Syndromes of amyotrophic lateral sclerosis and dementia: Relation to transmissible Creutzfeldt‐Jakob disease
Identifieur interne : 002E40 ( Main/Corpus ); précédent : 002E39; suivant : 002E41Syndromes of amyotrophic lateral sclerosis and dementia: Relation to transmissible Creutzfeldt‐Jakob disease
Auteurs : Andres M. Salazar ; Colin L. Masters ; D. Carleton Gajdusek ; Clarence J. Gibbs JrSource :
- Annals of Neurology [ 0364-5134 ] ; 1983-07.
Abstract
A review of over 2,000 cases of Creutzfeldt‐Jakob disease and related disorders in the literature and our own files yielded 231 cases of dementia with early lower motor neuron signs. The clinical‐pathological profiles of the 231 cases were distinctly different from those of cases of transmissible Creutzfeldt‐Jakob disease: the patients had a longer illness, and their brains lacked the typical spongiform change. Brain tissue from 33 of these patients has been inoculated intracerebrally into nonhuman primates, but only 2 atypical cases transmitted a spongiform encephalopathy; 23 have been incubating from three to twelve years and can be considered negative transmission experiments. The findings suggest that most cases of dementia associated with early amyotrophy are more closely related to classic amyotrophic lateral sclerosis than to transmissible Creutzfeldt‐Jakob disease and do not deserve the label of “amyotrophic Creutzfeldt‐Jakob disease.” When lower motor neuron involvement occurs in transmissible Creutzfeldt‐Jakob disease, it is usually late and accompanied by signs of a more fulminant cerebral and cerebellar involvement.
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DOI: 10.1002/ana.410140104
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Carleton Gajdusek</name><affiliation><mods:affiliation>Laboratory of Central Nervous System Studies, National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda, MD 20205</mods:affiliation></affiliation></author><author><name sortKey="Gibbs Jr, Clarence J" sort="Gibbs Jr, Clarence J" uniqKey="Gibbs Jr C" first="Clarence J." last="Gibbs Jr">Clarence J. 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The clinical‐pathological profiles of the 231 cases were distinctly different from those of cases of transmissible Creutzfeldt‐Jakob disease: the patients had a longer illness, and their brains lacked the typical spongiform change. Brain tissue from 33 of these patients has been inoculated intracerebrally into nonhuman primates, but only 2 atypical cases transmitted a spongiform encephalopathy; 23 have been incubating from three to twelve years and can be considered negative transmission experiments. The findings suggest that most cases of dementia associated with early amyotrophy are more closely related to classic amyotrophic lateral sclerosis than to transmissible Creutzfeldt‐Jakob disease and do not deserve the label of “amyotrophic Creutzfeldt‐Jakob disease.” When lower motor neuron involvement occurs in transmissible Creutzfeldt‐Jakob disease, it is usually late and accompanied by signs of a more fulminant cerebral and cerebellar involvement.</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Andres M. Salazar MD</name><affiliations><json:string>Laboratory of Central Nervous System Studies, National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda, MD 20205</json:string><json:string>Current Address: Vietnam Head Injury Study, Walter Reed Army Medical Center, Washington, DC 20307</json:string></affiliations></json:item><json:item><name>Colin L. Masters MD</name><affiliations><json:string>Department of Pathology, University of Western Australia, Perth, Australia, perth, Australia</json:string></affiliations></json:item><json:item><name>D. Carleton Gajdusek MD</name><affiliations><json:string>Laboratory of Central Nervous System Studies, National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda, MD 20205</json:string></affiliations></json:item><json:item><name>Clarence J. Gibbs Jr Phd</name><affiliations><json:string>Laboratory of Central Nervous System Studies, National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda, MD 20205</json:string></affiliations></json:item></author><articleId><json:string>ANA410140104</json:string></articleId><language><json:string>eng</json:string></language><abstract>A review of over 2,000 cases of Creutzfeldt‐Jakob disease and related disorders in the literature and our own files yielded 231 cases of dementia with early lower motor neuron signs. The clinical‐pathological profiles of the 231 cases were distinctly different from those of cases of transmissible Creutzfeldt‐Jakob disease: the patients had a longer illness, and their brains lacked the typical spongiform change. Brain tissue from 33 of these patients has been inoculated intracerebrally into nonhuman primates, but only 2 atypical cases transmitted a spongiform encephalopathy; 23 have been incubating from three to twelve years and can be considered negative transmission experiments. The findings suggest that most cases of dementia associated with early amyotrophy are more closely related to classic amyotrophic lateral sclerosis than to transmissible Creutzfeldt‐Jakob disease and do not deserve the label of “amyotrophic Creutzfeldt‐Jakob disease.” When lower motor neuron involvement occurs in transmissible Creutzfeldt‐Jakob disease, it is usually late and accompanied by signs of a more fulminant cerebral and cerebellar involvement.</abstract><qualityIndicators><score>6.956</score><pdfVersion>1.3</pdfVersion><pdfPageSize>612 x 828 pts</pdfPageSize><refBibsNative>true</refBibsNative><keywordCount>0</keywordCount><abstractCharCount>1145</abstractCharCount><pdfWordCount>6515</pdfWordCount><pdfCharCount>42735</pdfCharCount><pdfPageCount>10</pdfPageCount><abstractWordCount>163</abstractWordCount></qualityIndicators><title>Syndromes of amyotrophic lateral sclerosis and dementia: Relation to transmissible Creutzfeldt‐Jakob disease</title><genre><json:string>article</json:string></genre><host><volume>14</volume><publisherId><json:string>ANA</json:string></publisherId><pages><total>10</total><last>26</last><first>17</first></pages><issn><json:string>0364-5134</json:string></issn><issue>1</issue><subject><json:item><value>Special Article</value></json:item></subject><genre><json:string>Journal</json:string></genre><language><json:string>unknown</json:string></language><eissn><json:string>1531-8249</json:string></eissn><title>Annals of Neurology</title><doi><json:string>10.1002/(ISSN)1531-8249</json:string></doi></host><publicationDate>1983</publicationDate><copyrightDate>1983</copyrightDate><doi><json:string>10.1002/ana.410140104</json:string></doi><id>9EEE09399FA279EAA65C9EA08BE7C35F7B01C284</id><fulltext><json:item><original>true</original><mimetype>application/pdf</mimetype><extension>pdf</extension><uri>https://api.istex.fr/document/9EEE09399FA279EAA65C9EA08BE7C35F7B01C284/fulltext/pdf</uri></json:item><json:item><original>false</original><mimetype>application/zip</mimetype><extension>zip</extension><uri>https://api.istex.fr/document/9EEE09399FA279EAA65C9EA08BE7C35F7B01C284/fulltext/zip</uri></json:item><istex:fulltextTEI uri="https://api.istex.fr/document/9EEE09399FA279EAA65C9EA08BE7C35F7B01C284/fulltext/tei"><teiHeader><fileDesc><titleStmt><title level="a" type="main" xml:lang="en">Syndromes of amyotrophic lateral sclerosis and dementia: Relation to transmissible Creutzfeldt‐Jakob disease</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher>Little, Brown and Company</publisher><pubPlace>Boston</pubPlace><availability><p>WILEY</p></availability><date>1983</date></publicationStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Syndromes of amyotrophic lateral sclerosis and dementia: Relation to transmissible Creutzfeldt‐Jakob disease</title><author><persName><forename type="first">Andres M.</forename><surname>Salazar</surname></persName><roleName type="degree">MD</roleName><affiliation>Laboratory of Central Nervous System Studies, National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda, MD 20205</affiliation><affiliation>Current Address: Vietnam Head Injury Study, Walter Reed Army Medical Center, Washington, DC 20307</affiliation></author><author><persName><forename type="first">Colin L.</forename><surname>Masters</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Pathology, University of Western Australia, Perth, Australia, perth, Australia</affiliation></author><author><persName><forename type="first">D. 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The clinical‐pathological profiles of the 231 cases were distinctly different from those of cases of transmissible Creutzfeldt‐Jakob disease: the patients had a longer illness, and their brains lacked the typical spongiform change. Brain tissue from 33 of these patients has been inoculated intracerebrally into nonhuman primates, but only 2 atypical cases transmitted a spongiform encephalopathy; 23 have been incubating from three to twelve years and can be considered negative transmission experiments. The findings suggest that most cases of dementia associated with early amyotrophy are more closely related to classic amyotrophic lateral sclerosis than to transmissible Creutzfeldt‐Jakob disease and do not deserve the label of “amyotrophic Creutzfeldt‐Jakob disease.” When lower motor neuron involvement occurs in transmissible Creutzfeldt‐Jakob disease, it is usually late and accompanied by signs of a more fulminant cerebral and cerebellar involvement.</p></abstract><textClass><keywords scheme="Journal Subject"><list><head>article category</head><item><term>Special Article</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="1982-09-09">Received</change><change when="1982-12-17">Registration</change><change when="1983-07">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/9EEE09399FA279EAA65C9EA08BE7C35F7B01C284/fulltext/txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Wiley, elements deleted: body"><istex:xmlDeclaration>version="1.0" encoding="UTF-8" standalone="yes"</istex:xmlDeclaration><istex:document><component version="2.0" type="serialArticle" xml:lang="en"><header><publicationMeta level="product"><publisherInfo><publisherName>Little, Brown and Company</publisherName><publisherLoc>Boston</publisherLoc></publisherInfo><doi registered="yes">10.1002/(ISSN)1531-8249</doi><issn type="print">0364-5134</issn><issn type="electronic">1531-8249</issn><idGroup><id type="product" value="ANA"></id></idGroup><titleGroup><title type="main" xml:lang="en" sort="ANNALS OF NEUROLOGY">Annals of Neurology</title><title type="subtitle">Official Journal of the American Neurological Association and the Child Neurology Society</title><title type="short">Ann Neurol.</title></titleGroup></publicationMeta><publicationMeta level="part" position="10"><doi origin="wiley" registered="yes">10.1002/ana.v14:1</doi><numberingGroup><numbering type="journalVolume" number="14">14</numbering><numbering type="journalIssue">1</numbering></numberingGroup><coverDate startDate="1983-07">July 1983</coverDate></publicationMeta><publicationMeta level="unit" type="article" position="4" status="forIssue"><doi origin="wiley" registered="yes">10.1002/ana.410140104</doi><idGroup><id type="unit" value="ANA410140104"></id></idGroup><countGroup><count type="pageTotal" number="10"></count></countGroup><titleGroup><title type="articleCategory">Special Article</title><title type="tocHeading1">Special Articles</title></titleGroup><copyright ownership="thirdParty">Copyright © 1983 American Neurological Association</copyright><eventGroup><event type="manuscriptReceived" date="1982-09-09"></event><event type="manuscriptRevised" date="1982-12-13"></event><event type="manuscriptAccepted" date="1982-12-17"></event><event type="firstOnline" date="2004-10-07"></event><event type="publishedOnlineFinalForm" date="2004-10-07"></event><event type="xmlConverted" agent="Converter:JWSART34_TO_WML3G version:2.3.1 mode:FullText source:HeaderRef result:HeaderRef" date="2010-02-23"></event><event type="xmlConverted" agent="Converter:WILEY_ML3G_TO_WILEY_ML3GV2 version:3.8.8" date="2014-01-03"></event><event type="xmlConverted" agent="Converter:WML3G_To_WML3G version:4.1.7 mode:FullText,remove_FC" date="2014-10-14"></event></eventGroup><numberingGroup><numbering type="pageFirst">17</numbering><numbering type="pageLast">26</numbering></numberingGroup><linkGroup><link type="toTypesetVersion" href="file:ANA.ANA410140104.pdf"></link></linkGroup></publicationMeta><contentMeta><countGroup><count type="figureTotal" number="0"></count><count type="tableTotal" number="4"></count><count type="referenceTotal" number="117"></count></countGroup><titleGroup><title type="main" xml:lang="en">Syndromes of amyotrophic lateral sclerosis and dementia: Relation to transmissible Creutzfeldt‐Jakob disease</title><title type="short" xml:lang="en">Syndromes of ALS and Dementia</title></titleGroup><creators><creator xml:id="au1" creatorRole="author" affiliationRef="#af1" currentRef="#curr1"><personName><givenNames>Andres M.</givenNames><familyName>Salazar</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au2" creatorRole="author" affiliationRef="#af2"><personName><givenNames>Colin L.</givenNames><familyName>Masters</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au3" creatorRole="author" affiliationRef="#af1"><personName><givenNames>D. 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The findings suggest that most cases of dementia associated with early amyotrophy are more closely related to classic amyotrophic lateral sclerosis than to transmissible Creutzfeldt‐Jakob disease and do not deserve the label of “amyotrophic Creutzfeldt‐Jakob disease.” When lower motor neuron involvement occurs in transmissible Creutzfeldt‐Jakob disease, it is usually late and accompanied by signs of a more fulminant cerebral and cerebellar involvement.</p></abstract></abstractGroup></contentMeta></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Syndromes of amyotrophic lateral sclerosis and dementia: Relation to transmissible Creutzfeldt‐Jakob disease</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>Syndromes of ALS and Dementia</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Syndromes of amyotrophic lateral sclerosis and dementia: Relation to transmissible Creutzfeldt‐Jakob disease</title></titleInfo><name type="personal"><namePart type="given">Andres M.</namePart><namePart type="family">Salazar</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Laboratory of Central Nervous System Studies, National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda, MD 20205</affiliation><affiliation>Current Address: Vietnam Head Injury Study, Walter Reed Army Medical Center, Washington, DC 20307</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Colin L.</namePart><namePart type="family">Masters</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Pathology, University of Western Australia, Perth, Australia, perth, Australia</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">D. 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The clinical‐pathological profiles of the 231 cases were distinctly different from those of cases of transmissible Creutzfeldt‐Jakob disease: the patients had a longer illness, and their brains lacked the typical spongiform change. Brain tissue from 33 of these patients has been inoculated intracerebrally into nonhuman primates, but only 2 atypical cases transmitted a spongiform encephalopathy; 23 have been incubating from three to twelve years and can be considered negative transmission experiments. The findings suggest that most cases of dementia associated with early amyotrophy are more closely related to classic amyotrophic lateral sclerosis than to transmissible Creutzfeldt‐Jakob disease and do not deserve the label of “amyotrophic Creutzfeldt‐Jakob disease.” When lower motor neuron involvement occurs in transmissible Creutzfeldt‐Jakob disease, it is usually late and accompanied by signs of a more fulminant cerebral and cerebellar involvement.</abstract><relatedItem type="host"><titleInfo><title>Annals of Neurology</title><subTitle>Official Journal of the American Neurological Association and the Child Neurology Society</subTitle></titleInfo><titleInfo type="abbreviated"><title>Ann Neurol.</title></titleInfo><genre type="Journal">journal</genre><subject><genre>article category</genre><topic>Special Article</topic></subject><identifier type="ISSN">0364-5134</identifier><identifier type="eISSN">1531-8249</identifier><identifier type="DOI">10.1002/(ISSN)1531-8249</identifier><identifier type="PublisherID">ANA</identifier><part><date>1983</date><detail type="volume"><caption>vol.</caption><number>14</number></detail><detail type="issue"><caption>no.</caption><number>1</number></detail><extent unit="pages"><start>17</start><end>26</end><total>10</total></extent></part></relatedItem><identifier type="istex">9EEE09399FA279EAA65C9EA08BE7C35F7B01C284</identifier><identifier type="DOI">10.1002/ana.410140104</identifier><identifier type="ArticleID">ANA410140104</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 1983 American Neurological Association</accessCondition><recordInfo><recordContentSource>WILEY</recordContentSource><recordOrigin>Little, Brown and Company</recordOrigin></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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