJD), but lesions of the nigrostriatal pathway have never been thoroughly studied. We performed a detailed neuropathologic study of the nigrostriatal pathway in 15 sporadic CJD and 2 variant CJD cases that included clinical correlations and assessment of neuron subtype loss, distribution of prion protein, alpha-synuclein, ubiquitin, and 14-3-3 aggregation. We found evidence of nigrostriatal pathway damage in these CJD cases. Dopaminergic neurons and striatal outflow neurons were markedly affected in sporadic CJD, whereas cholinergic interneurons were spared. In cases of CJD with chorea or myoclonus, there was less presynaptic dopaminergic loss than in cases of CJD with parkinsonism. The 2 variant CJD cases with parkinsonism or chorea showed severe cholinergic interneuron loss in the caudate and putamen, a pattern that differed from that found in sporadic CJD. alpha-Synuclein, ubiquitin, and 14-3-3 aggregation coexisted with prion protein aggregation, thereby generating mixed pathological features. These findings suggest a possible pathophysiological overlap of abnormal protein aggregation in CJD and Parkinson disease.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Vital</LastName><ForeName>Anne</ForeName><Initials>A</Initials><AffiliationInfo><Affiliation>Université Victor Segalen-Bordeaux 2, Centre National de la Recherche Scientifique, Bordeaux Institute of Neuroscience, Bordeaux University Hospital, Bordeaux, France. anne.vital@chu-bordeaux.fr</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Fernagut</LastName><ForeName>Pierre-Olivier</ForeName><Initials>PO</Initials></Author><Author ValidYN="Y"><LastName>Canron</LastName><ForeName>Marie-Hélène</ForeName><Initials>MH</Initials></Author><Author ValidYN="Y"><LastName>Joux</LastName><ForeName>Julien</ForeName><Initials>J</Initials></Author><Author ValidYN="Y"><LastName>Bezard</LastName><ForeName>Erwan</ForeName><Initials>E</Initials></Author><Author ValidYN="Y"><LastName>Martin-Negrier</LastName><ForeName>Marie-Laure</ForeName><Initials>ML</Initials></Author><Author ValidYN="Y"><LastName>Vital</LastName><ForeName>Claude</ForeName><Initials>C</Initials></Author><Author ValidYN="Y"><LastName>Tison</LastName><ForeName>François</ForeName><Initials>F</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>England</Country><MedlineTA>J Neuropathol Exp Neurol</MedlineTA><NlmUniqueID>2985192R</NlmUniqueID><ISSNLinking>0022-3069</ISSNLinking></MedlineJournalInfo><ChemicalList><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D048948">14-3-3 Proteins</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D011328">Prions</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D025801">Ubiquitin</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D051844">alpha-Synuclein</NameOfSubstance></Chemical></ChemicalList><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D048948" MajorTopicYN="N">14-3-3 Proteins</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D000368" MajorTopicYN="N">Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D000369" MajorTopicYN="N">Aged, 80 and over</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D002819" MajorTopicYN="N">Chorea</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D003342" MajorTopicYN="N">Corpus Striatum</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName><QualifierName UI="Q000473" MajorTopicYN="Y">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D007562" MajorTopicYN="N">Creutzfeldt-Jakob Syndrome</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName><QualifierName UI="Q000473" MajorTopicYN="Y">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D005260" MajorTopicYN="N">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008875" MajorTopicYN="N">Middle Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D009207" MajorTopicYN="N">Myoclonus</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D009434" MajorTopicYN="N">Neural Pathways</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D009474" MajorTopicYN="N">Neurons</DescriptorName><QualifierName UI="Q000166" MajorTopicYN="N">cytology</QualifierName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D020734" MajorTopicYN="N">Parkinsonian Disorders</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D011328" MajorTopicYN="N">Prions</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D013378" MajorTopicYN="N">Substantia Nigra</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName><QualifierName UI="Q000473" MajorTopicYN="Y">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D025801" MajorTopicYN="N">Ubiquitin</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D055815" MajorTopicYN="N">Young Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D051844" MajorTopicYN="N">alpha-Synuclein</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="entrez"><Year>2009</Year><Month>6</Month><Day>19</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="pubmed"><Year>2009</Year><Month>6</Month><Day>19</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2009</Year><Month>8</Month><Day>11</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">19535991</ArticleId><ArticleId IdType="doi">10.1097/NEN.0b013e3181abdae8</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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