Valproate-induced hyperammonaemia superimposed upon severe neuropsychiatric lupus: a case report and review of the literature
Identifieur interne : 000E14 ( Istex/Corpus ); précédent : 000E13; suivant : 000E15Valproate-induced hyperammonaemia superimposed upon severe neuropsychiatric lupus: a case report and review of the literature
Auteurs : Estee Chan ; Fiona McqueenSource :
- Clinical Rheumatology [ 0770-3198 ] ; 2013-03-01.
English descriptors
Abstract
Abstract: This paper presents a case of systemic lupus erythematosus (SLE) with neuropsychiatric features, where the outcome was influenced by the development of hyperammonaemia, probably induced by sodium valproate. A case of severe SLE occurring in a 20-year-old Maori girl is described. Her disease had been characterised by neuropsychiatric features for several years, culminating in persistent seizure activity at the time of her final presentation. Her management with anticonvulsants was complicated by the development of intractable hyperammonaemia which contributed to irreversible clinical deterioration. We have reviewed the English literature for reports of valproate-related hyperammonaemia which has often been described in the setting of seizure and mood disorders. This is the first case where it has been reported, superimposed upon severe neuropsychiatric SLE (NP-SLE). The mechanism by which valproate induces hyperammonaemia remains incompletely understood but is likely to relate to the urea cycle. Under normal metabolic conditions, acyl-CoA is transported into the mitochondria via a carnitine transport system. It is then converted to acetyl-CoA via β-oxidation and eventually to N-acetyl glutamate. This pathway can be interrupted by the introduction of sodium valproate, leading to a reduction of free coenzyme A, acetyl-CoA and carnitine, and resulting in the decreased availability of cofactors necessary for the function of the urea cycle. As this is the primary means of ammonia metabolism, serious elevation in serum ammonia levels may occur in patients on this anticonvulsant medication. In this patient with active NP-SLE, the combined autoimmune and metabolic brain insult contributed to a fatal outcome.
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DOI: 10.1007/s10067-012-2150-x
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ISTEX:34F4459AADFA5B0BEF62675E32776718CB54BC67Le document en format XML
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A case of severe SLE occurring in a 20-year-old Maori girl is described. Her disease had been characterised by neuropsychiatric features for several years, culminating in persistent seizure activity at the time of her final presentation. Her management with anticonvulsants was complicated by the development of intractable hyperammonaemia which contributed to irreversible clinical deterioration. We have reviewed the English literature for reports of valproate-related hyperammonaemia which has often been described in the setting of seizure and mood disorders. This is the first case where it has been reported, superimposed upon severe neuropsychiatric SLE (NP-SLE). The mechanism by which valproate induces hyperammonaemia remains incompletely understood but is likely to relate to the urea cycle. Under normal metabolic conditions, acyl-CoA is transported into the mitochondria via a carnitine transport system. It is then converted to acetyl-CoA via β-oxidation and eventually to N-acetyl glutamate. This pathway can be interrupted by the introduction of sodium valproate, leading to a reduction of free coenzyme A, acetyl-CoA and carnitine, and resulting in the decreased availability of cofactors necessary for the function of the urea cycle. As this is the primary means of ammonia metabolism, serious elevation in serum ammonia levels may occur in patients on this anticonvulsant medication. In this patient with active NP-SLE, the combined autoimmune and metabolic brain insult contributed to a fatal outcome.</div></front></TEI><istex><corpusName>springer-journals</corpusName><author><json:item><name>Estee Chan</name><affiliations><json:string>Department of Rheumatology, Greenlane Clinical Centre, Auckland District Health Board, Private Bag 92024, Auckland Mail Centre, 1142, Auckland, New Zealand</json:string><json:string>E-mail: cheukyanestee@yahoo.co.nz</json:string></affiliations></json:item><json:item><name>Fiona McQueen</name><affiliations><json:string>Department of Rheumatology, Greenlane Clinical Centre, Auckland District Health Board, Private Bag 92024, Auckland Mail Centre, 1142, Auckland, New Zealand</json:string><json:string>Department of Molecular Medicine and Pathology, University of Auckland, Auckland, New Zealand</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>Anticonvulsants</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Hyperammonaemia</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Sodium valproate</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Systemic lupus erythematosus</value></json:item></subject><articleId><json:string>2150</json:string><json:string>s10067-012-2150-x</json:string></articleId><arkIstex>ark:/67375/VQC-41RJD0X9-C</arkIstex><language><json:string>eng</json:string></language><originalGenre><json:string>ReviewPaper</json:string></originalGenre><abstract>Abstract: This paper presents a case of systemic lupus erythematosus (SLE) with neuropsychiatric features, where the outcome was influenced by the development of hyperammonaemia, probably induced by sodium valproate. A case of severe SLE occurring in a 20-year-old Maori girl is described. Her disease had been characterised by neuropsychiatric features for several years, culminating in persistent seizure activity at the time of her final presentation. Her management with anticonvulsants was complicated by the development of intractable hyperammonaemia which contributed to irreversible clinical deterioration. We have reviewed the English literature for reports of valproate-related hyperammonaemia which has often been described in the setting of seizure and mood disorders. This is the first case where it has been reported, superimposed upon severe neuropsychiatric SLE (NP-SLE). The mechanism by which valproate induces hyperammonaemia remains incompletely understood but is likely to relate to the urea cycle. Under normal metabolic conditions, acyl-CoA is transported into the mitochondria via a carnitine transport system. It is then converted to acetyl-CoA via β-oxidation and eventually to N-acetyl glutamate. This pathway can be interrupted by the introduction of sodium valproate, leading to a reduction of free coenzyme A, acetyl-CoA and carnitine, and resulting in the decreased availability of cofactors necessary for the function of the urea cycle. As this is the primary means of ammonia metabolism, serious elevation in serum ammonia levels may occur in patients on this anticonvulsant medication. 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was influenced by the development of hyperammonaemia, probably induced by sodium valproate. A case of severe SLE occurring in a 20-year-old Maori girl is described. Her disease had been characterised by neuropsychiatric features for several years, culminating in persistent seizure activity at the time of her final presentation. Her management with anticonvulsants was complicated by the development of intractable hyperammonaemia which contributed to irreversible clinical deterioration. We have reviewed the English literature for reports of valproate-related hyperammonaemia which has often been described in the setting of seizure and mood disorders. This is the first case where it has been reported, superimposed upon severe neuropsychiatric SLE (NP-SLE). The mechanism by which valproate induces hyperammonaemia remains incompletely understood but is likely to relate to the urea cycle. Under normal metabolic conditions, acyl-CoA is transported into the mitochondria via a carnitine transport system. It is then converted to acetyl-CoA via β-oxidation and eventually to N-acetyl glutamate. This pathway can be interrupted by the introduction of sodium valproate, leading to a reduction of free coenzyme A, acetyl-CoA and carnitine, and resulting in the decreased availability of cofactors necessary for the function of the urea cycle. As this is the primary means of ammonia metabolism, serious elevation in serum ammonia levels may occur in patients on this anticonvulsant medication. 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CorrespondingAffiliationID="Aff1"><AuthorName DisplayOrder="Western"><GivenName>Estee</GivenName><FamilyName>Chan</FamilyName></AuthorName><Contact><Phone>+64-9-3737999</Phone><Fax>+64-9-3737677</Fax><Email>cheukyanestee@yahoo.co.nz</Email></Contact></Author><Author AffiliationIDS="Aff1 Aff2"><AuthorName DisplayOrder="Western"><GivenName>Fiona</GivenName><FamilyName>McQueen</FamilyName></AuthorName></Author><Affiliation ID="Aff1"><OrgDivision>Department of Rheumatology, Greenlane Clinical Centre</OrgDivision><OrgName>Auckland District Health Board</OrgName><OrgAddress><Street>Private Bag 92024, Auckland Mail Centre</Street><City>Auckland</City><Postcode>1142</Postcode><Country Code="NZ">New Zealand</Country></OrgAddress></Affiliation><Affiliation ID="Aff2"><OrgDivision>Department of Molecular Medicine and Pathology</OrgDivision><OrgName>University of Auckland</OrgName><OrgAddress><City>Auckland</City><Country Code="NZ">New Zealand</Country></OrgAddress></Affiliation></AuthorGroup><Abstract ID="Abs1" Language="En" OutputMedium="All"><Heading>Abstract</Heading><Para>This paper presents a case of systemic lupus erythematosus (SLE) with neuropsychiatric features, where the outcome was influenced by the development of hyperammonaemia, probably induced by sodium valproate. A case of severe SLE occurring in a 20-year-old Maori girl is described. Her disease had been characterised by neuropsychiatric features for several years, culminating in persistent seizure activity at the time of her final presentation. Her management with anticonvulsants was complicated by the development of intractable hyperammonaemia which contributed to irreversible clinical deterioration. We have reviewed the English literature for reports of valproate-related hyperammonaemia which has often been described in the setting of seizure and mood disorders. This is the first case where it has been reported, superimposed upon severe neuropsychiatric SLE (NP-SLE). The mechanism by which valproate induces hyperammonaemia remains incompletely understood but is likely to relate to the urea cycle. Under normal metabolic conditions, acyl-CoA is transported into the mitochondria via a carnitine transport system. It is then converted to acetyl-CoA via β-oxidation and eventually to <Emphasis Type="Italic">N</Emphasis>-acetyl glutamate. This pathway can be interrupted by the introduction of sodium valproate, leading to a reduction of free coenzyme A, acetyl-CoA and carnitine, and resulting in the decreased availability of cofactors necessary for the function of the urea cycle. As this is the primary means of ammonia metabolism, serious elevation in serum ammonia levels may occur in patients on this anticonvulsant medication. In this patient with active NP-SLE, the combined autoimmune and metabolic brain insult contributed to a fatal outcome.</Para></Abstract><KeywordGroup Language="En" OutputMedium="All"><Heading>Keywords</Heading><Keyword>Anticonvulsants</Keyword><Keyword>Hyperammonaemia</Keyword><Keyword>Sodium valproate</Keyword><Keyword>Systemic lupus erythematosus</Keyword></KeywordGroup><ArticleNote Type="ESMHint"><Heading>Electronic supplementary material</Heading><SimplePara>The online version of this article (doi:<ExternalRef><RefSource>10.1007/s10067-012-2150-x</RefSource><RefTarget Address="10.1007/s10067-012-2150-x" TargetType="DOI"></RefTarget></ExternalRef>) contains supplementary material, which is available to authorized users.</SimplePara></ArticleNote></ArticleHeader><NoBody></NoBody></Article></Issue></Volume></Journal></Publisher></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Valproate-induced hyperammonaemia superimposed upon severe neuropsychiatric lupus: a case report and review of the literature</title></titleInfo><titleInfo type="alternative" contentType="CDATA"><title>Valproate-induced hyperammonaemia superimposed upon severe neuropsychiatric lupus: a case report and review of the literature</title></titleInfo><name type="personal" displayLabel="corresp"><namePart type="given">Estee</namePart><namePart type="family">Chan</namePart><affiliation>Department of Rheumatology, Greenlane Clinical Centre, Auckland District Health Board, Private Bag 92024, Auckland Mail Centre, 1142, Auckland, New Zealand</affiliation><affiliation>E-mail: cheukyanestee@yahoo.co.nz</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Fiona</namePart><namePart type="family">McQueen</namePart><affiliation>Department of Rheumatology, Greenlane Clinical Centre, Auckland District Health Board, Private Bag 92024, Auckland Mail Centre, 1142, Auckland, New Zealand</affiliation><affiliation>Department of Molecular Medicine and Pathology, University of Auckland, Auckland, New Zealand</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="review-article" displayLabel="ReviewPaper" authority="ISTEX" authorityURI="https://content-type.data.istex.fr" valueURI="https://content-type.data.istex.fr/ark:/67375/XTP-L5L7X3NF-P">review-article</genre><originInfo><publisher>Springer-Verlag</publisher><place><placeTerm type="text">London</placeTerm></place><dateCreated encoding="w3cdtf">2012-11-09</dateCreated><dateIssued encoding="w3cdtf">2013-03-01</dateIssued><copyrightDate encoding="w3cdtf">2012</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><abstract lang="en">Abstract: This paper presents a case of systemic lupus erythematosus (SLE) with neuropsychiatric features, where the outcome was influenced by the development of hyperammonaemia, probably induced by sodium valproate. A case of severe SLE occurring in a 20-year-old Maori girl is described. Her disease had been characterised by neuropsychiatric features for several years, culminating in persistent seizure activity at the time of her final presentation. Her management with anticonvulsants was complicated by the development of intractable hyperammonaemia which contributed to irreversible clinical deterioration. We have reviewed the English literature for reports of valproate-related hyperammonaemia which has often been described in the setting of seizure and mood disorders. This is the first case where it has been reported, superimposed upon severe neuropsychiatric SLE (NP-SLE). The mechanism by which valproate induces hyperammonaemia remains incompletely understood but is likely to relate to the urea cycle. Under normal metabolic conditions, acyl-CoA is transported into the mitochondria via a carnitine transport system. It is then converted to acetyl-CoA via β-oxidation and eventually to N-acetyl glutamate. This pathway can be interrupted by the introduction of sodium valproate, leading to a reduction of free coenzyme A, acetyl-CoA and carnitine, and resulting in the decreased availability of cofactors necessary for the function of the urea cycle. As this is the primary means of ammonia metabolism, serious elevation in serum ammonia levels may occur in patients on this anticonvulsant medication. In this patient with active NP-SLE, the combined autoimmune and metabolic brain insult contributed to a fatal outcome.</abstract><note>Case Based Review</note><subject lang="en"><genre>Keywords</genre><topic>Anticonvulsants</topic><topic>Hyperammonaemia</topic><topic>Sodium valproate</topic><topic>Systemic lupus erythematosus</topic></subject><relatedItem type="host"><titleInfo><title>Clinical Rheumatology</title><subTitle>Journal of the International League of Associations for Rheumatology</subTitle></titleInfo><titleInfo type="abbreviated"><title>Clin Rheumatol</title></titleInfo><genre type="journal" authority="ISTEX" authorityURI="https://publication-type.data.istex.fr" valueURI="https://publication-type.data.istex.fr/ark:/67375/JMC-0GLKJH51-B">journal</genre><originInfo><publisher>Springer</publisher><dateIssued encoding="w3cdtf">2013-03-10</dateIssued><copyrightDate encoding="w3cdtf">2013</copyrightDate></originInfo><subject><genre>Journal-Subject-Collection</genre><topic authority="SpringerSubjectCodes" authorityURI="SC11">Medicine</topic></subject><subject><genre>Journal-Subject-Group</genre><topic authority="SpringerSubjectCodes" authorityURI="SCH">Medicine & Public Health</topic><topic authority="SpringerSubjectCodes" authorityURI="SCH57009">Rheumatology</topic></subject><identifier type="ISSN">0770-3198</identifier><identifier type="eISSN">1434-9949</identifier><identifier type="JournalID">10067</identifier><identifier type="IssueArticleCount">21</identifier><identifier type="VolumeIssueCount">12</identifier><part><date>2013</date><detail type="volume"><number>32</number><caption>vol.</caption></detail><detail type="issue"><number>3</number><caption>no.</caption></detail><extent unit="pages"><start>403</start><end>407</end></extent></part><recordInfo><recordOrigin>Clinical Rheumatology, 2013</recordOrigin></recordInfo></relatedItem><identifier type="istex">34F4459AADFA5B0BEF62675E32776718CB54BC67</identifier><identifier type="ark">ark:/67375/VQC-41RJD0X9-C</identifier><identifier type="DOI">10.1007/s10067-012-2150-x</identifier><identifier type="ArticleID">2150</identifier><identifier type="ArticleID">s10067-012-2150-x</identifier><accessCondition type="use and reproduction" contentType="copyright">Clinical Rheumatology, 2012</accessCondition><recordInfo><recordContentSource authority="ISTEX" authorityURI="https://loaded-corpus.data.istex.fr" valueURI="https://loaded-corpus.data.istex.fr/ark:/67375/XBH-3XSW68JL-F">springer</recordContentSource><recordOrigin>Clinical Rheumatology, 2012</recordOrigin></recordInfo></mods><json:item><extension>json</extension><original>false</original><mimetype>application/json</mimetype><uri>https://api.istex.fr/ark:/67375/VQC-41RJD0X9-C/record.json</uri></json:item></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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|texte= Valproate-induced hyperammonaemia superimposed upon severe neuropsychiatric lupus: a case report and review of the literature
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