Adult-onset subacute sclerosing panencephalitis: case reports and review of the literature.
Identifieur interne : 004677 ( PubMed/Corpus ); précédent : 004676; suivant : 004678Adult-onset subacute sclerosing panencephalitis: case reports and review of the literature.
Auteurs : C. Singer ; A E Lang ; O. SuchowerskySource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 1997.
English descriptors
- KwdEn :
- Adolescent, Adult, Age of Onset, Anticonvulsants (therapeutic use), Brain (blood supply), Brain (pathology), Cognition Disorders, Electroencephalography, Epilepsies, Myoclonic (diagnosis), Epilepsies, Myoclonic (drug therapy), Female, Humans, Immunoglobulin G (immunology), Magnetic Resonance Imaging, Male, Measles virus (immunology), Measles virus (pathogenicity), Movement Disorders, Subacute Sclerosing Panencephalitis (etiology), Subacute Sclerosing Panencephalitis (pathology), Tomography, X-Ray Computed, Vision Disorders.
- MESH :
- chemical , immunology : Immunoglobulin G.
- chemical , therapeutic use : Anticonvulsants.
- blood supply : Brain.
- diagnosis : Epilepsies, Myoclonic.
- drug therapy : Epilepsies, Myoclonic.
- etiology : Subacute Sclerosing Panencephalitis.
- immunology : Measles virus.
- pathogenicity : Measles virus.
- pathology : Brain, Subacute Sclerosing Panencephalitis.
- Adolescent, Adult, Age of Onset, Cognition Disorders, Electroencephalography, Female, Humans, Magnetic Resonance Imaging, Male, Movement Disorders, Tomography, X-Ray Computed, Vision Disorders.
Abstract
Subacute sclerosing panencephalitis (SSPE) is mainly thought of as a disorder of childhood and adolescence and may not be readily recognized when presenting later in life. Prior reports have suggested that adult-onset SSPE may have atypical features. We have added two cases to the existing literature on adult-onset SSPE, compared them with a more classic juvenile presentation, and extensively reviewed those reports that were published after the etiological link with the measles virus had been established. Adult-onset SSPE patients present at a mean age of 25.4 years (range 20-35 years). They have a higher proportion of either negative history of measles exposure or undocumented history by the reporting authors. Those with available history of measles exposure tend to have it either earlier (younger than 3 years old) or later (after 9 years) than the usual childhood measles infection. Where the primary infection is known, unusually long measles-to-SSPE intervals have been documented, ranging from 14 to 22 years. None of the cases followed measles vaccination. Visual symptomatology was very frequent, with 8 of the 13 cases reviewed having a purely ophthalmological presentation; only 2 patients presented with behavioral changes. Although the course of the disease was progressive and fatal in the majority, there appeared to be a higher rate of spontaneous remission as compared with childhood-onset SSPE. Myoclonus, spastic hemiparesis, bradykinesia, and rigidity were the predominant motor manifestations. Neuropathology revealed cortical and subcortical gray matter involvement preferentially of the occipital lobes, thalamus, and putamen. The importance of recognizing the spectrum of potential presentations of SSPE and providing an early diagnosis will increase as more effective treatments become available.
DOI: 10.1002/mds.870120313
PubMed: 9159729
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pubmed:9159729Le document en format XML
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Suchowersky</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="1997">1997</date><idno type="RBID">pubmed:9159729</idno><idno type="pmid">9159729</idno><idno type="doi">10.1002/mds.870120313</idno><idno type="wicri:Area/PubMed/Corpus">004677</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Adult-onset subacute sclerosing panencephalitis: case reports and review of the literature.</title><author><name sortKey="Singer, C" sort="Singer, C" uniqKey="Singer C" first="C" last="Singer">C. Singer</name><affiliation><nlm:affiliation>Department of Neurology, University of Miami School of Medicine, FL 33136, USA.</nlm:affiliation></affiliation></author><author><name sortKey="Lang, A E" sort="Lang, A E" uniqKey="Lang A" first="A E" last="Lang">A E Lang</name></author><author><name sortKey="Suchowersky, O" sort="Suchowersky, O" uniqKey="Suchowersky O" first="O" last="Suchowersky">O. Suchowersky</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="1997" type="published">1997</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Age of Onset</term><term>Anticonvulsants (therapeutic use)</term><term>Brain (blood supply)</term><term>Brain (pathology)</term><term>Cognition Disorders</term><term>Electroencephalography</term><term>Epilepsies, Myoclonic (diagnosis)</term><term>Epilepsies, Myoclonic (drug therapy)</term><term>Female</term><term>Humans</term><term>Immunoglobulin G (immunology)</term><term>Magnetic Resonance Imaging</term><term>Male</term><term>Measles virus (immunology)</term><term>Measles virus (pathogenicity)</term><term>Movement Disorders</term><term>Subacute Sclerosing Panencephalitis (etiology)</term><term>Subacute Sclerosing Panencephalitis (pathology)</term><term>Tomography, X-Ray Computed</term><term>Vision Disorders</term></keywords><keywords scheme="MESH" type="chemical" qualifier="immunology" xml:lang="en"><term>Immunoglobulin G</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Anticonvulsants</term></keywords><keywords scheme="MESH" qualifier="blood supply" xml:lang="en"><term>Brain</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Epilepsies, Myoclonic</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Epilepsies, Myoclonic</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Subacute Sclerosing Panencephalitis</term></keywords><keywords scheme="MESH" qualifier="immunology" xml:lang="en"><term>Measles virus</term></keywords><keywords scheme="MESH" qualifier="pathogenicity" xml:lang="en"><term>Measles virus</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain</term><term>Subacute Sclerosing Panencephalitis</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Age of Onset</term><term>Cognition Disorders</term><term>Electroencephalography</term><term>Female</term><term>Humans</term><term>Magnetic Resonance Imaging</term><term>Male</term><term>Movement Disorders</term><term>Tomography, X-Ray Computed</term><term>Vision Disorders</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Subacute sclerosing panencephalitis (SSPE) is mainly thought of as a disorder of childhood and adolescence and may not be readily recognized when presenting later in life. Prior reports have suggested that adult-onset SSPE may have atypical features. We have added two cases to the existing literature on adult-onset SSPE, compared them with a more classic juvenile presentation, and extensively reviewed those reports that were published after the etiological link with the measles virus had been established. Adult-onset SSPE patients present at a mean age of 25.4 years (range 20-35 years). They have a higher proportion of either negative history of measles exposure or undocumented history by the reporting authors. Those with available history of measles exposure tend to have it either earlier (younger than 3 years old) or later (after 9 years) than the usual childhood measles infection. Where the primary infection is known, unusually long measles-to-SSPE intervals have been documented, ranging from 14 to 22 years. None of the cases followed measles vaccination. Visual symptomatology was very frequent, with 8 of the 13 cases reviewed having a purely ophthalmological presentation; only 2 patients presented with behavioral changes. Although the course of the disease was progressive and fatal in the majority, there appeared to be a higher rate of spontaneous remission as compared with childhood-onset SSPE. Myoclonus, spastic hemiparesis, bradykinesia, and rigidity were the predominant motor manifestations. Neuropathology revealed cortical and subcortical gray matter involvement preferentially of the occipital lobes, thalamus, and putamen. The importance of recognizing the spectrum of potential presentations of SSPE and providing an early diagnosis will increase as more effective treatments become available.</div></front></TEI><pubmed><MedlineCitation Owner="NLM" Status="MEDLINE"><PMID Version="1">9159729</PMID><DateCreated><Year>1997</Year><Month>07</Month><Day>28</Day></DateCreated><DateCompleted><Year>1997</Year><Month>07</Month><Day>28</Day></DateCompleted><DateRevised><Year>2005</Year><Month>11</Month><Day>16</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0885-3185</ISSN><JournalIssue CitedMedium="Print"><Volume>12</Volume><Issue>3</Issue><PubDate><Year>1997</Year><Month>May</Month></PubDate></JournalIssue><Title>Movement disorders : official journal of the Movement Disorder Society</Title><ISOAbbreviation>Mov. Disord.</ISOAbbreviation></Journal><ArticleTitle>Adult-onset subacute sclerosing panencephalitis: case reports and review of the literature.</ArticleTitle><Pagination><MedlinePgn>342-53</MedlinePgn></Pagination><Abstract><AbstractText>Subacute sclerosing panencephalitis (SSPE) is mainly thought of as a disorder of childhood and adolescence and may not be readily recognized when presenting later in life. Prior reports have suggested that adult-onset SSPE may have atypical features. We have added two cases to the existing literature on adult-onset SSPE, compared them with a more classic juvenile presentation, and extensively reviewed those reports that were published after the etiological link with the measles virus had been established. Adult-onset SSPE patients present at a mean age of 25.4 years (range 20-35 years). They have a higher proportion of either negative history of measles exposure or undocumented history by the reporting authors. Those with available history of measles exposure tend to have it either earlier (younger than 3 years old) or later (after 9 years) than the usual childhood measles infection. Where the primary infection is known, unusually long measles-to-SSPE intervals have been documented, ranging from 14 to 22 years. None of the cases followed measles vaccination. Visual symptomatology was very frequent, with 8 of the 13 cases reviewed having a purely ophthalmological presentation; only 2 patients presented with behavioral changes. Although the course of the disease was progressive and fatal in the majority, there appeared to be a higher rate of spontaneous remission as compared with childhood-onset SSPE. Myoclonus, spastic hemiparesis, bradykinesia, and rigidity were the predominant motor manifestations. Neuropathology revealed cortical and subcortical gray matter involvement preferentially of the occipital lobes, thalamus, and putamen. The importance of recognizing the spectrum of potential presentations of SSPE and providing an early diagnosis will increase as more effective treatments become available.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Singer</LastName><ForeName>C</ForeName><Initials>C</Initials><AffiliationInfo><Affiliation>Department of Neurology, University of Miami School of Medicine, FL 33136, USA.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Lang</LastName><ForeName>A E</ForeName><Initials>AE</Initials></Author><Author ValidYN="Y"><LastName>Suchowersky</LastName><ForeName>O</ForeName><Initials>O</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D002363">Case Reports</PublicationType><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D016454">Review</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>UNITED STATES</Country><MedlineTA>Mov Disord</MedlineTA><NlmUniqueID>8610688</NlmUniqueID><ISSNLinking>0885-3185</ISSNLinking></MedlineJournalInfo><ChemicalList><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D000927">Anticonvulsants</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D007074">Immunoglobulin G</NameOfSubstance></Chemical></ChemicalList><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName MajorTopicYN="N" UI="D000293">Adolescent</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D000328">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D017668">Age of Onset</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D000927">Anticonvulsants</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000627">therapeutic use</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D001921">Brain</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000098">blood supply</QualifierName><QualifierName MajorTopicYN="N" UI="Q000473">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D003072">Cognition Disorders</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D004569">Electroencephalography</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D004831">Epilepsies, Myoclonic</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="N" UI="Q000188">drug therapy</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D005260">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D006801">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D007074">Immunoglobulin G</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000276">immunology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008279">Magnetic Resonance Imaging</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008297">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008459">Measles virus</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000276">immunology</QualifierName><QualifierName MajorTopicYN="N" UI="Q000472">pathogenicity</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D009069">Movement Disorders</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D013344">Subacute Sclerosing Panencephalitis</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000209">etiology</QualifierName><QualifierName MajorTopicYN="Y" UI="Q000473">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D014057">Tomography, X-Ray Computed</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D014786">Vision Disorders</DescriptorName></MeshHeading></MeshHeadingList><NumberOfReferences>24</NumberOfReferences></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>1997</Year><Month>5</Month><Day>1</Day></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>1997</Year><Month>5</Month><Day>1</Day><Hour>0</Hour><Minute>1</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>1997</Year><Month>5</Month><Day>1</Day><Hour>0</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">9159729</ArticleId><ArticleId IdType="doi">10.1002/mds.870120313</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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