Brainstem encephalopathy with stimulus-sensitive myoclonus leading to respiratory arrest, but with recovery: a description of two cases and review of the literature.
Identifieur interne : 004755 ( PubMed/Corpus ); précédent : 004754; suivant : 004756Brainstem encephalopathy with stimulus-sensitive myoclonus leading to respiratory arrest, but with recovery: a description of two cases and review of the literature.
Auteurs : D M Kullmann ; R S Howard ; D H Miller ; N P Hirsch ; P. Brown ; C D MarsdenSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 1996.
English descriptors
- KwdEn :
- MESH :
- physiology : Arousal, Reflex, Abnormal, Reflex, Startle.
- physiopathology : Brain Stem, Encephalomyelitis, Myoclonus, Respiratory Insufficiency.
- Adult, Follow-Up Studies, Humans, Male, Respiration, Artificial.
Abstract
Two unrelated patients developed bulbar symptoms, followed within several weeks by spontaneous myoclonus and painful, generalized, stimulus-sensitive jerks triggered by unexpected noises and cutaneous stimuli. They progressed to respiratory arrest and required mechanical ventilation, but both patients subsequently made an almost full recovery. These cases stress the importance of persevering with supportive treatment despite rapid progression of this severe generalized movement disorder. The relationship of brainstem reflex myoclonus to hyperekplexia, progressive encephalomyelitis with rigidity, and the stiff-man syndrome is discussed.
DOI: 10.1002/mds.870110618
PubMed: 8914099
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pubmed:8914099Le document en format XML
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Brown</name></author><author><name sortKey="Marsden, C D" sort="Marsden, C D" uniqKey="Marsden C" first="C D" last="Marsden">C D Marsden</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="1996">1996</date><idno type="RBID">pubmed:8914099</idno><idno type="pmid">8914099</idno><idno type="doi">10.1002/mds.870110618</idno><idno type="wicri:Area/PubMed/Corpus">004755</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Brainstem encephalopathy with stimulus-sensitive myoclonus leading to respiratory arrest, but with recovery: a description of two cases and review of the literature.</title><author><name sortKey="Kullmann, D M" sort="Kullmann, D M" uniqKey="Kullmann D" first="D M" last="Kullmann">D M Kullmann</name><affiliation><nlm:affiliation>National Hospital for Neurology and Neurosurgery, Institute of Neurology, London, England.</nlm:affiliation></affiliation></author><author><name sortKey="Howard, R S" sort="Howard, R S" uniqKey="Howard R" first="R S" last="Howard">R S Howard</name></author><author><name sortKey="Miller, D H" sort="Miller, D H" uniqKey="Miller D" first="D H" last="Miller">D H Miller</name></author><author><name sortKey="Hirsch, N P" sort="Hirsch, N P" uniqKey="Hirsch N" first="N P" last="Hirsch">N P Hirsch</name></author><author><name sortKey="Brown, P" sort="Brown, P" uniqKey="Brown P" first="P" last="Brown">P. Brown</name></author><author><name sortKey="Marsden, C D" sort="Marsden, C D" uniqKey="Marsden C" first="C D" last="Marsden">C D Marsden</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="1996" type="published">1996</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Arousal (physiology)</term><term>Brain Stem (physiopathology)</term><term>Encephalomyelitis (physiopathology)</term><term>Follow-Up Studies</term><term>Humans</term><term>Male</term><term>Myoclonus (physiopathology)</term><term>Reflex, Abnormal (physiology)</term><term>Reflex, Startle (physiology)</term><term>Respiration, Artificial</term><term>Respiratory Insufficiency (physiopathology)</term></keywords><keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Arousal</term><term>Reflex, Abnormal</term><term>Reflex, Startle</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Brain Stem</term><term>Encephalomyelitis</term><term>Myoclonus</term><term>Respiratory Insufficiency</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Follow-Up Studies</term><term>Humans</term><term>Male</term><term>Respiration, Artificial</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Two unrelated patients developed bulbar symptoms, followed within several weeks by spontaneous myoclonus and painful, generalized, stimulus-sensitive jerks triggered by unexpected noises and cutaneous stimuli. They progressed to respiratory arrest and required mechanical ventilation, but both patients subsequently made an almost full recovery. These cases stress the importance of persevering with supportive treatment despite rapid progression of this severe generalized movement disorder. The relationship of brainstem reflex myoclonus to hyperekplexia, progressive encephalomyelitis with rigidity, and the stiff-man syndrome is discussed.</div></front></TEI><pubmed><MedlineCitation Owner="NLM" Status="MEDLINE"><PMID Version="1">8914099</PMID><DateCreated><Year>1997</Year><Month>03</Month><Day>20</Day></DateCreated><DateCompleted><Year>1997</Year><Month>03</Month><Day>20</Day></DateCompleted><DateRevised><Year>2014</Year><Month>11</Month><Day>20</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0885-3185</ISSN><JournalIssue CitedMedium="Print"><Volume>11</Volume><Issue>6</Issue><PubDate><Year>1996</Year><Month>Nov</Month></PubDate></JournalIssue><Title>Movement disorders : official journal of the Movement Disorder Society</Title><ISOAbbreviation>Mov. Disord.</ISOAbbreviation></Journal><ArticleTitle>Brainstem encephalopathy with stimulus-sensitive myoclonus leading to respiratory arrest, but with recovery: a description of two cases and review of the literature.</ArticleTitle><Pagination><MedlinePgn>715-8</MedlinePgn></Pagination><Abstract><AbstractText>Two unrelated patients developed bulbar symptoms, followed within several weeks by spontaneous myoclonus and painful, generalized, stimulus-sensitive jerks triggered by unexpected noises and cutaneous stimuli. They progressed to respiratory arrest and required mechanical ventilation, but both patients subsequently made an almost full recovery. These cases stress the importance of persevering with supportive treatment despite rapid progression of this severe generalized movement disorder. The relationship of brainstem reflex myoclonus to hyperekplexia, progressive encephalomyelitis with rigidity, and the stiff-man syndrome is discussed.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Kullmann</LastName><ForeName>D M</ForeName><Initials>DM</Initials><AffiliationInfo><Affiliation>National Hospital for Neurology and Neurosurgery, Institute of Neurology, London, England.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Howard</LastName><ForeName>R S</ForeName><Initials>RS</Initials></Author><Author ValidYN="Y"><LastName>Miller</LastName><ForeName>D H</ForeName><Initials>DH</Initials></Author><Author ValidYN="Y"><LastName>Hirsch</LastName><ForeName>N P</ForeName><Initials>NP</Initials></Author><Author ValidYN="Y"><LastName>Brown</LastName><ForeName>P</ForeName><Initials>P</Initials></Author><Author ValidYN="Y"><LastName>Marsden</LastName><ForeName>C D</ForeName><Initials>CD</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><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName MajorTopicYN="N" UI="D000328">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D001143">Arousal</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000502">physiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D001933">Brain Stem</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D004679">Encephalomyelitis</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D005500">Follow-Up Studies</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D006801">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008297">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D009207">Myoclonus</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D012021">Reflex, Abnormal</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000502">physiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D013216">Reflex, Startle</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000502">physiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D012121">Respiration, Artificial</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D012131">Respiratory Insufficiency</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000503">physiopathology</QualifierName></MeshHeading></MeshHeadingList><NumberOfReferences>22</NumberOfReferences></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>1996</Year><Month>11</Month><Day>1</Day></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>1996</Year><Month>11</Month><Day>1</Day><Hour>0</Hour><Minute>1</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>1996</Year><Month>11</Month><Day>1</Day><Hour>0</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">8914099</ArticleId><ArticleId IdType="doi">10.1002/mds.870110618</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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