Mild encephalopathy with reversible splenial lesion: an important differential of encephalitis.
Identifieur interne : 001655 ( Main/Exploration ); précédent : 001654; suivant : 001656Mild encephalopathy with reversible splenial lesion: an important differential of encephalitis.
Auteurs : Amy Ka [Australie] ; Philip Britton [Australie] ; Christopher Troedson [Australie] ; Richard Webster [Australie] ; Peter Procopis [Australie] ; Joanne Ging [Australie] ; Yew Wee Chua [Australie] ; Adam Buckmaster [Australie] ; Nicholas Wood [Australie] ; Cheryl Jones [Australie] ; Russell C. Dale [Australie]Source :
- European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society [ 1532-2130 ] ; 2015.
Descripteurs français
- KwdFr :
- MESH :
- anatomopathologie : Encéphalopathies.
- Australie, Encéphalopathies, Enfant, Enfant d'âge préscolaire, Femelle, Humains, Imagerie par résonance magnétique, Mâle, Syndrome.
English descriptors
- KwdEn :
- MESH :
- complications : Brain Diseases.
- pathology : Brain Diseases.
- Australia, Child, Child, Preschool, Female, Humans, Magnetic Resonance Imaging, Male, Syndrome.
Abstract
Mild encephalopathy with a reversible splenial lesion (MERS) is a clinico-radiological syndrome characterized by a transient mild encephalopathy and a reversible lesion in the splenium of the corpus callosum on MRI. This syndrome has almost universally been described in children from Japan and East Asia. Here we describe seven cases of MERS occurring in Caucasian Australian children from one centre seen over a 3 year period. All patients had a fever-associated encephalopathy (n = 7), which presented with confusion (n = 4), irritability (n = 3), lethargy (n = 3), slurred speech (n = 3), drowsiness (n = 2) and hallucinations (n = 2). Other neurological symptoms included ataxia (n = 5) and seizures (n = 1). These symptoms resolved rapidly over 4-6 days followed by complete neurological recovery. In all patients, MRI performed within 1-3 days of onset of encephalopathy demonstrated a symmetrical diffusion-restricted lesion in the splenium of the corpus callosum. Three patients had additional lesions involving other parts of the corpus callosum and adjacent periventricular white matter. These same three patients had mild persisting white matter changes evident at followup MRI, while the other patients had complete resolution of radiological changes. A potential trigger was present in five of the seven cases: Kawasaki disease, Salmonella, cytomegalovirus, influenza B and adenovirus (all n = 1). Elevated white cell count (n = 4), elevated C reactive protein (n = 5) and hyponatremia (n = 6) were commonly observed. CSF was performed in four patients, which showed no pleocytosis. This case series of MERS demonstrates this condition occurs outside of East Asia and is an important differential to consider in children presenting with acute encephalopathy.
DOI: 10.1016/j.ejpn.2015.01.011
PubMed: 25707871
Affiliations:
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Discipline of Paediatrics and Child Health, University of Sydney, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>Department of Paediatrics, The Children's Hospital, Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney</wicri:regionArea><placeName><settlement type="city">Sydney</settlement><region type="état">Nouvelle-Galles du Sud</region></placeName><orgName type="university">Université de Sydney</orgName></affiliation></author><author><name sortKey="Jones, Cheryl" sort="Jones, Cheryl" uniqKey="Jones C" first="Cheryl" last="Jones">Cheryl Jones</name><affiliation wicri:level="4"><nlm:affiliation>Department of Infectious Disease, The Children's Hospital, Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>Department of Infectious Disease, The Children's Hospital, Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney</wicri:regionArea><placeName><settlement type="city">Sydney</settlement><region type="état">Nouvelle-Galles du Sud</region></placeName><orgName type="university">Université de Sydney</orgName></affiliation></author><author><name sortKey="Dale, Russell C" sort="Dale, Russell C" uniqKey="Dale R" first="Russell C" last="Dale">Russell C. Dale</name><affiliation wicri:level="4"><nlm:affiliation>TY Nelson Department of Neurology and Neurosurgery, The Children's Hospital, Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney, Australia. 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Department of Paediatrics, The Children's Hospital, Westmead, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>TY Nelson Department of Neurology and Neurosurgery, The Children's Hospital, Westmead, Australia; Department of Paediatrics, The Children's Hospital, Westmead</wicri:regionArea><wicri:noRegion>Westmead</wicri:noRegion></affiliation></author><author><name sortKey="Britton, Philip" sort="Britton, Philip" uniqKey="Britton P" first="Philip" last="Britton">Philip Britton</name><affiliation wicri:level="1"><nlm:affiliation>Department of Infectious Disease, The Children's Hospital, Westmead, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>Department of Infectious Disease, The Children's Hospital, Westmead</wicri:regionArea><wicri:noRegion>Westmead</wicri:noRegion></affiliation></author><author><name sortKey="Troedson, Christopher" sort="Troedson, Christopher" uniqKey="Troedson C" first="Christopher" last="Troedson">Christopher Troedson</name><affiliation wicri:level="1"><nlm:affiliation>TY Nelson Department of Neurology and Neurosurgery, The Children's Hospital, Westmead, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>TY Nelson Department of Neurology and Neurosurgery, The Children's Hospital, Westmead</wicri:regionArea><wicri:noRegion>Westmead</wicri:noRegion></affiliation></author><author><name sortKey="Webster, Richard" sort="Webster, Richard" uniqKey="Webster R" first="Richard" last="Webster">Richard Webster</name><affiliation wicri:level="1"><nlm:affiliation>TY Nelson Department of Neurology and Neurosurgery, The Children's Hospital, Westmead, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>TY Nelson Department of Neurology and Neurosurgery, The Children's Hospital, Westmead</wicri:regionArea><wicri:noRegion>Westmead</wicri:noRegion></affiliation></author><author><name sortKey="Procopis, Peter" sort="Procopis, Peter" uniqKey="Procopis P" first="Peter" last="Procopis">Peter Procopis</name><affiliation wicri:level="4"><nlm:affiliation>TY Nelson Department of Neurology and Neurosurgery, The Children's Hospital, Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>TY Nelson Department of Neurology and Neurosurgery, The Children's Hospital, Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney</wicri:regionArea><placeName><settlement type="city">Sydney</settlement><region type="état">Nouvelle-Galles du Sud</region></placeName><orgName type="university">Université de Sydney</orgName></affiliation></author><author><name sortKey="Ging, Joanne" sort="Ging, Joanne" uniqKey="Ging J" first="Joanne" last="Ging">Joanne Ging</name><affiliation wicri:level="1"><nlm:affiliation>Department of Paediatrics, The Children's Hospital, Westmead, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>Department of Paediatrics, The Children's Hospital, Westmead</wicri:regionArea><wicri:noRegion>Westmead</wicri:noRegion></affiliation></author><author><name sortKey="Chua, Yew Wee" sort="Chua, Yew Wee" uniqKey="Chua Y" first="Yew Wee" last="Chua">Yew Wee Chua</name><affiliation wicri:level="1"><nlm:affiliation>Department of Paediatrics, The Children's Hospital, Westmead, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>Department of Paediatrics, The Children's Hospital, Westmead</wicri:regionArea><wicri:noRegion>Westmead</wicri:noRegion></affiliation></author><author><name sortKey="Buckmaster, Adam" sort="Buckmaster, Adam" uniqKey="Buckmaster A" first="Adam" last="Buckmaster">Adam Buckmaster</name><affiliation wicri:level="1"><nlm:affiliation>Department of Paediatrics, Gosford Hospital, NSW, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>Department of Paediatrics, Gosford Hospital, NSW</wicri:regionArea><wicri:noRegion>NSW</wicri:noRegion></affiliation></author><author><name sortKey="Wood, Nicholas" sort="Wood, Nicholas" uniqKey="Wood N" first="Nicholas" last="Wood">Nicholas Wood</name><affiliation wicri:level="4"><nlm:affiliation>Department of Paediatrics, The Children's Hospital, Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>Department of Paediatrics, The Children's Hospital, Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney</wicri:regionArea><placeName><settlement type="city">Sydney</settlement><region type="état">Nouvelle-Galles du Sud</region></placeName><orgName type="university">Université de Sydney</orgName></affiliation></author><author><name sortKey="Jones, Cheryl" sort="Jones, Cheryl" uniqKey="Jones C" first="Cheryl" last="Jones">Cheryl Jones</name><affiliation wicri:level="4"><nlm:affiliation>Department of Infectious Disease, The Children's Hospital, Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>Department of Infectious Disease, The Children's Hospital, Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney</wicri:regionArea><placeName><settlement type="city">Sydney</settlement><region type="état">Nouvelle-Galles du Sud</region></placeName><orgName type="university">Université de Sydney</orgName></affiliation></author><author><name sortKey="Dale, Russell C" sort="Dale, Russell C" uniqKey="Dale R" first="Russell C" last="Dale">Russell C. Dale</name><affiliation wicri:level="4"><nlm:affiliation>TY Nelson Department of Neurology and Neurosurgery, The Children's Hospital, Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney, Australia. Electronic address: Russell.dale@health.nsw.gov.au.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>TY Nelson Department of Neurology and Neurosurgery, The Children's Hospital, Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney</wicri:regionArea><placeName><settlement type="city">Sydney</settlement><region type="état">Nouvelle-Galles du Sud</region></placeName><orgName type="university">Université de Sydney</orgName></affiliation></author></analytic><series><title level="j">European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society</title><idno type="eISSN">1532-2130</idno><imprint><date when="2015" type="published">2015</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Australia</term><term>Brain Diseases (complications)</term><term>Brain Diseases (pathology)</term><term>Child</term><term>Child, Preschool</term><term>Female</term><term>Humans</term><term>Magnetic Resonance Imaging</term><term>Male</term><term>Syndrome</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Australie</term><term>Encéphalopathies ()</term><term>Encéphalopathies (anatomopathologie)</term><term>Enfant</term><term>Enfant d'âge préscolaire</term><term>Femelle</term><term>Humains</term><term>Imagerie par résonance magnétique</term><term>Mâle</term><term>Syndrome</term></keywords><keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Encéphalopathies</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Brain Diseases</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain Diseases</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Australia</term><term>Child</term><term>Child, Preschool</term><term>Female</term><term>Humans</term><term>Magnetic Resonance Imaging</term><term>Male</term><term>Syndrome</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Australie</term><term>Encéphalopathies</term><term>Enfant</term><term>Enfant d'âge préscolaire</term><term>Femelle</term><term>Humains</term><term>Imagerie par résonance magnétique</term><term>Mâle</term><term>Syndrome</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Mild encephalopathy with a reversible splenial lesion (MERS) is a clinico-radiological syndrome characterized by a transient mild encephalopathy and a reversible lesion in the splenium of the corpus callosum on MRI. This syndrome has almost universally been described in children from Japan and East Asia. Here we describe seven cases of MERS occurring in Caucasian Australian children from one centre seen over a 3 year period. All patients had a fever-associated encephalopathy (n = 7), which presented with confusion (n = 4), irritability (n = 3), lethargy (n = 3), slurred speech (n = 3), drowsiness (n = 2) and hallucinations (n = 2). Other neurological symptoms included ataxia (n = 5) and seizures (n = 1). These symptoms resolved rapidly over 4-6 days followed by complete neurological recovery. In all patients, MRI performed within 1-3 days of onset of encephalopathy demonstrated a symmetrical diffusion-restricted lesion in the splenium of the corpus callosum. Three patients had additional lesions involving other parts of the corpus callosum and adjacent periventricular white matter. These same three patients had mild persisting white matter changes evident at followup MRI, while the other patients had complete resolution of radiological changes. A potential trigger was present in five of the seven cases: Kawasaki disease, Salmonella, cytomegalovirus, influenza B and adenovirus (all n = 1). Elevated white cell count (n = 4), elevated C reactive protein (n = 5) and hyponatremia (n = 6) were commonly observed. CSF was performed in four patients, which showed no pleocytosis. This case series of MERS demonstrates this condition occurs outside of East Asia and is an important differential to consider in children presenting with acute encephalopathy.</div></front></TEI><affiliations><list><country><li>Australie</li></country><region><li>Nouvelle-Galles du Sud</li></region><settlement><li>Sydney</li></settlement><orgName><li>Université de Sydney</li></orgName></list><tree><country name="Australie"><noRegion><name sortKey="Ka, Amy" sort="Ka, Amy" uniqKey="Ka A" first="Amy" last="Ka">Amy Ka</name></noRegion><name sortKey="Britton, Philip" sort="Britton, Philip" uniqKey="Britton P" first="Philip" last="Britton">Philip Britton</name><name sortKey="Buckmaster, Adam" sort="Buckmaster, Adam" uniqKey="Buckmaster A" first="Adam" last="Buckmaster">Adam Buckmaster</name><name sortKey="Chua, Yew Wee" sort="Chua, Yew Wee" uniqKey="Chua Y" first="Yew Wee" last="Chua">Yew Wee Chua</name><name sortKey="Dale, Russell C" sort="Dale, Russell C" uniqKey="Dale R" first="Russell C" last="Dale">Russell C. Dale</name><name sortKey="Ging, Joanne" sort="Ging, Joanne" uniqKey="Ging J" first="Joanne" last="Ging">Joanne Ging</name><name sortKey="Jones, Cheryl" sort="Jones, Cheryl" uniqKey="Jones C" first="Cheryl" last="Jones">Cheryl Jones</name><name sortKey="Procopis, Peter" sort="Procopis, Peter" uniqKey="Procopis P" first="Peter" last="Procopis">Peter Procopis</name><name sortKey="Troedson, Christopher" sort="Troedson, Christopher" uniqKey="Troedson C" first="Christopher" last="Troedson">Christopher Troedson</name><name sortKey="Webster, Richard" sort="Webster, Richard" uniqKey="Webster R" first="Richard" last="Webster">Richard Webster</name><name sortKey="Wood, Nicholas" sort="Wood, Nicholas" uniqKey="Wood N" first="Nicholas" last="Wood">Nicholas Wood</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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