Brain abnormalities in patients with hyperimmunoglobulin E syndrome.
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| Title: | Brain abnormalities in patients with hyperimmunoglobulin E syndrome. |
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| Authors: | Freeman AF (AUTHOR), Collura-Burke CJ (AUTHOR), Patronas NJ (AUTHOR), Ilcus LS (AUTHOR), Darnell D (AUTHOR), Davis J (AUTHOR), Puck JM (AUTHOR), Holland SM (AUTHOR) |
| Source: | Pediatrics. May2007, Vol. 119 Issue 5, pe1121-5. 1p. |
| Abstract: | OBJECTIVES: Hyperimmunoglobulin E syndrome is a multisystem disorder with abnormalities of the immunologic, connective tissue, and skeletal tissue systems. Central nervous system abnormalities have not been considered a feature of hyperimmunoglobulin E syndrome. We aimed to determine whether central nervous system abnormalities detected on brain MRI exist in hyperimmunoglobulin E syndrome and to characterize any identified abnormalities. PATIENTS AND METHODS: Fifty patients aged from 3 to 52 years (mean: 24 years) with established diagnoses of hyperimmunoglobulin E syndrome had MRI of the brain as part of an hyperimmunoglobulin E syndrome natural history protocol. Abnormalities were described, measured, counted, and mapped. Patient charts were reviewed for neurologic findings and blood pressure measurements. RESULTS: Focal brain lesions exhibiting high signal intensities on flow-attenuated inversion recovery and on T2-weighted techniques were found in 35 of the 50 patients. The focal hyperintensities were predominantly in the white matter of the cerebral hemispheres, and the number ranged from 2 to >50. The hyperintensities occurred more frequently in adults than in children, and no association with elevated blood pressure was found. Five patients had lacunar infarctions. Chiari type 1 malformations were found in 9 of 50 patients. Two patients had infectious complications presenting on MRI as cerebritis in 1 patient and as a hemorrhagic infarct in the other; both were found on autopsy to be fungal. Neurologic abnormalities were present in 1 patient with a lacunar infarction, the 2 patients with infectious complications, and in 1 patient with a subarachnoid hemorrhage secondary to a berry aneurysm. CONCLUSIONS: Central nervous system abnormalities are common in hyperimmunoglobulin E syndrome. Focal T2 hyperintensities, not appreciated previously, represent a prominent feature of this rare disease that may assist in diagnosis. The etiology and clinical implications of these abnormalities remain to be investigated. [ABSTRACT FROM AUTHOR] |
| Copyright of Pediatrics is the property of American Academy of Pediatrics and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.) | |
| Database: | Psychology and Behavioral Sciences Collection |
| FullText | Text: Availability: 0 |
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| Header | DbId: pbh DbLabel: Psychology and Behavioral Sciences Collection An: 106131801 AccessLevel: 6 PubType: Academic Journal PubTypeId: academicJournal PreciseRelevancyScore: 0 |
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| Items | – Name: Title Label: Title Group: Ti Data: Brain abnormalities in patients with hyperimmunoglobulin E syndrome. – Name: Author Label: Authors Group: Au Data: <searchLink fieldCode="AR" term="%22Freeman+AF%22">Freeman AF</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Collura-Burke+CJ%22">Collura-Burke CJ</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Patronas+NJ%22">Patronas NJ</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Ilcus+LS%22">Ilcus LS</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Darnell+D%22">Darnell D</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Davis+J%22">Davis J</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Puck+JM%22">Puck JM</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Holland+SM%22">Holland SM</searchLink> (AUTHOR) – Name: TitleSource Label: Source Group: Src Data: <searchLink fieldCode="JN" term="%22Pediatrics%22">Pediatrics</searchLink>. May2007, Vol. 119 Issue 5, pe1121-5. 1p. – Name: Abstract Label: Abstract Group: Ab Data: OBJECTIVES: Hyperimmunoglobulin E syndrome is a multisystem disorder with abnormalities of the immunologic, connective tissue, and skeletal tissue systems. Central nervous system abnormalities have not been considered a feature of hyperimmunoglobulin E syndrome. We aimed to determine whether central nervous system abnormalities detected on brain MRI exist in hyperimmunoglobulin E syndrome and to characterize any identified abnormalities. PATIENTS AND METHODS: Fifty patients aged from 3 to 52 years (mean: 24 years) with established diagnoses of hyperimmunoglobulin E syndrome had MRI of the brain as part of an hyperimmunoglobulin E syndrome natural history protocol. Abnormalities were described, measured, counted, and mapped. Patient charts were reviewed for neurologic findings and blood pressure measurements. RESULTS: Focal brain lesions exhibiting high signal intensities on flow-attenuated inversion recovery and on T2-weighted techniques were found in 35 of the 50 patients. The focal hyperintensities were predominantly in the white matter of the cerebral hemispheres, and the number ranged from 2 to >50. The hyperintensities occurred more frequently in adults than in children, and no association with elevated blood pressure was found. Five patients had lacunar infarctions. Chiari type 1 malformations were found in 9 of 50 patients. Two patients had infectious complications presenting on MRI as cerebritis in 1 patient and as a hemorrhagic infarct in the other; both were found on autopsy to be fungal. Neurologic abnormalities were present in 1 patient with a lacunar infarction, the 2 patients with infectious complications, and in 1 patient with a subarachnoid hemorrhage secondary to a berry aneurysm. CONCLUSIONS: Central nervous system abnormalities are common in hyperimmunoglobulin E syndrome. Focal T2 hyperintensities, not appreciated previously, represent a prominent feature of this rare disease that may assist in diagnosis. The etiology and clinical implications of these abnormalities remain to be investigated. [ABSTRACT FROM AUTHOR] – Name: AbstractSuppliedCopyright Label: Group: Ab Data: <i>Copyright of Pediatrics is the property of American Academy of Pediatrics and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.) |
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| RecordInfo | BibRecord: BibEntity: Identifiers: – Type: doi Value: 10.1542/peds.2006-2649 Languages: – Code: eng Text: English PhysicalDescription: Pagination: PageCount: 1 StartPage: e1121 Titles: – TitleFull: Brain abnormalities in patients with hyperimmunoglobulin E syndrome. Type: main BibRelationships: HasContributorRelationships: – PersonEntity: Name: NameFull: Freeman AF – PersonEntity: Name: NameFull: Collura-Burke CJ – PersonEntity: Name: NameFull: Patronas NJ – PersonEntity: Name: NameFull: Ilcus LS – PersonEntity: Name: NameFull: Darnell D – PersonEntity: Name: NameFull: Davis J – PersonEntity: Name: NameFull: Puck JM – PersonEntity: Name: NameFull: Holland SM IsPartOfRelationships: – BibEntity: Dates: – D: 01 M: 05 Text: May2007 Type: published Y: 2007 Identifiers: – Type: issn-print Value: 00314005 Numbering: – Type: volume Value: 119 – Type: issue Value: 5 Titles: – TitleFull: Pediatrics Type: main |
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