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Primitive megalencephaly in children: Natural history, medium term prognosis with special reference to external hydrocephalus

Identifieur interne : 001558 ( Istex/Corpus ); précédent : 001557; suivant : 001559

Primitive megalencephaly in children: Natural history, medium term prognosis with special reference to external hydrocephalus

Auteurs : B. Laubscher ; T. Deonna ; A. Uske ; G. Van Melle

Source :

RBID : ISTEX:C012DFADBF730A965F2D8529C898CD82F44236F8

Abstract

Abstract: We studied 74 children with primitive megalencephaly retrospectively with attention directed to familial megalencephaly, birth history, enlarged pericerebral subarachnoid space (SAS) (idiopathic external hydrocephalus), head and statural growth dynamics, developmental and school prognosis, morphological findings and development of subdural haematoma. In the megalencephalic children, no significant differences were found between those with normal or those with enlarged pericerebral SAS. Out of 62, 31 children (50%) were already megalencephalic at birth. Of 74, 37 children (50%) showed variable degrees of developmental delay which in 18 was transient. Eight out of 74 were mentally retarded. Of 52 children at school age, 42 attend normal schools and 10, of whom 7 are mentally retarded, attend special schools. Three children showed subdural haematoma resulting from apparently minor trauma or occurring spontaneously. We suggest that idiopathic external hydrocephalus represents a variant of primitive megalencephaly with transient increase of intracranial pressure and that it could predispose to the development of idiopathic (spontaneous or non-traumatic) subdural haematoma.

Url:
DOI: 10.1007/BF01959405

Links to Exploration step

ISTEX:C012DFADBF730A965F2D8529C898CD82F44236F8

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<GivenName>B.</GivenName>
<FamilyName>Laubscher</FamilyName>
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<AuthorName DisplayOrder="Western">
<GivenName>T.</GivenName>
<FamilyName>Deonna</FamilyName>
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<AuthorName DisplayOrder="Western">
<GivenName>A.</GivenName>
<FamilyName>Uske</FamilyName>
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<AuthorName DisplayOrder="Western">
<GivenName>G.</GivenName>
<Particle>van</Particle>
<FamilyName>Melle</FamilyName>
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<OrgDivision>Department of Paediatrics (Neuropaediatric Unit)</OrgDivision>
<OrgName>Centre Hospitalier Universitaire Vaudois</OrgName>
<OrgAddress>
<Postcode>CH-1011</Postcode>
<City>Lausanne</City>
<Country>Switzerland</Country>
</OrgAddress>
</Affiliation>
<Affiliation ID="Aff2">
<OrgDivision>Department of Radiology</OrgDivision>
<OrgName>Centre Hospitalier Universitaire Vaudois</OrgName>
<OrgAddress>
<Postcode>CH-1011</Postcode>
<City>Lausanne</City>
<Country>Switzerland</Country>
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<OrgDivision>Institute of Social and Preventive Medicine</OrgDivision>
<OrgName>University of Lausanne</OrgName>
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<City>Lausanne</City>
<Country>Switzerland</Country>
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<Heading>Abstract</Heading>
<Para>We studied 74 children with primitive megalencephaly retrospectively with attention directed to familial megalencephaly, birth history, enlarged pericerebral subarachnoid space (SAS) (idiopathic external hydrocephalus), head and statural growth dynamics, developmental and school prognosis, morphological findings and development of subdural haematoma. In the megalencephalic children, no significant differences were found between those with normal or those with enlarged pericerebral SAS. Out of 62, 31 children (50%) were already megalencephalic at birth. Of 74, 37 children (50%) showed variable degrees of developmental delay which in 18 was transient. Eight out of 74 were mentally retarded. Of 52 children at school age, 42 attend normal schools and 10, of whom 7 are mentally retarded, attend special schools. Three children showed subdural haematoma resulting from apparently minor trauma or occurring spontaneously. We suggest that idiopathic external hydrocephalus represents a variant of primitive megalencephaly with transient increase of intracranial pressure and that it could predispose to the development of idiopathic (spontaneous or non-traumatic) subdural haematoma.</Para>
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<Heading>Key words</Heading>
<Keyword>Childhood</Keyword>
<Keyword>Hydrocephalus</Keyword>
<Keyword>Megalencephaly</Keyword>
<Keyword>Subdural haematoma</Keyword>
</KeywordGroup>
<AbbreviationGroup>
<Heading>Abbreviations</Heading>
<DefinitionList>
<DefinitionListEntry>
<Term>CSF</Term>
<Description>
<Para>cerebrospinal fluid</Para>
</Description>
</DefinitionListEntry>
<DefinitionListEntry>
<Term>DD</Term>
<Description>
<Para>developmental delay</Para>
</Description>
</DefinitionListEntry>
<DefinitionListEntry>
<Term>IEH</Term>
<Description>
<Para>idiopathic external hydrocephalus</Para>
</Description>
</DefinitionListEntry>
<DefinitionListEntry>
<Term>OFC</Term>
<Description>
<Para>occipitofrontal circumference</Para>
</Description>
</DefinitionListEntry>
<DefinitionListEntry>
<Term>PMG</Term>
<Description>
<Para>primitive megalencephaly</Para>
</Description>
</DefinitionListEntry>
<DefinitionListEntry>
<Term>SAS</Term>
<Description>
<Para>subarachnoid space</Para>
</Description>
</DefinitionListEntry>
</DefinitionList>
</AbbreviationGroup>
<ArticleNote Type="Misc">
<SimplePara>This work has been presented in part at the Société de Neurologie Infantile, Tlemcen, Algeria, November 1987</SimplePara>
</ArticleNote>
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</Journal>
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</role>
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<namePart type="family">van Melle</namePart>
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<abstract lang="en">Abstract: We studied 74 children with primitive megalencephaly retrospectively with attention directed to familial megalencephaly, birth history, enlarged pericerebral subarachnoid space (SAS) (idiopathic external hydrocephalus), head and statural growth dynamics, developmental and school prognosis, morphological findings and development of subdural haematoma. In the megalencephalic children, no significant differences were found between those with normal or those with enlarged pericerebral SAS. Out of 62, 31 children (50%) were already megalencephalic at birth. Of 74, 37 children (50%) showed variable degrees of developmental delay which in 18 was transient. Eight out of 74 were mentally retarded. Of 52 children at school age, 42 attend normal schools and 10, of whom 7 are mentally retarded, attend special schools. Three children showed subdural haematoma resulting from apparently minor trauma or occurring spontaneously. We suggest that idiopathic external hydrocephalus represents a variant of primitive megalencephaly with transient increase of intracranial pressure and that it could predispose to the development of idiopathic (spontaneous or non-traumatic) subdural haematoma.</abstract>
<note>Neuropediatrics</note>
<subject>
<genre>Abbreviations</genre>
<topic>CSF : cerebrospinal fluid</topic>
<topic>DD : developmental delay</topic>
<topic>IEH : idiopathic external hydrocephalus</topic>
<topic>OFC : occipitofrontal circumference</topic>
<topic>PMG : primitive megalencephaly</topic>
<topic>SAS : subarachnoid space</topic>
</subject>
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<title>European Journal of Pediatrics</title>
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<title>Eur J Pediatr</title>
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<dateIssued encoding="w3cdtf">1990-04-01</dateIssued>
<copyrightDate encoding="w3cdtf">1990</copyrightDate>
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<subject>
<genre>Medicine & Public Health</genre>
<topic>Pediatrics</topic>
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<identifier type="ISSN">0340-6199</identifier>
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